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Hand tumors: diagnosis & management of benign (ganglia, glomus) and malignant lesions (sarcomas), prioritizing functional preservation.

210 citationsUpdated Oct 2026

Overview¶

Tumors of the hand and upper extremity encompass a diverse spectrum of benign and malignant lesions, including nerve sheath tumors, soft-tissue sarcomas, and cutaneous neoplasms. Definitive diagnosis requires pathologic evaluation, as MRI findings alone are not conclusive [1]. For brachial plexus tumors, asymptomatic benign lesions warrant observation, whereas biopsies are mandatory for suspected malignancies prior to definitive treatment [2]. The biopsy procedure for malignant primary bone and soft-tissue tumors must be planned with the same rigor as definitive surgery, and patients should be referred to a treating center if the initial institution lacks the capacity for accurate diagnostics or definitive care [43, 57]. Knowledge regarding the treatment of skin tumors in this region is often derived from case reports and retrospective reviews rather than controlled trials [42].

Surgical excision is the primary treatment modality for most symptomatic lesions, including perionychium tumors, intravenous pyogenic granuloma, extraskeletal chondroma, and pigmented onychomatricoma [5, 45, 141, 173]. For malignant peripheral nerve sheath tumours, complete surgical excision with clear margins is the only curative treatment, though prognosis remains poor, particularly in patients with neurofibromatosis [34]. Wide surgical excision is the standard of care for synovial sarcoma, with adjuvant radiation utilized for larger and deeper lesions [36]. Management of benign tumors emphasizes balancing tumor recurrence risk against postoperative functionality within the context of histologic diagnoses and surgical approaches [180].

Long-term clinical follow-up is critical, with a 10-year period advised for soft-tissue sarcoma of the hand even after adequate oncologic treatment [11]. Patients should not be deceived by the smaller size of these tumors regarding the need for surveillance [11]. Recurrence rates are rare following complete removal of perionychium tumors [5], and the prognosis for intravenous pyogenic granuloma is excellent following excision [45]. However, specific characteristics of squamous cell carcinoma may increase recurrence chances, although the technique of tumor excision did not have a major role in outcome [135]. The absence of disease on MRI should not be the sole criterion for determining whether repeat resection is necessary after marginal excision of unsuspected soft tissue sarcomas [10].

Anatomy & Pathophysiology¶

Bony Anatomy¶

The skeleton of the hand and wrist comprises 27 bones, 19 of which are long bones [26]. The hand skeleton is organized into five rays, each forming a polyarticulated chain of metacarpals and phalanges [26]. The base of each metacarpal articulates with the distal carpal row, while the carpus articulates with the forearm via its proximal row [26]. The radioulnocarpal articulation possesses two axes of movement, supplemented by a third axis of pronation and supination from the forearm, granting the wrist three total axes that permit the hand to assume any spatial configuration [26].

The radial ray (first ray) is the shortest, consisting of a metacarpal and two phalanges, and continues the external carpal column formed by the scaphoid and trapezium [26]. The trapezium is angled anterior to the carpal plane, creating an approximately 45-degree angle between the first and second metacarpals in the sagittal plane [26]. The thumb metacarpal is the shortest, whereas the index metacarpal is the longest [26]. The proximal and middle phalanges of the long and ring fingers are longer than those of the index finger [26]. Epiphyseal plates are located at the proximal ends of the phalanges and the first metacarpal, and at the distal ends of the other metacarpals [26].

The index metacarpal is the most firmly fixed, while the ring metacarpal exhibits approximately 10 degrees of mobility in flexion and extension, and the fifth metacarpal has a range of approximately 20 degrees [92]. The second through fifth metacarpals are bound by fibrous structures, most distally the deep transverse intermetacarpal ligament, also known as the interglenoid ligament because it ties together the anterior glenoid ligaments of the metacarpophalangeal articulations [92]. The metacarpophalangeal joints serve as the keystones of the hand’s longitudinal arches, with their stability essential for supporting both the longitudinal and transverse metacarpal arches [92]. Thick anterior glenoid capsules, or volar plates, prevent hyperextension at these joints [92].

The hand presents a longitudinal and transverse concavity, forming a cup shape with palmar concavity when the thumb is placed next to the index finger [26]. The transverse axis of the palm corresponds to the metacarpophalangeal articulations and is oblique, being more distal at the index joint and more proximal at the fifth joint [26]. This transverse palmar axis forms an acute angle of approximately 75 degrees with the longitudinal axis [26].

Muscular Anatomy¶

Control of digital posture requires a complex balance of extrinsic and intrinsic muscle forces [23]. Extrinsic muscles originate outside the hand and insert on the hand or carpus, while intrinsic muscles have both origin and insertion within the hand [23]. The extrinsic extensors traverse six fibroosseous retinacular compartments at the wrist: * First compartment: Abductor pollicis longus and extensor pollicis brevis [23]. * Second compartment: Extensor carpi radialis longus and extensor carpi radialis brevis [23]. * Third compartment: Extensor pollicis longus, which turns abruptly radialward about Lister tubercle [23]. * Fourth compartment: Extensor indicis proprius, lying deep to the four tendons of the extensor digitorum communis [23]. * Fifth compartment: Extensor digiti quinti [23]. * Sixth compartment: Extensor carpi ulnaris, which inserts at the base of the little finger metacarpal [23].

The extensor digitorum communis tendons of the middle, ring, and little fingers are tethered by juncturae tendinum over the dorsum of the hand proximal to the metacarpophalangeal joint [23]. Digital extensor tendons are stabilized over the mid-line of the metacarpophalangeal joint by attachment to sagittal band fibers, which insert onto the volar proximal phalanx and lateral borders of the volar plate [23]. Rupture or attenuation of these fibers allows the extrinsic extensor tendon to sublux to the ulnar side of the metacarpal head, causing ulnar deviation of the finger [23].

The extrinsic finger flexors are the flexor digitorum profundus and the flexor digitorum superficialis [23]. The flexor digitorum profundus inserts on the proximal volar aspect of the distal phalanx, while the flexor digitorum superficialis acts as a flexor of the proximal interphalangeal and metacarpophalangeal joints [23]. There are seven interosseous muscles: four dorsal (abductors) and three volar (adductors) [84]. The middle finger has two dorsal interossei and no volar interossei because the central axis of the hand lies within it [84]. Each dorsal interosseous muscle, except the third, has two heads: a superficial head that abducts and weakly flexes the proximal phalanx, and a deep head that flexes and weakly abducts the proximal phalanx while extending the middle and distal phalanges [84]. The volar interossei have a single head and do not insert onto the proximal phalanx [84].

The flexor digitorum profundus originates from the proximal ulna and interosseous membrane, forming the deep compartment of the volar forearm with the flexor pollicis longus [106]. The profundus of the index and middle fingers is innervated by the anterior interosseous branch of the median nerve, while the profundus of the ring and little fingers is innervated by the ulnar nerve [106]. The flexor digitorum superficialis has a radial head from the proximal radius and a humeral ulnar head from the medial humeral epicondyle and coronoid process, receiving innervation entirely from the median nerve [106]. Its primary function is digital flexion at the proximal interphalangeal joint [106]. The flexor pollicis longus originates from a radial head (proximal radius and interosseous membrane) and an accessory head (coronoid process and medial epicondyle), is innervated by the anterior interosseous branch of the median nerve, and flexes both the interphalangeal and metacarpophalangeal joints of the thumb [106].

Tendon and Sheath Anatomy¶

The tenosynovial sheath of the flexor pollicis longus is continuous with the radial bursa, while the sheath to the little finger is continuous with the ulnar digital bursa [106]. In some patients, these bursae communicate, allowing a horseshoe abscess to spread between the thumb and little finger if infection occurs in either flexor tendon sheath [106]. The fibroosseous tunnel, or digital flexor sheath, extends distally to the proximal aspect of the distal phalanx [106]. The tendinous sheath consists of annular pulleys for mechanical stability and cruciate pulleys for flexibility [106].

The annular pulleys are distributed as follows: * A1, A3, and A5: Located over the metacarpophalangeal, proximal interphalangeal, and distal interphalangeal joints, respectively [106]. * A2 and A4: Situated over the middle portion of the proximal and middle phalanges [106].

The A2 and A4 pulleys are the most essential for maintaining the mechanical advantage of the flexor tendons [106]. The tenosynovium lining the fibroosseous tunnel supplies nutrition and lubrication to the poorly vascularized flexor tendons [106]. Within the sheath, tendon vascularity is supplied by the vincula system (vinculum longus and brevis) [106].

In unrestricted areas, tendon gliding depends on the paratenon and areolar connective tissue, whereas in narrow crowded areas, it is assured by the synovial sheath [108]. Synovial sheaths at specific sites are surrounded by fibrous sheaths that keep the tendon close to the skeleton, assuming the role of a pulley when the tendon changes direction [108]. The gliding mechanism represented by synovial sheaths is much more developed on the palmar aspect [108]. There are three digitopalmar synovial sheaths for the flexor tendons of the index, long, and ring fingers [108]. On the dorsal aspect, extensor synovial tendon sheaths are present only at the level of the wrist [108].

Vascular and Neural Anatomy¶

The hand’s blood and nerve supplies are continuous with those of the rest of the limb [78]. The most common variations of the palmar arteries of the thumb are schematized by dividing the thumb into three segments defined by the metacarpophalangeal and interphalangeal flexion creases [97]. The "princeps pollicis" artery, a terminal branch of the radial artery crossing the first intermetacarpal space, runs along the ulnar side of the first metacarpal and the volar surface of the adductor muscle [97]. It divides into two terminal rami, the collateral palmar arteries of the thumb, at the level of the cutaneous flexion crease of the metacarpophalangeal joint [97]. An arcade located deep in the flexor tendon joins these two arteries at the level of the distal metaphysis of the first phalanx [97]. Only 15% of dissections of the palmar arteries of the thumb fall into the classical "typical" category [97].

In the second segment of the thumb, the main artery is the ulnar collateral artery, with a subtendinous anastomosis at the neck of the first phalanx acting as a moderator between the two collateral arteries [97]. The dorsal arteries of the thumb originate from the palmar arteries (princeps, commissural, or anastomoses of the superficial arcade) at the level of the first metacarpal [97]. Digital neurovascular structures are surrounded by a diffuse network of thin transverse oblique fibers, with fibers dorsal to the bundle called Cleland ligament and fibers palmar to the bundle called Grayson ligament [115].

The superficial palmar fascia lies in a coronal plane beneath the palmar subcutaneous tissue, covering a triangular area of the central palm with the proximal corner facing directly proximal [115]. The palmaris longus tendon, when present, terminates in continuity with the fibers of this proximal corner [115]. Four central bands of fascia extend distally from the proximal corner toward each of the fingers, with no central band for the thumb [115]. These central bands are bridged transversely by the superficial transverse palmar ligament at the distal palmar crease level [115]. The recurrent branch (motor) of the median nerve is the most important structure in the thenar area and should be exposed and protected if its exact location is in doubt [98]. Anatomic studies show that no single longitudinal incision in the proximal palm completely avoids the palmar cutaneous branches of the median and ulnar nerves [98].

Cutaneous Anatomy¶

The dorsal skin possesses a papillary dermis, but the papillary ridges are neutralized by the epidermis and do not show on the surface [110]. The dorsal epidermis is thin, lined by a horny layer only 0.02 mm thick, and the dorsal dermis is thinner and less resistant than the palmar dermis [110]. Unlike the palm, dorsal skin possesses a normal pilosebaceous system [110]. Dorsal skin becomes fragile in old age and has greater vulnerability to factors causing cutaneous atrophy, such as steroid therapy [110]. Flexion of the fingers produces significant lengthening of the dorsal skin; in the middle finger, the distance between the wrist and the ungual fold increases by an average of 3 cm from extension to full flexion, with flexion at the metacarpophalangeal joint alone requiring an average skin lengthening of 1.25 cm [110]. The dorsal and palmar areas of skin are independent due to a system of adhesions anchoring their common boundary to the underlying plane [110].

The palm forms a cutaneous unit extending from the distal transverse crease of the wrist up to the transverse crease at the base of the digits, subdivided into two zones by the oppositional crease of the thumb [81]. The skin of the radial portion covers the thenar eminence and is relatively well vascularized and mobile, while the skin of the ulnar and distal portion covers the hypothenar eminence where mobility is poor [81]. The central triangular part of the palm has fixed and poorly vascularized skin covering almost directly the superficial palmar aponeurosis [81]. The integument of the palmar face of the digits is subdivided into phalangeal units separated by digital flexion folds [81]. When a digit is completely flexed, the integument of adjacent phalanges comes into contact in the zones of the flexion creases, establishing areas of cutaneous contact in the form of a diamond [81]. The sides of this diamond do not undergo variations in length during movements of flexion and extension, and incisions made along these lines present a minimal chance of retraction [81].

The web spaces are formed from the union of two nonsymmetrical cutaneous surfaces [81]. The dorsal slope of the web space has a gradual incline and its supple skin is not adherent to the subjacent region, whereas the palmar surface is flat and precipitously interrupted, with skin densely adherent to the commissural skeleton [81]. The commissural skeleton is formed by the interdigital palmar (natatory) ligament between the fingers and by the distal transverse ligament at the level of the thumb web [81].

Pathophysiology of Tumors¶

Benign peripheral nerve tumors encompass a wide range of neoplasms and non-neoplastic tumor-like lesions, some of which have different levels of malignant potential [18]. Unicameral bone cysts are common lesions of childhood more consistent with a developmental or reactive lesion than a true tumor [66]. Eighty-five percent of unicameral bone cysts occur in the first two decades with a 2:1 male predominance [66]. These cysts are most common in the proximal humerus and femur in children, while in adults they are more common in the ilium and calcaneus [66].

Classification¶

General Staging Systems¶

Enneking: Proposed by W. F. Enneking at the University of Florida and agreed upon by the Musculoskeletal Tumor Society in 1979, this system classifies primary bone tumors based on grade (high or low), presence of metastases, and whether the tumor is intracompartmental or extracompartmental [27, 67]. A compartment is defined as an enclosed tissue space, such as a bone, a joint space, or a muscle group confined by its fascial envelope [147]. The system remains intuitive for surgeons [156].

AJCC: The American Joint Committee on Cancer (AJCC) staging system is based on tumor size and grade, lymph node involvement, and the presence or absence of distant metastases (TNM) [51]. It is more comprehensive than the Enneking system because it incorporates these additional factors [51]. The AJCC has widespread acceptance due to its multidisciplinary nature and ability to facilitate more nuanced prediction of prognosis [156].

TNM: In the TNM staging system for soft-tissue sarcomas, size is dichotomized as small (<5 cm) or large (>5 cm) [147]. For bone sarcomas, size is dichotomized as small (<8 cm) or large (>8 cm) [147]. The TNM system has gained favor for staging soft-tissue sarcomas and has been adopted by the AJCC and the Union for International Cancer Control (UICC) [147].

Surgical Margins: Surgical margins are defined as intralesional, marginal, wide, or radical to obviate confusion in understanding the extent of a proposed or completed procedure [67].

Staging Principles: The basis of all staging systems includes the grade of the tumor, size of the tumor, local extent, and presence of metastases [147]. A good surgical staging system incorporates prognostic factors into a system that describes progressive degrees of risk to the patient, defines progressive stages of the disease that have implications for surgical management, assists in chemotherapy guidelines, and promotes interinstitutional protocols and pooling of rare tumors in rare locations [67].

Benign Bone Tumor Classification¶

Enneking Benign: Benign bone lesions are classified into three stages: stage 1 (latent), stage 2 (active), and stage 3 (locally aggressive) [69]. A stage 1 tumor is latent, meaning it does not progress or heals spontaneously [70]. A stage 2 tumor is active, meaning it progresses but respects natural barriers such as the bone cortex [70]. A stage 3 tumor is aggressive, meaning it progresses and will ultimately destroy natural barriers [70]. Enchondromas are usually stage 1 but are occasionally stage 2 [70].

Enneking Clinico-Radiological: The Enneking clinico-radiological classification for benign bone tumors includes stage 1 (latent), stage 2 (active), stage 3 (aggressive), and stage IV (malignant) [37]. An Enneking stage 1 benign bone tumor is confined totally by bone, asymptomatic, inactive on bone scan, and histologically benign [37]. An Enneking stage 2 benign bone tumor has an expanded cortex with no breakthrough, is symptomatic (often with a pathologic fracture), active on bone scan, and histologically benign [37]. An Enneking stage 3 benign bone tumor is a rapidly growing mass with cortical perforation and soft tissue mass, may metastasize, is symptomatic, has extensive activity on bone scan, and is histologically benign [37]. An Enneking stage IV benign bone tumor refers to a sarcomatous lesion contiguous with a benign giant cell tumor [37].

Campanacci: The Campanacci classification grades giant cell tumors (GCT) into three grades based on radiographic appearance: grade 1 (latent), grade 2 (active), and grade 3 (aggressive) [37]. A Campanacci grade 1 GCT lesion has a well-defined margin and an intact cortex [37]. A Campanacci grade 2 GCT lesion has a relatively well-defined margin but no radiopaque rim, with thinned and moderately expanded cortex [37]. A Campanacci grade 3 GCT lesion has indistinct borders and cortical destruction [37]. The Campanacci grading system for GCT is similar to the Enneking classification for benign bone tumors overall [37].

Jaffe: Jaffe et al. classified giant cell tumors as benign, aggressive, and malignant based on the histological appearance of stromal cells and the number of giant cells and mitoses [37].

Other Considerations: A novel GCT grading system demonstrates excellent interrater agreement and classified more nonrecurrent curetted tumors as low grade compared with the Campanacci classification [197]. Benign bone tumors can be grouped according to tissue type, including cartilage tumors, cystic lesions, osteogenic tumors, and miscellaneous lesions [69]. Fibro-osseous and cystic tumors of bone are common lesions with distinct clinical, radiologic, and pathologic features that aid in their classification [144].

Malignant Bone Tumor Classification¶

Enneking Malignant: Nonmetastatic low-grade chondrosarcomas are considered stage I neoplasms according to the Enneking system [70]. Nonmetastatic intermediate- and high-grade chondrosarcomas are stage II [70]. Metastatic chondrosarcomas are stage III [70]. Chondrosarcomas are subclassified as stage A or stage B based on whether they are located within the bone or extend outside the bone [70]. A low-grade intramedullary chondrosarcoma without metastases is stage IA [70]. A high-grade nonmetastatic chondrosarcoma with an associated soft tissue mass is stage IIB [70].

TNM Bone: The TNM staging system for primary sarcomas of bone takes into consideration histological subtype, size, continuity, grade, and local and distant spread [147]. The application of the TNM system to bone tumors has limitations due to the rarity of lymph-node metastases [147].

Other Considerations: Malignant primary bone tumors are rare and varied, requiring management tailored to specific tumor types [41]. High-grade malignant primary bone lesions typically undergo neoadjuvant therapies and surgery, while low-grade lesions are usually managed with surgery alone [41]. The lungs are the most common site for metastases from bone and soft-tissue sarcomas, although other sites may include bone, lymph nodes, liver, and other soft-tissue locations [147].

Soft Tissue Tumor Classification¶

WHO: Sarcomas are classified by the World Health Organization into 12 different groups based on tissue type [51]. The 2020 WHO Classification of Tumors of Soft Tissue provides an updated classification scheme and essential diagnostic criteria, including newly recognized entities, subtypes, and reclassified tumors [22]. The World Health Organization 2013 Soft-Tissue Tumor Categories include adipocytic, fibroblastic/myofibroblastic, so-called fibrohistiocytic, smooth muscle, pericytic, and skeletal muscle groups [51].

AJCC Soft Tissue: Soft-tissue sarcomas are staged via the American Joint Committee on Cancer (AJCC) or Enneking staging systems [51]. The AJCC Cancer Staging Manual, Eighth Edition, creates different staging schemes depending on the anatomic location of the sarcoma, including additional size classifications and grouping lymph node metastases with distant metastasis [51].

Other Considerations: The heterogeneous nature of sarcoma behavior limits the prognostic value of staging systems when compared with other cancers [51]. Soft-tissue sarcomas comprise a heterogeneous and rare group of tumors that display a wide spectrum of local growth patterns and metastatic potential [72]. Malignant fibrous histiocytoma is an aggressive lesion originating in the tissue histiocyte that contains five cell types and has often been grouped with other sarcomas [196].

Specific Tumor Classifications¶

Vascular: Vascular bone tumors are classified into hemangioma, hemangioendothelioma, angiosarcoma, and their epithelioid variants [16]. The international classification distinguishes between vascular tumors and malformations, which have radically opposite evolutive profiles [21]. Vascular tumors of the hand are classified into benign, congenital, and malignant categories [167]. Vascular leiomyomas in the hand have three pathologic types: solid, cavernous, and venous type tumors [162]. A review of 516 vascular tumors of the upper extremity classified 90% as either hemangiomas or malformations [58].

Other Specific: The PUMCH classification for extremity and pelvic hemophilic pseudotumors is based on anatomic pathology and surgical strategy [192]. Confirming PIK3CA mutation status allows for more accurate diagnosis and classification of isolated macrodactyly based on its molecular pathogenesis [199]. Lipoblastoma and infiltrating lipoma are classified as benign fatty tumors of the upper limb in children despite their rapid growth [158]. Schwannomas and neurofibromas are two types of peripheral nerve tumors, with schwannomas being resectable and neurofibromas often requiring epineurotomy if unresectable [12]. Schwannomas and neurofibromas are commonly associated in Von Recklinghausen's disease but have not been reported in association outside this syndrome [159].

Clinical Presentation¶

General Diagnostic Principles¶

A thorough history, physical examination, and baseline plain x-rays form the foundation of accurate diagnosis for benign tumors of the hand [95]. For benign-appearing hand and finger tumors, a pertinent history, physical examination, and plain radiographs are frequently sufficient to determine the diagnosis and appropriate treatment [118, 130]. In one series, the clinical diagnosis of tumorous conditions of the hand was accurate in 94% of cases [103]. For soft-tissue tumors in children, a thorough history and physical examination followed by appropriate imaging studies can establish a correct diagnosis and guide treatment recommendations [19].

The history must elicit the duration of symptoms and accurately identify the size and location of the mass [94]. An adequate history and physical examination are the first steps in evaluating a patient with a musculoskeletal tumor [105]. Patients may present to the orthopaedic oncologist with pain, a mass, or an abnormal radiographic finding detected during the evaluation of an unrelated problem [105]. Age is often the most important historical information because most benign and malignant musculoskeletal neoplasms occur within specific age ranges [105]. Family history is helpful in cases of multiple hereditary exostosis and neurofibromatosis, both of which have autosomal dominant inheritance [105]. Some tumors show a sex predilection, such as female predominance with giant cell tumors, but this is rarely of diagnostic significance [105]. Race is of little diagnostic significance, with the exception that Ewing sarcoma is exceedingly rare in individuals of African descent [105].

Distinguishing soft-tissue and bone tumors is a key starting point for treatment [31]. Pain, or lack thereof, is not indicative of malignancy in soft-tissue tumors [31]. Plain radiographs are an excellent starting point for most bone tumors [31]. MRI is often helpful for soft-tissue tumors, bone masses with a soft-tissue component, and any malignant tumor [31]. The diagnostic strategy for a bone tumor depends on the clinician’s ability to make an accurate differential diagnosis based on clinical information and plain radiographs [96]. The clinician must classify the patient as having a non-progressive or progressive primary benign bone tumor, a primary malignant bone tumor, or a metastatic bone tumor [96]. If clinical and radiographic information favors a diagnosis of malignant or aggressive benign bone tumor, the clinician should refer the patient to an experienced orthopaedic oncologist without performing additional diagnostic tests or a biopsy [96]. Similarly, if a soft-tissue mass is five centimeters in diameter or larger on physical examination, and especially if it is deep to the fascia, the patient should be referred to an orthopaedic oncologist without additional evaluation or biopsy [96].

Most benign bone tumors in children have characteristic clinical and radiographic presentations that allow for diagnosis without biopsy [24]. However, some benign bone tumors in children may simulate malignancy and require referral to an orthopaedic oncologist [24]. Most benign bone tumors have characteristic features that suggest their specific diagnosis, allowing practicing orthopaedic surgeons to follow with imaging or refer to an orthopaedic oncologist [25]. Many benign tumors have characteristics that enable the clinician to arrive at a diagnosis without biopsy [95]. Certain lesions with a characteristic appearance and benign features may be managed nonsurgically with observation alone [95]. Vigilance in monitoring for signs of malignancy is encouraged, even for seemingly benign tumors [95]. Signs of malignancy, such as rapid growth, increasing pain, or other atypical features, mandate a thorough workup [95]. If a lesion increases in size or becomes symptomatic, or if the physical or radiographic appearance suggests an aggressive lesion, appropriate staging studies including a tissue diagnosis must be obtained [27]. Diagnostic delay or inappropriate treatment may result if patients do not receive appropriate followup, biopsy (usually open), or referral whenever the diagnosis is in doubt [28]. A lower threshold for further workup in patients with atypical signs and symptoms allows for the potential to identify potential malignancy earlier [89].

Awareness of tumor features may help physicians with diagnosis [109]. Awareness and understanding of tumor characteristics may help physicians with diagnosis and treatment [79]. Updated knowledge and understanding of the nature and demographic characteristics of the tumorous conditions of the hand is crucial for an accurate diagnosis [6]. Awareness of uncommon tumors in adult patients is essential to prevent mistaken diagnosis and subsequent extensive workup [100]. It must be considered in the differential diagnosis of tumors of the extremities [102]. Glomus tumor should be included in the differential diagnosis in neurofibromatosis patients who present with a painful lesion of the hand or finger [101].

Specific Tumor Presentations¶

Patients with bone tumors most frequently present with pain [105]. The pain from bone tumors initially may be activity related, but a patient with a malignancy of bone often complains of progressive pain at rest and at night [105]. Patients with benign bone tumors may have activity-related pain if the lesion is large enough to weaken the bone [105]. Osteoid osteoma may cause night pain initially [105]. Patients with soft-tissue tumors rarely complain of pain but more often complain of a mass [105]. Exceptions to the rule that soft-tissue tumors present as masses are patients with nerve sheath tumors who have pain or neurologic signs [105]. Soft tissue sarcomas typically present as enlarging painless masses greater than 5 cm [87]. Tumor-like conditions of the hand and upper extremity typically present as masses and can be confused with more serious conditions [80]. Diagnosis of tumor-like conditions often relies on clinical examination and imaging, with excisional biopsy needed for definitive diagnosis to guide treatment [80].

The suspicion of glomus tumor is based on clinical examination, primarily searching for localized pain [93]. In a series of 11 glomus tumor cases, patients presented with pain which had lasted for a few months to several years (mean duration: 14 years) prior to being admitted for surgery [111]. In the same series, the patient was able to indicate the presumed tumor location due to the presence of pain at that site in 4 out of 9 cases [111]. In the same series, pain was initiated by touch in 7 out of 9 cases and also by cold in 8 out of 9 cases [111]. In the same series, a definitive diagnosis was established after the appearance of a subcutaneous tumefaction in cases with very long symptom duration due to misdiagnosis related to atypical site [111]. Excision of the tumor relieves symptoms in most patients, but clinicians should search for synchronous glomus tumors during excision to avoid recurrence or persistence of symptoms due to undiscovered multiple lesions [104]. Recurrent symptoms following tumor excision most likely are due to the presence of a previously undetected tumor [20].

The tumor presented with characteristics similar to other intraneural tumors (slow growing, without symptoms of neuropathy) making preoperative diagnosis difficult [82]. The case highlights the difficulties encountered in clinical practice to diagnose such tumors due to shared clinical, epidemiological, and imaging characteristics with other soft tissue tumors [91]. Diagnosis and management of neurofibromas affecting digital nerves are both difficult and demanding [85]. Clinical and pathologic diagnosis of granular cell tumor in the hand may be difficult [88]. Diagnosis of MPNST can be challenging due to the rarity of these tumours and unreliable imaging, making a high index of suspicion paramount for early diagnosis and treatment [107]. The current concepts and difficulties in diagnosing peripheral primitive neuroectodermal tumours are discussed [112]. Patients with GCT should be followed indefinitely, and referred promptly if new symptoms, particularly pain, emerge [83].

Imaging and Diagnostic Limitations¶

Sonography generally conveys less detail concerning precise anatomic relationships and tumor margins than CT and MR images [27]. Echo patterns of solid masses are nonspecific; however, deep, fluid-filled lesions (for example, ganglion cysts) can be easily differentiated from solid tumors [27]. Sonography is usually much less expensive than CT or MR imaging [27]. MRI is not definitive for the diagnosis of nerve tumors; definitive diagnosis requires pathologic evaluation [1]. A preoperative diagnosis based on radiographic and MRI characteristics of calcifying aponeurotic fibroma may prevent unnecessary biopsy [32]. History, clinical examination, imaging and histopathological examination are crucial in diagnosis of ancient schwannomas [3].

Plain radiographs are the most useful modality to determine whether the mass is arising in the soft tissues or in bone [114]. Magnetic resonance imaging gives the best resolution both to characterize the lesion and to define the anatomical features [114]. Local imaging studies, including bone scans, angiograms, CT, and MRI, are helpful in surgical planning for aggressive processes [118, 130]. A metastatic workup is indicated in most malignant lesions, and a chest CT is necessary for those tumors with a propensity for metastasizing to the lungs [118, 130].

Investigations¶

Clinical Evaluation¶

A careful physical examination is essential to direct care and future testing, as diagnostic tests such as imaging and serum laboratory studies can be expensive, time-consuming, and often nonspecific [17]. History, clinical examination, imaging, and histopathological examination are crucial in the diagnosis of rare nerve tumors such as ancient schwannomas [3]. A high level of suspicion and histological study are required when there is an underlying history of malignancy [165].

Imaging Modalities¶

MRI: MRI is the preferred modality for the staging of soft tissue tumors and for evaluating patients for tumor recurrence [123]. It is preferred over CT in the preoperative evaluation of soft-tissue tumors due to better spatial orientation and sharper contrast between tumor and adjacent tissues [148]. For all tumor types, MRI, CT, and plain radiographs were frequently rated as helpful studies [121]. MR imaging has become the modality of choice in evaluating synovial tumors and tumor-like lesions, allowing a presumptive diagnosis in most cases [136]. For single benign peripheral nerve tumors, MRI was able to identify tumor type and determine tumor resectability in 80% of cases [44]. In most patients, the resectability of the tumor, its type, and benignity can be predicted based on medical imaging for isolated benign peripheral nerve tumors [55].

In the coronal plane, MRI accurately defined intramedullary abnormality and provided greater interpretive certainty than CT or scintigraphy in defining the true proximal and distal extent of primary malignant bone tumors [178]. MRI examinations depicted glomus tumors in excellent detail and facilitated diagnosis in patients with atypical presentation [183]. High-resolution MRI and ensuring complete excision are recommended to decrease recurrence rates for glomus tumors [151]. High resolution MR imaging indicated a new location of a glomus tumor before the last operation, supporting the hypothesis that a new tumor had developed rather than a recurrence due to incomplete excision [187]. MRI confirmed the diagnosis and extent of lesions in glomus tumors of the hand [120]. Imaging examinations, particularly ultrasound and MRI, should be performed to support the diagnosis of glomus tumors of the forearm [133].

The diagnosis of parosteal osteochondromatous proliferations relies on radiological examination, ideally supplemented by MRI, and is confirmed by histological examination [161]. MRI may be more useful than plain x-rays to identify bone and soft tissue extensions in unusual manifestations of chronic lymphocytic leukemia in the hand [165]. MRI features of soft-tissue osteoma of the hand included a high-signal, heterogeneous, well-defined lesion with no connection to adjacent tendons, joints, or bones [186]. Magnetic resonance imaging was helpful in delineating the lesion in tumoral calcinosis of the triangular fibrocartilage complex but could not identify its nature [145]. MRI did not recognize the intravascular nature of an intravascular myopericytoma of the superficial palmar arch [185]. A repeat MRI did not show recurrence in a case of unifocal giant cell tumor of the triquetrum [146]. Twenty-four months after surgery, a patient with primitive neuroectodermal tumor of the thumb metacarpal bone was free of tumor clinically, on computed tomography scan, and on MRI [164].

CT: Improved investigation techniques, particularly bone scan and tomography, have improved the diagnostic accuracy of osteoid osteoma, with CT scans useful for precise localization of the lesion [168]. Soft tissue radiographic techniques and mammographic films were an extremely valuable diagnostic tool for intraosseous growth of giant cell tumors of the tendon sheath, although CT and MRI may be necessary in some cases [179].

Ultrasound: Ultrasound and MRI confirmed the neural nature of tumors in 90% of cases for single benign peripheral nerve tumors [44].

Plain Radiography: Longitudinal radiographs allowed observation of the timing of the first appearance and the potential for resolution for each tumor type in asymptomatic children with benign bone tumors [13]. Plain radiograph and classic clinical presentation may not always be of diagnostic help for osteoid osteoma of the distal phalanx, so histopathological confirmation is required [169].

Angiography: MRI should be used as a complementary method, while angiography remains necessary for specific cases involving highly vascularized tumors [149].

Diagnostic Limitations and Pathology¶

Definitive diagnosis of nerve tumors requires pathologic evaluation as MRI is not definitive [1].

Treatment¶

General Principles and Multidisciplinary Care¶

Biopsy of musculoskeletal tumors requires rigorous planning comparable to definitive surgery, as a poorly executed procedure can lead to devastating patient outcomes [31]. The coordinated work of a multidisciplinary team—including trained pathologists, radiologists, medical oncologists, radiation oncologists, and surgeons—is mandatory for optimal care in soft-tissue sarcomas [72]. Treatment at specialized cancer centers improves overall survival and reduces local recurrences for adipose tumors [126], while patients with primary malignant bone tumors treated at high-volume facilities experience slightly better overall survival than those at low-volume facilities [171]. Pediatric soft tissue tumor cases must be discussed at a tumor board meeting once diagnosed [7]. Surgery remains an essential component of pediatric soft tissue tumor treatment and must be performed at the appropriate time following potential systemic or local therapy [7].

Benign Tumors¶

Observation and Conservative Management: Asymptomatic benign tumors are managed with observation [2]. Conservative management of juvenile aponeurotic fibroma is justified because most patients do well, though surveillance is warranted to identify recurrence or metastasis early [59]. This condition should be treated conservatively by repeated surgical removal rather than radical excision [190]. Unicameral bone cysts are often asymptomatic unless a pathologic fracture occurs, with two-thirds of patients presenting with fractures that can stimulate cyst healing [66]. These cysts are most active during skeletal growth and usually heal spontaneously at maturity [66]. Unicameral bone cysts in flat bones are typically asymptomatic, found incidentally, and rarely fracture [66]. For desmoid tumors, upfront surgery is not advantageous compared to observation or medical treatment [201], despite the benign nature of these musculoskeletal lesions requiring multidisciplinary care for combined treatment options [30].

Surgical Excision: Definitive surgery for benign tumors is usually accomplished by marginal or wide excision, depending on the biologic nature of the tumor [67]. Surgical excision is the primary treatment for symptomatic perionychium tumors, with rare recurrence following complete removal [5]. It is also the treatment of choice for intravenous pyogenic granuloma of the hand, which carries an excellent prognosis [45]. Local excision of Nora’s lesion is a satisfactory treatment method [166]. Complete surgical excision is uniformly curative for leiomyoma of the hand, as this tumor has never been reported to metastasize [150]. For low-grade myofibroblastic sarcoma of the distal phalanx, treatment includes simple local excision with free surgical margins and long clinical follow-up with imaging [14]. At 18 months following resection of fibrous hamartoma of the wrist in infancy, there is no evidence of recurrence [49]. Benign soft-tissue tumors and reactive lesions are managed based on specific diagnosis, with options including observation, excision, or interventional radiology techniques [211]. Treatment options for non-neoplastic tumors of the hand and upper extremity range from observation and nonsurgical methods to surgical excision, with recurrence rates varying by lesion type and treatment modality [134]. Surgical treatment of benign soft tissue chondromatous lesions of the hand, even in cases of recurrence, should be locally extensive but conservative [200]. Fibromatoses and related tumors of the hand in children are benign and do not metastasize; proper clinical, radiologic, and pathologic assessment is mandatory to avoid mutilating surgery [210]. Complete en bloc excision is the recommended treatment for osteoid osteoma of the clavicle when conservative therapy fails [195]. For benign bone lesions with pathologic fractures, prompt and simultaneous management of the fracture and tumor using minimally invasive techniques and adjuvants minimizes recurrence and maximizes recovery [198].

Specific Lesion Management: Surgical management with curettage is the standard of care for symptomatic enchondromas, with most patients returning to full function after surgery [202]. While previous studies regarded persistent bony defects as evidence of complete excision without recurrence for solitary enchondromas, this opinion is incorrect given the slow asymptomatic growth of this tumor [216]. Early surgical treatment of enchondromas in Ollier Disease may limit the growth of large lesions and facilitate future surgery [53]. Longer-term follow-up is required to confirm the absence of recurrence and malignant transformation for enchondromas treated in childhood [53]. Most osteochondromas in the upper limb are treated similarly to those in other skeletal regions, with surgical removal only necessary if pain or functional impairment occurs [230]. Osteochondromas are not a malignant condition and may undergo spontaneous regression [230]. Whether prophylactic removal of forearm osteochondromas should be performed to prevent rotational dysfunction remains controversial [230]. In a series of 17 forearms with osteochondromas, 85% of the excision-only cohort demonstrated enhanced wrist/elbow range of motion at 20-year follow-up, compared to 70% improvement rates in patients undergoing concurrent limb-lengthening procedures [230]. Forearm lengthening surgery is associated with fixator use, which may lead to severe complications, with four out of six patients requiring unplanned reoperations [230]. The non-surgical group for forearm osteochondromas had more significant functional and cosmetic deficits compared to the surgical group [230]. Tumoral resection for lipofibromatous hamartoma of the radial nerve is only justified in the case of complete preoperative deficit [205]. Adjuvant radiation therapy for extra-abdominal desmoid tumors is probably beneficial, but the precise indication for its use is not well defined [207].

Malignant Tumors¶

Biopsy and Staging: Biopsies should be performed for tumors suspected to be malignant before starting definitive treatment [2].

Surgical Resection: Wide tumor resection aimed at achieving R0 surgical margins remains the cornerstone of management for adult soft-tissue sarcomas of the extremities [72]. Treatment of localized malignant peripheral nerve sheath tumours is surgical excision with curative intent [231]. Malignant peripheral nerve sheath tumours have not been shown to be particularly radio- or chemo-sensitive; therefore, wide excision is the mainstay of treatment [231]. The 5-year survival rate for malignant peripheral nerve sheath tumours is approximately 40% [231]. Risk factors for these tumors include proximal location, age older than 60 years, size greater than 5 cm, and the presence of NF1 [231]. In the setting of nonmetastatic angiosarcoma in the upper extremity, amputation is the treatment of choice [132]. Angiosarcoma uniformly has a poor survival rate [132]. The prognosis for metastatic tumors of the soft tissues of the fingers is extremely poor, with survival generally remaining under 6 months regardless of the aggressiveness of the treatment [125]. Surgical resection is the main treatment for intraperitoneal extraosseous osteosarcoma, and the prognosis is poor [140]. Curettage was found to be ineffective as a method of treatment for giant-cell tumors of the bones of the hand; 13 of 15 tumors treated by curettage recurred [142]. The optimum treatment for Grade III giant-cell tumor lesions and patients with local recurrence is unclear [157]. Grade 1 and grade 2 giant-cell tumor lesions should be treated with intralesional curettage [37]. Conservative treatment for chondrosarcoma of the hand is difficult to conceive due to the narrow spaces of the hand which do not allow for wide resections [217]. The early treatment that will increase survival prospects and may improve the chances of cure for sub-ungual melanoma is rarely achieved [163]. The surgeon should consider sweat gland carcinoma as a potentially fatal entity in the evaluation and management of any long-standing undiagnosed tumor of the hand [206]. Multidisciplinary management combining neoadjuvant modern targeted oral chemotherapy with staged conservative surgical resection led to successful tumor eradication and near full hand function at 2-year follow-up for infantile fibrosarcoma of the hand [215]. An experience with multimodal therapy for sarcomas of the forearm and hand demonstrated a 91% survival rate, with an 8% rate of local recurrence [138]. No significant survival benefit was identified for individual tumors in a study of improved survival for sarcomas of the wrist and hand [152]. There are four basic principles or goals of limb-sparing procedures: local recurrence should be no greater and survival no worse than with amputation; the procedure should not delay adjuvant therapy; reconstruction should be enduring and not associated with a large number of local complications; and function of the limb should approach that obtained by amputation [218]. Other than tumor curettage surgery, tumor resection is the preferred option for tumor-induced osteomalacia [39]. Benign tumors of the brachial plexus were treated with microsurgical techniques and malignant tumors with large extirpations in a clinical surgical study [221]. Secondary brachial plexus tumors were treated with neurolysis to palliate pain [221]. Surgery improves the quality of life in malignant brachial plexus tumor cases [221]. Primary tumors of the spine are best treated with an en bloc resection to minimize risk of local and distant metastases [227].

Adjuvant and Systemic Therapy: The role of chemotherapy for synovial sarcoma remains controversial with survival benefits observed in certain populations [36]. High-grade malignant primary bone lesions typically undergo neoadjuvant therapies and surgery [41]. Low-grade malignant primary bone lesions are usually managed with surgery alone [41]. Radiation therapy, either preoperatively or postoperatively, is recommended for high-grade, large, and deep-seated soft-tissue sarcoma lesions [72]. Chemotherapy has marginal efficacy in the management of localized soft-tissue sarcoma disease and is considered in high-risk patients with large, deep, and high-grade tumors [72]. The effectiveness of the chemotherapy regimen correlates with the extent of tumor necrosis [122]. In spite of the advances of adjuvant therapy, surgical removal of the tumor still remains the most effective treatment for Ewing's sarcoma of the hand [143]. Surgical excision remains the primary treatment for invasive skin tumors of the hand, though nonsurgical options exist for unresectable or low-grade cancers [154]. Multidrug neoadjuvant chemotherapy is usually initiated as appropriate after histologic diagnosis and staging for malignant bone tumors [218]. Neoadjuvant therapy helps control systemic disease by attacking micrometastases, dramatically increasing overall survival rates [218]. Neoadjuvant therapy "sterilizes" the reactive zone around the tumor by destroying microscopic disease at the periphery of the primary lesion, thus facilitating resection [218]. Three treatment modalities are reported to be of value in the management of fibromatosis: surgery, irradiation, and drug treatment (chemotherapy or hormone therapy) [220]. Margins of 1–2 cm of soft tissue or a thick fascial plane are accepted as adequate surgical management for fibromatosis without the necessity of irradiation [220]. Preoperative irradiation offers potential benefit for tumors encompassing the brachial plexus [220].

Multidisciplinary and Emerging Approaches: The field of musculoskeletal tumor surgery continues to advance through multidisciplinary collaboration, with recent literature highlighting the importance of aggressive surgical resection for non-sacral pelvic Ewing sarcoma [203]. Recent literature highlights the prognostic value of preoperative imaging in osteosarcoma and chondrosarcoma [203]. Recent literature highlights the role of percutaneous sclerotherapy for benign bone tumors [203]. Proper care for a patient with a spine tumor requires a multidisciplinary team approach with continued follow-up and optimization of the care as the patient’s needs can be dynamic and needs to be individualized [227].

Complications¶

Nerve Tumors¶

Neurofibromas: These lesions require prolonged monitoring due to the risk of recurrence [40]. For unresectable neurofibromas, epineurotomy is performed to prevent further deterioration [12]. Schwannomas: Enucleation is the standard procedure for resectable schwannomas [12]. A cellular schwannoma of the hand may demonstrate a long clinical course, with one case showing 60 years of stability without significant growth or metastases [77]. Glomus Tumors: Recurrent symptoms following excision are most likely attributable to a previously undetected tumor [20]. Specifically, recurrence from digital glomus tumors is often caused by small, synchronous satellite lesions missed during the original surgery rather than inadequate resection [237]. Hamartomas: Small areas of residual, inaccessible fibrous hamartoma tumor may be neglected because long-term follow-up monitoring indicates that progression and functional compromise do not appear to occur [46]. Similarly, fibrolipomatous hamartoma of the median nerve showed unchanged size and no symptoms at 30 months of follow-up [62, 63].

Bone Tumors¶

Benign Lesions: Benign bone tumors in children may simulate malignancy and require referral to an orthopaedic oncologist [24]. The benign clinical course of chondromyxoid fibroma makes differentiation from chondrosarcoma important [75]. Giant-Cell Tumors: A secondary malignant tumor can develop as long as twenty-five years after the initial presentation of a benign giant-cell tumor [68]. Secondary malignant giant-cell tumors of bone have occurred in patients who received therapeutic irradiation four to thirty-nine years earlier for a benign giant-cell tumor [252]. At a mean follow-up period of 54 months, there were no local recurrences for giant cell tumors of the hand treated with curettage, cryosurgery, and cementation [64]. Treatment Outcomes: No tumour recurrence was found during more than 10 years of follow-up for benign bone tumours treated with sintered bone implantation [47]. For enchondromas treated with bone substitute, bone regained normal X-ray appearance and full range of motion with normal hand function were restored by 9 months [50]. At one year of follow-up, there was no local recurrence for chondrome des parties molles de la main [48].

Soft Tissue and Malignant Tumors¶

Sarcomas and Malignant Nerve Tumors: Long-term clinical follow-up of 10 years is advised for soft-tissue sarcomas of the hand, as their smaller size should not be deceptive [11]. Long-term follow-up is also indicated for granular cell schwannoma of the hand due to reported cases of malignant or locally invasive behavior [52]. The natural history of epithelioid sarcoma in the hand is characterized by recurrence and repeated operations, often with the diagnosis being made only after three or four excisions or after death [213]. After aggressive surgical management with negative margins, 71% of patients with primary epithelioid sarcoma of the hand were alive without evidence of disease at the last followup [71]. The 5- and 10-year survivorship for primary epithelioid sarcoma of the hand after aggressive surgical management was 85% [71]. Treatment failures for primary epithelioid sarcoma of the hand included one local recurrence, four regional metastases, and five distant metastases [71]. Five patients died secondary to disease in a cohort of 28 patients treated for primary epithelioid sarcoma of the hand [71]. After 2 years of follow-up, there is no evidence of local recurrence or metastatic dissemination for epithelioid sarcoma in the median nerve of the hand [194]. Early diagnosis and radical surgical excision offer the best chance of long-term survival from neurofibrosarcoma of the upper extremity [74]. Five patients with neurofibrosarcoma of the upper extremity died of metastases an average of 3 years after diagnosis [74]. One patient with neurofibrosarcoma of the upper extremity remains alive 18 years after upper extremity amputation despite an early local recurrence [74]. Benign and Low-Grade Lesions: The recidivism of indolent myxomas of the hand suggests that long-term follow-up is required to monitor for local recurrence [189]. A 5-year follow-up showed no recurrence for desmoid tumor of the hand [191]. No recurrence was observed at follow-up 1 to 8 years after surgery for subscapular elastofibroma [193]. With both patients at 5-year follow-up, recurrence is unlikely for Merkel Cell Tumor of the hand [188]. Eighteen patients (72%) with pediatric lipofibromatosis had regrowth of their tumor or had persistent disease [246]. No tumor of pediatric lipofibromatosis has given rise to metastases [246]. Since recurrence of extraskeletal soft tissue chondrosarcoma has been reported as early as two months and as late as fifteen years after the primary operation, all patients should be carefully followed [76]. Metastatic and Cutaneous Malignancies: Short-term prognosis for metastatic tumours of the hand was poor with a median survival of 7 months [54]. In two patients, hand metastases were the first sign of malignant disease [54]. Although the average time from diagnosis to death for metastatic tumors of the hand is usually short, prolonged survival is possible [219]. In patients with an apparently isolated metastatic lesion to the hand, wide excision may produce long disease-free intervals [219]. A history of extensive recurrent disease well removed from the primary tumor is associated with a poor prognosis for cutaneous metastatic breast carcinoma of the hand [65]. Eleven patients (22%) had a recurrence of squamous cell carcinoma of the hand, mostly within 15 months [224]. In eight patients (67%) with squamous cell carcinoma of the hand, metastases followed disease recurrence [224]. Four patients died from metastatic disease at five years after operation for squamous cell carcinoma of the hand [224]. Three patients died from metastatic disease in the sixth, seventh, and eighth years after operation for squamous cell carcinoma of the hand [224].

Other Considerations¶

Diagnostic Pitfalls: Diagnostic delay or inappropriate treatment may result if patients with tumors masquerading as hematomas do not receive appropriate followup, biopsy, or referral whenever the diagnosis is in doubt [28].

Recovery¶

Light activity (weeks): The evidence base does not provide specific week-based timelines for the resumption of desk work, driving, or light activities of daily living following hand tumor excision. Consequently, no standard week range can be established for this phase based on the provided data.

Full activity (months): Specific month-based ranges for the return to manual work, sport, or full range of motion and strength are not defined in the available evidence. While functional recovery is documented in specific cases, such as grip strength recovering to 70% of the normal side in a patient with primary osteogenic sarcoma of a finger proximal phalanx [56], or range of motion scoring as normal in a patient with deforming chondrosarcoma secondary to long-term enchondromatosis [15], these individual outcomes do not establish a generalized timeline for full activity resumption.

Complete recovery / outcome plateau (months): The provided evidence does not specify a standard month range for the stabilization of pain, strength, and final functional outcomes.

Rehabilitation protocol: No specific physical therapy phasing, immobilisation duration, weight-bearing or range of motion progression schedules, or sling/brace removal timing are detailed in the evidence. The focus of the available data is on surgical management and long-term surveillance rather than structured rehabilitation protocols.

Functional milestones: Validated patient-reported outcome measure trajectories, such as Constant, ASES, or WOMAC scores, are not reported in the evidence. Specific functional benchmarks include 30° motion in both radial and palmar abduction and 90° flexion-extension at the interphalangeal joint of the thumb for a patient with giant cell tumor of the thumb metacarpal [33]. Additionally, all patients with enchondromas of the hand treated with curettage and cemented internal fixation returned to their presurgical functional capability within 4 weeks [177].

Other Considerations: Long-term clinical follow-up is a critical component of post-operative management for various hand tumors. For soft-tissue sarcoma of the hand, 10 years of follow-up is advised [11], while regular oncologic follow-up is necessary for primary malignant bone tumors [35]. Low-grade myofibroblastic sarcoma of the distal phalanx requires a long clinical follow-up period with imaging studies [14]. Extraskeletal soft tissue chondrosarcoma patients must be carefully followed because recurrence has been reported as early as two months and as late as fifteen years after the primary operation [76]. Granular cell schwannoma also requires long-term follow-up due to reported cases of malignant or locally invasive behavior [52].

For benign lesions, longitudinal radiographs allow observation of the timing of first appearance and potential resolution for benign bone tumors in asymptomatic children [13]. Small areas of residual inaccessible tumor in fibrous hamartoma of infancy may be neglected because progression and functional compromise do not appear to occur in long-term follow-up monitoring [46]. No local recurrence was observed at one year for chondrome of the soft tissues of the hand [48], and no recurrence of fibrous hamartoma of the wrist was noted at 18 months following resection [49]. At a mean follow-up period of 54 months, there were no local recurrences for giant cell tumor of the hand treated with curettage, cryosurgery, and cementation [64]. Similarly, none of the patients with enchondromas of the hand treated with curettage and cemented internal fixation had local tumor recurrence at the most recent follow-up [177].

Specific case outcomes include a patient with well-differentiated liposarcoma in the digit showing no evidence of local recurrence or metastasis and no loss of hand function at 5-year follow-up [60]. A fibrolipomatous hamartoma of the median nerve remained unchanged in size and the patient was without symptoms at 30 months [62, 63]. A cellular schwannoma of the hand demonstrated a long clinical course of 60 years with a lack of any significant growth or evidence of metastases [77]. Postoperatively, grip strength and movements were normal for segmental neurofibromatosis of the upper extremity, and no tumour recurrence was found at 1-year follow-up [181]. A patient with subungual keratoacanthoma in the hand was free of disease at 5½ years follow-up [273].

Prognostic considerations vary significantly by tumor type. The prognosis for metastatic basal cell carcinoma of the upper extremity is poor, with a median survival time of 10 to 14 months from the time of diagnosis of metastatic disease [245]. The prognosis for 5-year disease-free survival in sweat gland carcinoma of the hand is less than 30% [269]. Generally, the prognosis for patients with hand metastases is poor, with 85% dead at 1 year and 96% at 2 years [271]. A history of extensive recurrent disease well removed from the primary tumor is associated with a poor prognosis in metastatic breast carcinoma of the hand and upper extremity [65]. In a patient with congenital primitive neuroectodermal tumor of the hand, intracranial metastases occurred 7 months later despite initial response, and the patient died 15 months after presentation [259].

Surgical outcomes for malignant tumors include 71% of patients with epithelioid sarcoma of the hand being alive without evidence of disease at the last follow-up after aggressive surgical management with negative margins [71]. The 5- and 10-year survivorship for epithelioid sarcoma of the hand after aggressive surgical management was 85% [71]. Eccrine tumours of the hand should be carefully reviewed on a long-term basis when established histologically and completely excised [73]. The benign clinical course of chondromyxoid fibroma of bone makes differentiation from chondrosarcoma important [75]. The ossified lipoma is a rare entity of benign tumors with a favorable prognosis, although its diagnosis can be delayed due to its indolent nature and slow evolution [274].

Surveillance strategies must account for specific tumor behaviors. Neurofibroma excision carries a higher risk of nerve dysfunction and pain worsening [1]. Enucleation is performed for resectable tumours such as schwannomas [12], while epineurotomy is performed for unresectable tumours such as neurofibromas to prevent further deterioration [12]. The possibility of sequential appearance of neurilemomas in the upper extremity indicates that routine follow-up examination of patients is important [254]. Multiple schwannomas of the ulnar nerve may grow over time and appear as recurrences, although they are actually multicentric [277]. Eight recurrences were noted in 96 patients with giant cell tumors of the synovial sheaths of the hand, occurring in seven patients with a mean recurrence delay of 2.75 years [275]. A multi-disciplinary approach with long-term follow-up is recommended to monitor for local recurrence in intraosseous myoepithelioma of the hand [182].

Key Evidence¶

[L5] Definitive diagnosis of nerve tumors requires pathologic evaluation as MRI is not definitive; schwannomas treated by excision typically have few long-term deficits, whereas neurofibroma excision carries a higher risk of nerve dysfunction and pain worsening. [1] (10.1016/j.jhsa.2010.07.001)
[L4] Benign tumors that are asymptomatic should be observed, while biopsies should be performed for tumors suspected to be malignant before starting definitive treatment. [2] (10.2106/jbjs.rvw.24.00019)
[L5] History, clinical examination, imaging and histopathological examination are crucial in diagnosis of these rare tumours. [3] (10.1142/s021881041772039x)
[L5] Surgical excision is the primary treatment for symptomatic tumors, with recurrence rates being rare following complete removal. [5] (10.1016/s0749-0712(02)00070-7)
[L4] Updated knowledge and understanding nature and demographic characteristics of the tumorous conditions of the hand is crucial for an accurate diagnosis and appropriate treatment. [6] (10.1177/1558944716660555ha)
[L5] Once diagnosed, cases should be discussed at a tumor board meeting, and surgery is an essential component of treatment that must be done at the appropriate time after potential systemic or local treatment. [7] (10.1016/j.otsr.2024.104058)
[L5] Treatment options include surgical excision, destructive techniques, and medical therapies, with cure rates varying by tumor type, size, and histologic subtype. [9] (10.1016/j.hcl.2004.03.018)
[L3] The absence of disease on MRI should not be used as the sole criterion in determining whether a repeat resection should be performed. [10] (10.1016/j.jhsa.2010.05.009)
[L4] Even with adequate oncologic treatment, long-term clinical follow-up (10 years) is advised as they should not be deceived by their smaller size. [11] (10.5435/jaaos-d-20-00434)
[L5] Surgical management is similar across types, with enucleation performed for resectable tumours (schwannomas) and epineurotomy for unresectable tumours (neurofibromas) to prevent further deterioration. [12] (10.1016/s1297-3203(03)00038-6)
[L4] Longitudinal radiographs allowed observation of the timing of the first appearance and the potential for resolution for each tumor type. [13] (10.2106/jbjs.20.00999)
[L5] The treatment of this tumor should include a simple local excision with free surgical margins and a long clinical follow-up period with imaging studies. [14] (10.1016/j.jhsa.2004.05.003)
[L5] Range of motion of the nonamputated fingers was scored as normal and the patient showed a high degree of satisfaction. [15] (10.1016/j.main.2004.05.006)
[L4] The authors propose a classification scheme for vascular bone tumors that includes hemangioma, hemangioendothelioma, angiosarcoma, and their epithelioid variants. [16] (10.1007/s00256-012-1510-6)
[L5] [18] (10.1016/j.injury.2019.10.054)
[L5] Soft-tissue tumors in children are a heterogeneous group of lesions that may be benign or malignant; a thorough history and physical examination, followed by appropriate imaging studies, can establish a correct diagnosis and help determine appropriate treatment recommendations. [19] (10.5435/00124635-200309000-00006)
[L4] The authors believe that recurrent symptoms following tumor excision most likely are due to the presence of a previously undetected tumor. [20] (10.1016/s0363-5023(79)80076-3)
[L5] The international classification distinguishes between vascular tumors and malformations, which have radically opposite evolutive profiles. [21] (10.1016/j.main.2007.10.004)
[L5] This new edition provides an updated classification scheme and essential diagnostic criteria, including newly recognized entities, subtypes, and reclassified tumors. [22] (10.1097/pap.0000000000000284)
[L5] Most benign bone tumors in children have characteristic clinical and radiographic presentations that allow for diagnosis without biopsy, though some may simulate malignancy and require referral to an orthopaedic oncologist. [24] (10.5435/00124635-199911000-00004)
[L5] [27] (10.1016/s0749-0712(21)00048-2)
[L4] Diagnostic delay or inappropriate treatment may result if patients do not receive appropriate followup, biopsy (usually open), or referral whenever the diagnosis is in doubt. [28] (10.1097/blo.0b013e31815953a7)
[L5] Despite the benign nature of these tumors, multidisciplinary care is needed to provide combined treatment options. [30] (10.5435/00124635-200804000-00002)
[L5] A preoperative diagnosis based on radiographic and MRI characteristics of these tumors may prevent unnecessary biopsy, although meticulous resection is advocated to prevent recurrence. [32] (10.1016/j.jhsa.2006.04.011)
[L5] The patient had 30° motion in both radial and palmar abduction and 90° flexion-extension at the interphalangeal joint of the thumb. [33] (10.1016/s0363-5023(84)80220-8)
[L5] Complete surgical excision with clear margins is the only curative treatment for malignant peripheral nerve sheath tumours, though prognosis remains poor, particularly for patients with neurofibromatosis. [34] (10.1177/17531934241238739)
[L4] Regular oncologic follow-up is necessary. [35] (10.1016/s0363-5023(89)80054-1)
[L4] Wide surgical excision remains the standard of care for definitive treatment with adjuvant radiation utilized for larger and deeper lesions, while the role of chemotherapy remains controversial with survival benefits observed in certain populations. [36] (10.3390/curroncol28030177)
[Paper] [37] (10.1051/sicotj/2017041)
[L4] Other than tumor curettage surgery, tumor resection is the preferred options for these tumors. [39] (10.1186/s12891-015-0496-3)
[L4] Neurofibromas require prolonged monitoring due to possible recurrence. [40] (10.1016/j.hansur.2016.10.097)
[L3] The Society suggests that the biopsy should be planned as carefully as definitive surgery and that patients should be referred to a treating center prior to biopsy if the institution is not prepared to perform accurate diagnostic studies or proceed with definitive treatment. [43] (10.1097/01.blo.0000229299.36969.b5)
[L4] MRI was able to identify tumour type and determine tumour resectability in 80% of cases, while ultrasound and MRI confirmed the neural nature of tumours in 90% of cases. [44] (10.1016/j.hansur.2016.10.098)
[L5] Surgical excision is the treatment of choice and the prognosis is excellent. [45] (10.1016/0363-5023(89)90028-2)
[L5] Small areas of residual inaccessible tumor may be neglected, because progression and functional compromise do not appear to occur in long-term follow-up monitoring. [46] (10.1016/s0363-5023(97)80139-6)
[L4] No tumour recurrence has been found during more than 10 years of follow-up. [47] (10.1016/s0266-7681(99)90055-4)
[L5] At one year of follow-up, there was no local recurrence. [48] (10.1016/j.main.2004.04.008)
[L5] At 18 months following resection, there is no evidence of recurrence of the tumor. [49] (10.1016/s0363-5023(86)80235-0)
[L4] Bone regained normal X-ray appearance by 9 months, and the full range of motion and normal function of the hand were restored. [50] (10.1016/s0266-7681(97)80272-0)
[L4] Long-term follow-up is indicated due to a few cases of malignant or locally invasive behavior reported. [52] (10.1016/s0363-5023(80)80043-8)
[L4] Early surgical treatment may limit the growth of large lesions and facilitate future surgery, though longer-term follow-up is needed to confirm the absence of recurrence and malignant transformation. [53] (10.1016/j.jhsa.2018.02.010)
[L4] Short-term prognosis was poor with a median survival of 7 months, and in two patients, hand metastases were the first sign of malignant disease. [54] (10.1016/s0266-7681(96)80063-5)
[L4] In most patients, the resectability of the tumor, its type and benignity can be predicted based on medical imaging. [55] (10.1016/j.hansur.2016.08.001)
[L5] Grip strength recovered to 70% of the normal side. [56] (10.1053/jhsu.2001.28945)
[L4] The authors suggest that the biopsy should be planned as carefully as definitive surgery and that patients should be referred to a treating center prior to biopsy if the institution is not prepared to perform accurate diagnostic studies or proceed with definitive treatment. [57] (10.2106/00004623-198264080-00002)
[L4] This review summarizes 17 years' experience with 516 vascular tumors of the upper extremity, classifying 90% as either hemangiomas or malformations. [58] (10.1016/s0749-0712(21)00050-0)
[L5] Conservative management of these tumors could be justified since most patients do well, but surveillance of these patients is warranted since early identification of recurrence or metastasis may improve the outcome. [59] (10.1016/s0363-5023(86)80024-7)
[L5] At the 5-year follow-up, the patient showed no evidence of local recurrence or metastasis, with no loss of hand function. [60] (10.1142/s242483551872027x)
[L4] The absence of neurofibroma and NF2 criteria makes schwannomatosis a diagnosis of exclusion. [61] (10.1016/j.hansur.2017.07.001)
[L5] At follow-up 30 months later, the size of the tumour was unchanged and the patient without symptoms. [62] (10.1016/0266-7681(87)90018-0)
[L5] At follow-up 30 months later, the size of the tumour was unchanged and the patient without symptoms. [63] (10.1016/0266-7681_87_90018-0)
[L4] At a mean follow-up period of 54 months there were no local recurrences. [64] (10.1053/jhsu.2001.22525)
[Case_report] A history of extensive recurrent disease well removed from the primary tumor is associated with a poor prognosis. [65] (10.1016/j.jhsa.2006.10.022)
[L5] [67] (10.1016/s0363-5023(83)80277-9)
[Case_report] This case demonstrates that a secondary malignant tumor can develop as long as twenty-five years after the initial presentation of a benign giant-cell tumor, a latency period longer than previously recorded. [68] (10.2106/00004623-198971050-00019)
[L5] [69] (10.5435/jaaos-d-23-01020)
[L5] [70] (10.5435/00124635-200009000-00003)
[L4] [71] (10.1097/01.blo.0000150317.50594.96)
[L5] [72] (10.2106/jbjs.21.00196)
[L5] When established histologically and completely excised, these tumours should be carefully reviewed on a long-term basis. [73] (10.1016/0266-7681(87)90075-1)
[L4] [74] (10.1016/s0363-5023(10)80153-4)
[L5] The long clinical course of this case (60 years), and the lack of any significant growth or evidence of metastases apparently support the concept of a cellular schwannoma, as suggested by Woodruff and Fletcher. [77] (10.1016/s0363-5023(89)80101-7)
[L4] Awareness and understanding of tumor characteristics may help physicians with diagnosis and treatment. [79] (10.1177/1558944720928499)
[L5] Tumor-like conditions of the hand and upper extremity typically present as masses and can be confused with more serious conditions; diagnosis often relies on clinical examination and imaging, with excisional biopsy needed for definitive diagnosis to guide treatment. [80] (10.1016/j.jhsa.2017.09.012)
[L5] The tumor presented with characteristics similar to other intraneural tumors (slow growing, without symptoms of neuropathy) making preoperative diagnosis difficult. [82] (10.1016/s0363-5023(96)80120-1)
[L4] Patients with GCT should be followed indefinitely, and referred promptly if new symptoms, particularly pain, emerge. [83] (10.1302/0301-620x.104b12.bjj-2022-0401.r1)
[L4] Diagnosis and management of neurofibromas affecting digital nerves are both difficult and demanding. [85] (10.1016/s0266-7681(97)80019-8)
[L5] Clinical and pathologic diagnosis may be difficult. [88] (10.1016/s0363-5023(87)80073-4)
[Case_report] It shows that a lower threshold for further workup in patients with atypical signs and symptoms allows for the potential to identify potential malignancy earlier. [89] (10.1177/15589447231151260)
[L5] The case highlights the difficulties encountered in clinical practice to diagnose such tumors due to shared clinical, epidemiological, and imaging characteristics with other soft tissue tumors. [91] (10.1016/j.hansur.2019.05.001)
[L4] The suspicion of glomus tumor is based on clinical examination, primarily searching for localized pain. [93] (10.1016/j.main.2005.12.007)
[L5] Physical diagnosis must include a complete history that elicits duration of symptoms, as well as accurate identification of both size and location of a particular mass. [94] (10.1016/s0749-0712(21)00042-1)
[L5] [95] (10.1016/j.jhsa.2010.08.015)
[L5] [96] (10.2106/00004623-199304000-00019)
[L5] Awareness of this uncommon tumor in adult patients is essential to prevent mistaken diagnosis and subsequent extensive workup. [100] (10.1007/s11552-014-9729-4)
[L3] Glomus tumor should be included in the differential diagnosis in neurofibromatosis patients who present with a painful lesion of the hand or finger. [101] (10.1016/j.jhsa.2013.05.025)
[L5] It must be considered in the differential diagnosis of tumors of the extremities. [102] (10.1016/j.main.2012.07.007)
[L4] The clinical diagnosis of the tumorous condition was accurate in 94% of the cases. [103] (10.1016/s0363-5023(85)80124-6)
[Case_report] Excision of the tumor relieves symptoms in most patients, but clinicians should search for synchronous glomus tumors during excision to avoid recurrence or persistence of symptoms due to undiscovered multiple lesions. [104] (10.1016/j.jhsa.2013.07.024)
[L4] Diagnosis of MPNST can be challenging due to the rarity of these tumours and unreliable imaging, making a high index of suspicion paramount for early diagnosis and treatment. [107] (10.1177/1753193408092039)
[L4] Awareness of tumor features may help physicians with diagnosis, and awareness of recurrence rates is important when counseling patients. [109] (10.1177/1558944720922921)
[L4] [111] (10.1016/s1297-3203(00)73487-1)
[L5] The current concepts and difficulties in diagnosing these tumours are discussed. [112] (10.1016/j.jhse.2007.04.001)
[L5] [114] (10.2106/00004623-199601000-00018)
[L4] MRI confirmed the diagnosis and extent of the lesions. [120] (10.1016/j.hansur.2017.10.207)
[L4] For all tumor types, MRI, CT, and plain radiographs were frequently rated as helpful studies. [121] (10.5435/jaaosglobal-d-25-00015)
[L5] The effectiveness of the chemotherapy regimen correlates with the extent of tumor necrosis. [122] (10.5435/jaaos-21-08-480)
[L4] Magnetic resonance imaging is the preferred modality for the staging of soft tissue tumors and for evaluating patients for tumor recurrence. [123] (10.1016/0730-725x(88)90397-9)
[L4] The prognosis is extremely poor, with survival generally remaining under 6 months regardless of the aggressiveness of the treatment. [125] (10.1016/s1297-3203(00)73461-5)
[L5] Treatment at a specialized cancer center has been proven to be effective as it improves overall survival and reduces local recurrences. [126] (10.1016/j.otsr.2021.103162)
[Case_report] In the setting of nonmetastatic disease, amputation is the treatment of choice; however, this aggressive tumor uniformly has a poor survival rate. [132] (10.1177/1558944717702466)
[L5] Imaging examinations, particularly ultrasound and MRI, should be performed to support the diagnosis. [133] (10.1016/j.main.2005.01.005)
[L5] Treatment options range from observation and nonsurgical methods to surgical excision, with recurrence rates varying by lesion type and treatment modality. [134] (10.1016/s0749-0712(21)00039-1)
[L4] Specific characteristics of the tumor may increase chances of recurrence, while the technique of tumor excision did not have a major role in outcome. [135] (10.1016/j.jhsa.2013.08.090)
[L4] MR imaging has become the modality of choice in evaluating synovial tumors and tumor-like lesions, because with it a presumptive diagnosis can be made in most cases. [136] (10.1007/s003300000759)
[L4] Our experience with multimodal therapy demonstrating a 91% survival rate, with an 8% rate of local recurrence, matches the best of any currently reported patient series. [138] (10.1016/s0363-5023(88)80067-4)
[Case_report] Preoperative diagnosis by MRI and selection of the appropriate surgical approach are important in these cases because they give clinicians the best chance to separate the nerve from the tumor and decrease the risk of postsurgical complications. [139] (10.1016/j.jhsa.2008.05.033)
[Case_report] Surgical resection is the main treatment and the prognosis is poor. [140] (10.1186/s12891-020-03429-5)
[L5] Surgical resection of the tumor is the treatment of choice. [141] (10.1016/j.hansur.2019.09.002)
[L4] Curettage was found to be ineffective as a method of treatment; 13 of 15 tumors treated by curettage recurred. [142] (10.1016/s0363-5023(80)80042-6)
[L5] In spite of the advances of adjuvant therapy, surgical removal of the tumor still remains the most effective treatment. [143] (10.1016/s0363-5023(09)90033-8)
[L5] Fibro-osseous and cystic tumors of bone are common lesions with distinct clinical, radiologic, and pathologic features that aid in their classification. [144] (10.1016/j.mpdhp.2020.07.003)
[L5] Magnetic resonance imaging was helpful in delineating the lesion, but could not identify its nature. [145] (10.1016/s0363-5023(97)80177-3)
[L5] The repeat MRI did not show recurrence. [146] (10.1177/1753193411421720)
[L2] Although no major differences between MRI and CT were demonstrable in the preoperative evaluation of soft-tissue tumors, MRI is to be preferred in view of its better spatial orientation and the sharper contrast it gives between tumor and adjacent tissues. [148] (10.1007/bf00395801)
[L4] MRI should be used as a complementary method, while angiography remains necessary for specific cases involving highly vascularized tumors. [149] (10.1007/bf00395793)
[L5] Treatment is complete surgical excision, which is uniformly curative, as this tumor has never been reported to metastasize. [150] (10.1007/s11552-008-9143-x)
[Case_report] They recommend using high-resolution MRI and ensuring complete excision to decrease recurrence rates. [151] (10.1111/sae.12041)
[L2] No significant survival benefit was identified for individual tumors. [152] (10.1016/j.jhsa.2005.11.005)
[L4] Good results can be obtained with surgical treatment, but, as with large tumours located elsewhere, these require a thorough pre-operative assessment. [153] (10.1016/j.jhsb.2005.05.002)
[L5] Surgical excision remains the primary treatment for invasive tumors, though nonsurgical options exist for unresectable or low-grade cancers. [154] (10.5435/jaaos-d-14-00040)
[L2] Most benign upper limb tumours are indicated for surgery, however, degree of improvement varies according to tumour pathology and location, but not to size. [155] (10.1016/j.bjps.2016.12.010)
[L5] While the Musculoskeletal Tumor Society system remains intuitive for surgeons, the American Joint Committee on Cancer staging system has widespread acceptance due to its multidisciplinary nature and ability to facilitate more nuanced prediction of prognosis. [156] (10.5435/jaaos-d-17-00055)
[L4] The optimum treatment for Grade III lesions and patients with local recurrence is unclear. [157] (10.1097/01.blo.0000160024.06739.ff)
[L5] The classification of these fatty tumors and their management are presented to increase awareness of the benign nature of these tumors despite their rapid growth and to demonstrate that complete surgical excision should be done to prevent local recurrence. [158] (10.1016/s0363-5023(05)80051-6)
[L5] These two types of nerve tumour are commonly associated in Von Recklinghausen's, but have not been reported in association outside this syndrome. [159] (10.1016/0266-7681(88)90063-0)
[L4] The diagnosis relies on radiological examination, ideally supplemented by MRI, and is confirmed by histological examination. [161] (10.1016/j.main.2005.08.014)
[L4] There are three pathologic types—solid, cavernous, and venous type tumors. [162] (10.1016/s0363-5023(87)80223-x)
[L5] The early treatment that will increase survival prospects and may improve the chances of cure is rarely achieved. [163] (10.1016/0266-7681(90)90003-m)
[L5] Twenty-four months after surgery the patient is free of tumor clinically, on computed tomography scan and on MRI. [164] (10.1053/jhsu.2003.50056)
[L4] A high level of suspicion and histological study are required when there is an underlying history of malignancy, and MRI may be more useful than plain x-rays to identify bone and soft tissue extensions. [165] (10.1016/j.jhsa.2020.03.014)
[L5] Local excision of the tumour appears to be a satisfactory method of treatment. [166] (10.1016/s0266-7681(97)80269-0)
[L5] Vascular tumors of the hand are classified into benign, congenital, and malignant categories, with treatment ranging from observation and medical therapy to surgical excision or amputation depending on the specific lesion type and patient factors. [167] (10.1016/j.hcl.2004.03.009)
[L4] Improved investigation techniques, particularly the bone scan and tomography, have improved the diagnostic accuracy, and a CT scan is useful for precise localization of the lesion. [168] (10.1016/0363-5023(92)90349-t)
[L5] Plain radiograph and classic clinical presentation may not be always of diagnostic help so histopathological confirmation is must. [169] (10.1142/s2424835516720140)
[L3] Patients undergoing treatment for primary malignant bone tumors at high-volume facilities experience a slightly better overall survival than those receiving treatment at low-volume facilities. [171] (10.1097/corr.0000000000001034)
[L5] The article provides an update on current management of primary malignant and metastatic tumors of the hand, emphasizing the importance of optimal biopsy, negative surgical margins, and the roles of adjuvant radiotherapy and chemotherapy. [172] (10.1016/j.jhsa.2010.08.014)
[L4] Ultrasound is an acceptable imaging modality for diagnosis, and surgical excision is the treatment of choice. [173] (10.1016/j.jhsa.2016.10.003)
[L4] All patients returned to their presurgical functional capability within 4 weeks, and none had local tumor recurrence at the most recent follow-up. [177] (10.1053/jhsu.2002.34369)
[L4] Magnetic resonance imaging in the coronal plane accurately defined the intramedullary abnormality in every patient and provided greater interpretive certainty than computerized tomography or scintigraphy in defining the true proximal and distal extent of a tumor. [178] (10.2106/00004623-198668060-00003)
[L4] Soft tissue radiographic techniques and mammographic films were an extremely valuable diagnostic tool, although computed tomography and magnetic resonance imaging may be necessary in some cases. [179] (10.1016/s0363-5023(98)80062-2)
[L4] Emphasis is placed on balancing tumor recurrence risk and postoperative functionality within the context of histologic diagnoses and surgical approaches. [180] (10.5435/jaaosglobal-d-21-00207)
[L5] Postoperatively, grip strength and movements were normal, and no tumour recurrence was found at 1-year follow-up. [181] (10.1177/1753193413517807)
[L4] A multi-disciplinary approach with long-term follow-up is recommended to monitor for local recurrence. [182] (10.1177/1753193416676229)
[L4] The MRI examinations depicted the tumors in excellent detail and facilitated diagnosis in the two patients with atypical presentation. [183] (10.1016/0363-5023(92)90353-q)
[L5] It is also concerning that MRI did not recognise the intravascular nature of the lesion. [185] (10.1016/j.jhsb.2007.01.011)
[L5] Magnetic resonance imaging features included a high-signal, heterogeneous, well-defined lesion with no connection to adjacent tendons, joints, or bones. [186] (10.1016/j.jhsa.2006.02.003)
[L4] High resolution MR imaging indicated a new (palmar) location of the tumour before the last operation, supporting the hypothesis that a new tumour had developed rather than a recurrence due to incomplete excision. [187] (10.1054/jhsb.1998.0047)
[L4] With both patients at 5-year follow-up, recurrence is unlikely. [188] (10.1016/j.jhsa.2007.12.003)
[L5] The recidivism of these indolent tumors suggests that long-term follow-up is required to monitor for local recurrence. [189] (10.1053/jhsu.2000.9412)
[L5] It should be recognized and treated conservatively by repeated surgical removal rather than radical excision. [190] (10.1016/s0363-5023(77)80120-2)
[L4] A 5-year follow-up showed no recurrence. [191] (10.1016/s0363-5023(83)80064-1)
[L4] The PUMCH classification is based on the anatomic pathology and surgical strategy for HPTs. [192] (10.2106/jbjs.22.00781)
[L4] No recurrence was observed at follow-up 1 to 8 years after surgery. [193] (10.1016/s1058-2746(05)80008-4)
[Case_report] After 2 years of follow-up, there is no evidence of local recurrence or metastatic dissemination. [194] (10.1177/15589447211030689)
[L5] Complete en bloc excision of the lesion has been the recommended treatment for failure of conservative therapy. [195] (10.1016/s1058-2746(97)90009-4)
[L5] The malignant fibrous histiocytoma is an aggressive lesion originating in the tissue histiocyte that contains five cell types and has often been grouped with other sarcomas. [196] (10.1016/s0363-5023(77)80129-9)
[L4] The novel GCT grading system demonstrates excellent interrater agreement and classified more nonrecurrent curetted tumors as low grade, suggesting improved predictive performance compared with the Campanacci classification. [197] (10.5435/jaaosglobal-d-24-00321)
[L5] For benign lesions, prompt and simultaneous management of fracture and tumor with minimally invasive techniques and adjuvants must be used to minimize recurrence and maximize patient recovery. [198] (10.1016/j.injury.2025.112853)
[L4] Confirming PIK3CA mutation status allows for more accurate diagnosis and classification of the disease based on its molecular pathogenesis. [199] (10.1177/1753193418770366)
[L4] Surgical treatment, even of recurrence, should be locally extensive but conservative. [200] (10.1016/s0363-5023(85)80169-6)
[L3] Upfront surgery is not advantageous compared to more conservative treatments such as observation or medical treatment for patients with desmoid tumors. [201] (10.1186/s12891-020-03897-9)
[L4] Surgical management with curettage is the standard of care for symptomatic lesions, with most patients returning to full function after surgery. [202] (10.5435/jaaos-d-15-00452)
[L5] The field of musculoskeletal tumor surgery continues to advance through multidisciplinary collaboration, with recent literature highlighting the importance of aggressive surgical resection for non-sacral pelvic Ewing sarcoma, the prognostic value of preoperative imaging in osteosarcoma and chondrosarcoma, and the role of percutaneous sclerotherapy for benign bone tumors. [203] (10.2106/jbjs.20.01510)
[L5] Tumoral resection can only be justified in this benign context in the case of complete preoperative deficit. [205] (10.1016/j.hansur.2016.05.007)
[L4] The surgeon should consider this potentially fatal entity in the evaluation and management of any long-standing undiagnosed tumor of the hand. [206] (10.1016/s0363-5023(84)80079-9)
[L3] Adjuvant radiation therapy is probably beneficial, but the precise indication for its use is not well defined. [207] (10.2106/00004623-199606000-00007)
[L5] It emphasizes that these lesions are benign and do not metastasize, and that proper clinical, radiologic, and pathologic assessment is mandatory to avoid mutilating surgery. [210] (10.1016/s0749-0712(21)00222-5)
[L4] The danger of the condition lies in the difficulty of diagnosis, with the natural history characterized by recurrence and repeated operations, often with the diagnosis being made only after three or four excisions or after death. [213] (10.1016/s0266-7681(84)80035-2)
[L4] Multidisciplinary management combining neoadjuvant modern targeted oral chemotherapy with staged conservative surgical resection led to successful tumor eradication and near full hand function at 2-year follow-up. [215] (10.1016/j.jhsg.2025.100887)
[L4] Previous studies have regarded persistent bony defects as evidence of complete excision without recurrence, however, in view of the slow asymptomatic growth of this tumour this opinion is incorrect. [216] (10.1016/j.jhsb.2003.08.003)
[L4] Conservative treatment is difficult to conceive due to the narrow spaces of the hand which do not allow for wide resections. [217] (10.1016/s1297-3203(01)00023-3)
[L4] [218] (10.5435/00124635-200301000-00005)
[L4] [219] (10.1016/s0363-5023(87)80299-x)
[L4] [220] (10.1016/0363-5023(94)90283-6)
[L4] [221] (10.1016/s0266-7681(96)80262-2)
[L4] [224] (10.1016/s0363-5023(88)80018-2)
[L4] [230] (10.1530/eor-2025-0149)
[L5] [231] (10.5435/jaaos-d-20-00333)
[Paper] [233] (10.1016/j.hansur.2016.12.010)
[L4] Recurrent symptoms might be attributed to small, synchronous, satellite lesions not detected during the original surgery rather than inadequate resection. [237] (10.1016/j.jhsa.2010.02.019)
[L5] The prognosis for metastatic disease is poor, with a median survival time of 10 to 14 months from the time of diagnosis of metastatic disease. [245] (10.1016/s0363-5023(09)91071-1)
[L4] [246] (10.1097/00000478-200011000-00004)
[L4] [252] (10.2106/00004623-198668070-00016)
[L4] The possibility of sequential appearance of tumors indicates that routine follow-up examination of patients is important. [254] (10.1016/s0363-5023(87)80298-8)
[L5] Despite the tumor's initial response, intracranial metastases occurred 7 months later, and the patient died 15 months after presentation. [259] (10.1016/s0363-5023(97)80140-2)
[L5] The prognosis for 5-year disease-free survival is less than 30%. [269] (10.1016/s0363-5023(89)80018-8)
[L5] The prognosis for patients with hand metastases is generally poor, with 85% dead at 1 year and 96% at 2 years. [271] (10.1016/0363-5023(94)90189-9)
[L5] The patient is free of disease at 5½ years follow-up. [273] (10.1016/0363-5023(89)90074-9)
[L5] The ossified lipoma is a rare entity of benign tumors with a favorable prognosis, although its diagnosis can be delayed due to its indolent nature and slow evolution. [274] (10.1016/j.main.2013.02.013)
[L4] Eight recurrences were noted (8.3%), occurring in seven patients, with a mean recurrence delay of 2.75 years. [275] (10.1016/j.main.2012.10.120)
[L5] These in situ tumors may grow over time and appear as recurrences, although they are actually multicentric. [277] (10.1016/s0363-5023(96)80207-3)

References¶

[1] Nerve Tumors. The Journal of Hand Surgery. 2010. DOI: 10.1016/j.jhsa.2010.07.001

[2] Tumors of the Brachial Plexus. JBJS Reviews. 2024. DOI: 10.2106/jbjs.rvw.24.00019

[3] Ancient Schwannoma of Radial Nerve: A Report of Two Cases. The Journal of Hand Surgery (Asian-Pacific Volume). 2017. DOI: 10.1142/s021881041772039x

[5] Tumors of the perionychium. Hand Clinics. 2002. DOI: 10.1016/s0749-0712(02)00070-7

[6] Deep Palmar Tumors of the Hand. HAND. 2016. DOI: 10.1177/1558944716660555ha

[7] Pediatric soft tissue tumors. Orthopaedics & Traumatology: Surgery & Research. 2025. DOI: 10.1016/j.otsr.2024.104058

[9] Non-melanotic skin tumors of the upper extremity. Hand Clinics. 2004. DOI: 10.1016/j.hcl.2004.03.018

[10] Predictive Value of Magnetic Resonance Imaging in Determining Presence of Residual Disease After Marginal Excision of Unsuspected Soft Tissue Sarcomas of the Hand. The Journal of Hand Surgery. 2010. DOI: 10.1016/j.jhsa.2010.05.009

[11] Soft-tissue Sarcoma of the Hand: Patient Characteristics, Treatment, and Oncologic Outcomes. Journal of the American Academy of Orthopaedic Surgeons. 2021. DOI: 10.5435/jaaos-d-20-00434

[12] Tumeurs multiples des nerfs périphériques : mise au point et revue de la littérature. Chirurgie de la Main. 2003. DOI: 10.1016/s1297-3203(03)00038-6

[13] The Natural History of Benign Bone Tumors of the Extremities in Asymptomatic Children. Journal of Bone and Joint Surgery. 2021. DOI: 10.2106/jbjs.20.00999

[14] Low-grade myofibroblastic sarcoma of the distal phalanx. The Journal of Hand Surgery. 2004. DOI: 10.1016/j.jhsa.2004.05.003

[15] Deforming chondrosarcoma of the fingers secondary to a long term enchondromatosis of 28 years. Chirurgie de la Main. 2004. DOI: 10.1016/j.main.2004.05.006

[16] Vascular bone tumors: a proposal of a classification based on clinicopathological, radiographic and genetic features. Skeletal Radiology. 2012. DOI: 10.1007/s00256-012-1510-6

[17] Orthopaedic Knowledge Update 13 Ebook Without Multimedia. Anatomy, Evaluation, Clinical Examination, and Imaging > Evaluation and Clinical Examination: Current Concepts.

[18] Benign peripheral nerve tumors. Injury. 2019. DOI: 10.1016/j.injury.2019.10.054

[19] Pediatric Soft-Tissue Tumors. Journal of the American Academy of Orthopaedic Surgeons. 2003. DOI: 10.5435/00124635-200309000-00006

[20] Multiple digital glomus tumors. The Journal of Hand Surgery. 1979. DOI: 10.1016/s0363-5023(79)80076-3

[21] Tumeurs et malformations vasculaires des membres. Chirurgie de la Main. 2007. DOI: 10.1016/j.main.2007.10.004

[22] The 2020 WHO Classification of Tumors of Soft Tissue: Selected Changes and New Entities. Advances in Anatomic Pathology. 2020. DOI: 10.1097/pap.0000000000000284

[23] A Lange Medical Book Current Diagnosis Treatment In Orthopedics Fifth Edition. 9Hand Surgery > Image DISORDERS OF THE MUSCULATURE OF THE HAND.

[24] Benign Bone Tumors of Childhood. Journal of the American Academy of Orthopaedic Surgeons. 1999. DOI: 10.5435/00124635-199911000-00004

[25] Chapter 66 Benign Bone Tumors. 2020.

[26] Exam Of The Hand Wrist 2Ed. 1.1 SKELETON OF THE HAND > The osseous skeleton.

[27] MALIGNANT BONY TUMORS OF THE UPPER EXTREMITY. Hand Clinics. 1995. DOI: 10.1016/s0749-0712(21)00048-2

[28] Tumors Masquerading as Hematomas. Clinical Orthopaedics & Related Research. 2007. DOI: 10.1097/blo.0b013e31815953a7

[30] Musculoskeletal Desmoid Tumors. Journal of the American Academy of Orthopaedic Surgeons. 2008. DOI: 10.5435/00124635-200804000-00002

[31] Chapter 65 Evaluation and Treatment of Musculoskeletal Tumors. 2020.

[32] Calcifying Aponeurotic Fibroma of the Hand: Radiologic Differentiation from Giant Cell Tumors of the Tendon Sheath. The Journal of Hand Surgery. 2006. DOI: 10.1016/j.jhsa.2006.04.011

[33] Giant cell tumor of the thumb metacarpal in an elderly patient: A case report. The Journal of Hand Surgery. 1984. DOI: 10.1016/s0363-5023(84)80220-8

[34] An algorithmic approach to the management of peripheral nerve tumours. Journal of Hand Surgery (European Volume). 2024. DOI: 10.1177/17531934241238739

[35] Primary malignant bone tumors of the hand. The Journal of Hand Surgery. 1989. DOI: 10.1016/s0363-5023(89)80054-1

[36] Synovial Sarcoma: A Clinical Review. Current Oncology. 2021. DOI: 10.3390/curroncol28030177

[37] Giant cell tumor of bone revisited. SICOT-J. 2017. DOI: 10.1051/sicotj/2017041

[39] Surgical treatment of tumor-induced osteomalacia: a retrospective review of 40 cases with extremity tumors. BMC Musculoskeletal Disorders. 2015. DOI: 10.1186/s12891-015-0496-3

[40] Multiple tumours of the peripheral nerves: Classification predicts outcomes. Hand Surgery and Rehabilitation. 2016. DOI: 10.1016/j.hansur.2016.10.097

[41] Chapter 67 Malignant Primary Bone Tumors. 2020.

[42] Skin Tumors of the Hand and Upper Extremity. 2021.

[43] THE CLASSIC: The Hazards of Biopsy in Patients with Malignant Primary Bone and Soft-Tissue Tumors. Clinical Orthopaedics and Related Research. 2006. DOI: 10.1097/01.blo.0000229299.36969.b5

[44] The role of imaging in single benign peripheral nerve tumours: A practical review for surgeons. Hand Surgery and Rehabilitation. 2016. DOI: 10.1016/j.hansur.2016.10.098

[45] Intravenous pyogenic granuloma of the hand. The Journal of Hand Surgery. 1989. DOI: 10.1016/0363-5023(89)90028-2

[46] Fibrous hamartoma of infancy in the hand: A case report. The Journal of Hand Surgery. 1997. DOI: 10.1016/s0363-5023(97)80139-6

[47] Sintered Bone Implantation for the Treatment of Benign Bone Tumours in the Hand. Journal of Hand Surgery. 1999. DOI: 10.1016/s0266-7681(99)90055-4

[48] Chondrome des parties molles de la main. Un nouveau cas. Chirurgie de la Main. 2004. DOI: 10.1016/j.main.2004.04.008

[49] Fibrous hamartoma of the wrist in infancy. The Journal of Hand Surgery. 1986. DOI: 10.1016/s0363-5023(86)80235-0

[50] Treatment of Enchondromas of the Hand with Bone Substitute. Journal of Hand Surgery. 1997. DOI: 10.1016/s0266-7681(97)80272-0

[51] Chapter 68 Soft-Tissue Tumors: Evaluation and Diagnosis. 2020.

[52] Granular cell schwannoma of the hand. The Journal of Hand Surgery. 1980. DOI: 10.1016/s0363-5023(80)80043-8

[53] Surgical Treatment of Enchondromas of the Hand During Childhood in Ollier Disease. The Journal of Hand Surgery. 2018. DOI: 10.1016/j.jhsa.2018.02.010

[54] Metastatic Tumours of the Hand. Journal of Hand Surgery. 1996. DOI: 10.1016/s0266-7681(96)80063-5

[55] The role of imaging in isolated benign peripheral nerve tumors: A practical review for surgeons. Hand Surgery and Rehabilitation. 2016. DOI: 10.1016/j.hansur.2016.08.001

[56] Primary osteogenic sarcoma of a finger proximal phalanx: A case report and literature review. The Journal of Hand Surgery. 2001. DOI: 10.1053/jhsu.2001.28945

[57] The hazards of biopsy in patients with malignant primary bone and soft-tissue tumors.. The Journal of Bone & Joint Surgery. 1982. DOI: 10.2106/00004623-198264080-00002

[58] VASCULAR TUMORS IN CHILDREN. Hand Clinics. 1995. DOI: 10.1016/s0749-0712(21)00050-0

[59] Juvenile aponeurotic fibroma with disseminated fibrosarcoma. The Journal of Hand Surgery. 1986. DOI: 10.1016/s0363-5023(86)80024-7

[60] A Well-Differentiated Liposarcoma in the Digit: A Case Report of the 5-Year Post-Surgical Follow-Up. The Journal of Hand Surgery (Asian-Pacific Volume). 2018. DOI: 10.1142/s242483551872027x

[61] Six cases of sporadic schwannomatosis: Topographic distribution and outcomes of peripheral nerve tumors. Hand Surgery and Rehabilitation. 2017. DOI: 10.1016/j.hansur.2017.07.001

[62] Fibrolipomatous hamartoma of the median nerve. The Journal of Hand Surgery: Journal of the British Society for Surgery of the Hand. 1987. DOI: 10.1016/0266-7681(87)90018-0

[63] Fibrolipomatous Hamartoma of the Median Nerve. Journal of Hand Surgery. 1987. DOI: 10.1016/0266-7681_87_90018-0

[64] Giant cell tumor of the hand: Superior results with curettage, cryosurgery, and cementation. The Journal of Hand Surgery. 2001. DOI: 10.1053/jhsu.2001.22525

[65] Extensive Cutaneous Metastatic Breast Carcinoma of the Hand and Upper Extremity: A Case Report. The Journal of Hand Surgery. 2007. DOI: 10.1016/j.jhsa.2006.10.022

[66] Benign Bone Tumors and Nonneoplastic Conditions Simulating Bone Tumors. 2020.

[67] Tumors in the hand. The Journal of Hand Surgery. 1983. DOI: 10.1016/s0363-5023(83)80277-9

[68] Recurrence of a giant-cell tumor with malignant transformation to a fibrosarcoma twenty-five years after primary treatment. A case report.. The Journal of Bone & Joint Surgery. 1989. DOI: 10.2106/00004623-198971050-00019

[69] Benign Bone Tumors of the Hand: Beyond Enchondromas. Journal of the American Academy of Orthopaedic Surgeons. 2024. DOI: 10.5435/jaaos-d-23-01020

[70] Cartilage Tumors: Evaluation and Treatment. Journal of the American Academy of Orthopaedic Surgeons. 2000. DOI: 10.5435/00124635-200009000-00003

[71] Epithelioid Sarcoma of the Hand. Clinical Orthopaedics & Related Research. 2005. DOI: 10.1097/01.blo.0000150317.50594.96

[72] Adult Soft-Tissue Sarcomas of the Extremities. Journal of Bone and Joint Surgery. 2021. DOI: 10.2106/jbjs.21.00196

[73] Eccrine tumours of the hand: Two cases. The Journal of Hand Surgery: Journal of the British Society for Surgery of the Hand. 1987. DOI: 10.1016/0266-7681(87)90075-1

[74] Neurofibrosarcomas of the upper extremity. The Journal of Hand Surgery. 1991. DOI: 10.1016/s0363-5023(10)80153-4

[75] CHONDROMYXOID FIBROMA OF BONE: Report of Two Cases.. The Journal of Bone and Joint Surgery. American Volume. 1953.

[76] Chondrosarcoma of the Extraskeletal Soft Tissues: A REPORT OF SEVEN CASES AND REVIEW OF THE LITERATURE.. The Journal of Bone and Joint Surgery. American Volume. 1967.

[77] Cellular schwannoma of the hand. The Journal of Hand Surgery. 1989. DOI: 10.1016/s0363-5023(89)80101-7

[78] Exam Of The Hand Wrist 2Ed. INTRODUCTION.

[79] Benign Hand Tumors (Part II): Soft Tissue Tumors. HAND. 2020. DOI: 10.1177/1558944720928499

[80] Tumor-Like Conditions of the Hand and Upper Extremity. The Journal of Hand Surgery. 2017. DOI: 10.1016/j.jhsa.2017.09.012

[81] Exam Of The Hand Wrist 2Ed. Functional cutaneous units.

[82] Angiomyoma (vascular leiomyoma) of the median nerve. The Journal of Hand Surgery. 1996. DOI: 10.1016/s0363-5023(96)80120-1

[83] A systematic review of multicentric giant cell tumour with the presentation of three cases at long-term follow-up. The Bone & Joint Journal. 2022. DOI: 10.1302/0301-620x.104b12.bjj-2022-0401.r1

[84] Green S Operative Hand Surgery. Interosseous and Hypothenar Muscles.

[85] Neurofibromas of Digital Nerves. Journal of Hand Surgery. 1997. DOI: 10.1016/s0266-7681(97)80019-8

[87] MALIGNANT SOFT TISSUE TUMORS (SOFT TISSUE SARCOMAS). 2024.

[88] Granular cell tumor in the hand. The Journal of Hand Surgery. 1987. DOI: 10.1016/s0363-5023(87)80073-4

[89] Cutaneous Paraneoplastic Syndrome Affecting the Hand in a Case of Underlying Waldenström Macroglobulinemia. HAND. 2023. DOI: 10.1177/15589447231151260

[91] Atypical schwannoma of the median nerve. A case report. Hand Surgery and Rehabilitation. 2019. DOI: 10.1016/j.hansur.2019.05.001

[92] Exam Of The Hand Wrist 2Ed. The arches of the hand > The metacarpal arch.

[93] Tumeurs glomiques sous-unguéales des doigts : diagnostic échographique. Chirurgie de la Main. 2006. DOI: 10.1016/j.main.2005.12.007

[94] BENIGN AND MALIGNANT MUSCLE TUMORS IN THE HAND AND FOREARM. Hand Clinics. 1995. DOI: 10.1016/s0749-0712(21)00042-1

[95] Benign Bony and Soft Tissue Tumors of the Hand. The Journal of Hand Surgery. 2010. DOI: 10.1016/j.jhsa.2010.08.015

[96] Diagnostic strategy for bone and soft-tissue tumors.. The Journal of Bone & Joint Surgery. 1993. DOI: 10.2106/00004623-199304000-00019

[97] Exam Of The Hand Wrist 2Ed. Techniques of investigation of the arterial supply by J P Melki > Vascularization of the thumb > Palmar aspect.

[98] Campbell S Operative Orthopaedics 4 Volume Set. RESULTS OF SUTURE OF THE SCIATIC NERVE > PALMAR INCISIONS.

[100] Solitary, Adult-Onset, Intraosseous Myofibroma of the Finger: Report of a Case and Review of Literature. HAND. 2014. DOI: 10.1007/s11552-014-9729-4

[101] The Association Between Glomus Tumors and Neurofibromatosis. The Journal of Hand Surgery. 2013. DOI: 10.1016/j.jhsa.2013.05.025

[102] Neurofibrome myxoïde du pouce. Chirurgie de la Main. 2012. DOI: 10.1016/j.main.2012.07.007

[103] Tumorous lesions of the hand. The Journal of Hand Surgery. 1985. DOI: 10.1016/s0363-5023(85)80124-6

[104] Multiple Intraneural Glomus Tumors on a Digital Nerve: Case Report. The Journal of Hand Surgery. 2013. DOI: 10.1016/j.jhsa.2013.07.024

[105] Campbell S Operative Orthopaedics 4 Volume Set. ARTHROSCOPIC DEBRIDEMENT AND PARTIAL SYNOVECTOMY OF THE HIP IN AN ADULT > DIAGNOSTIC EVALUATION > GENERAL APPROACH TO MUSCULOSKELETAL NEOPLASMS.

[106] A Lange Medical Book Current Diagnosis Treatment In Orthopedics Fifth Edition. 9Hand Surgery > FLEXOR TENDON INJURY.

[107] Re: Malignant peripheral nerve sheath tumour of the hand: challenges in diagnosis and management of a child. Journal of Hand Surgery (European Volume). 2008. DOI: 10.1177/1753193408092039

[108] Exam Of The Hand Wrist 2Ed. 1.3 MOVEMENTS OF THE HAND AND WRIST > Gliding mechanisms.

[109] Benign Hand Tumors (Part I): Cartilaginous and Bone Tumors. HAND. 2020. DOI: 10.1177/1558944720922921

[110] Exam Of The Hand Wrist 2Ed. The dorsal skin.

[111] Glomus tumors: report on eleven cases and a review of the literature. Chirurgie de la Main. 2000. DOI: 10.1016/s1297-3203(00)73487-1

[112] Peripheral Primitive Neuroectodermal Tumour of the Hand in an Adult. Journal of Hand Surgery (European Volume). 2007. DOI: 10.1016/j.jhse.2007.04.001

[114] Instructional Course Lectures, The American Academy of Orthopaedic Surgeons - Evaluation, Diagnosis, and Classification of Benign Soft-Tissue Tumors†. The Journal of Bone & Joint Surgery*. 1996. DOI: 10.2106/00004623-199601000-00018

[115] Green S Operative Hand Surgery. PERTINENT ANATOMY.

[118] Tumors and Tumorous Conditions of the Hand. 2020.

[120] Tumeur glomique de la main. Hand Surgery and Rehabilitation. 2017. DOI: 10.1016/j.hansur.2017.10.207

[121] Rating the Efficacy of Diagnostic Imaging Studies Conducted for the Workup of Musculoskeletal Tumors. JAAOS: Global Research and Reviews. 2025. DOI: 10.5435/jaaosglobal-d-25-00015

[122] Sarcoma Chemotherapy. Journal of the American Academy of Orthopaedic Surgeons. 2013. DOI: 10.5435/jaaos-21-08-480

[123] Magnetic resonance imaging of soft tissue masses: An evaluation of fifty-three histologically proven tumors. Magnetic Resonance Imaging. 1988. DOI: 10.1016/0730-725x(88)90397-9

[125] Tumeurs métastatiques des parties molles des doigts. À propos de six cas. Chirurgie de la Main. 2000. DOI: 10.1016/s1297-3203(00)73461-5

[126] Management of adipose tumors in the limbs. Orthopaedics & Traumatology: Surgery & Research. 2022. DOI: 10.1016/j.otsr.2021.103162

[130] Campbell S Operative Orthopaedics 4 Volume Set. TUMORS AND TUMOROUS CONDITIONS OF THE HAND > DIAGNOSIS.

[132] Case Report: Angiosarcoma in the Upper Extremity Related to a Nonfunctioning Arteriovenous Fistula. HAND. 2017. DOI: 10.1177/1558944717702466

[133] Tumeur glomique de l'avant-bras : à propos d'un cas. Chirurgie de la Main. 2005. DOI: 10.1016/j.main.2005.01.005

[134] NON-NEOPLASTIC TUMORS OF THE HAND AND UPPER EXTREMITY. Hand Clinics. 1995. DOI: 10.1016/s0749-0712(21)00039-1

[135] Squamous Cell Carcinoma of the Hand: A 20-Year Review. The Journal of Hand Surgery. 2013. DOI: 10.1016/j.jhsa.2013.08.090

[136] MR imaging of synovial tumors and tumor-like lesions. European Radiology. 2001. DOI: 10.1007/s003300000759

[138] Therapeutic and functional results of limb salvage to treat sarcomas of the forearm and hand. The Journal of Hand Surgery. 1988. DOI: 10.1016/s0363-5023(88)80067-4

[139] Posterior Interosseous Nerve Palsy Due to Schwannoma: Case Report. The Journal of Hand Surgery. 2008. DOI: 10.1016/j.jhsa.2008.05.033

[140] Intraperitoneal extraosseous osteosarcoma: a case report and literatures review. BMC Musculoskeletal Disorders. 2020. DOI: 10.1186/s12891-020-03429-5

[141] Pigmented onychomatricoma may originate from a childhood trauma: A case report. Hand Surgery and Rehabilitation. 2019. DOI: 10.1016/j.hansur.2019.09.002

[142] Giant-cell tumors of the bones of the hand. The Journal of Hand Surgery. 1980. DOI: 10.1016/s0363-5023(80)80042-6

[143] Ewing's sarcoma of the hand: Localization and treatment. The Journal of Hand Surgery. 1990. DOI: 10.1016/s0363-5023(09)90033-8

[144] Histopathology of fibro-osseous and cystic tumors of bone. Diagnostic Histopathology. 2020. DOI: 10.1016/j.mpdhp.2020.07.003

[145] Tumoral calcinosis of the triangular fibrocartilage complex: A case report. The Journal of Hand Surgery. 1997. DOI: 10.1016/s0363-5023(97)80177-3

[146] Unifocal giant cell tumor of the triquetrum: a case report. Journal of Hand Surgery (European Volume). 2011. DOI: 10.1177/1753193411421720

[147] Apley And Solomon S Concise System Of Orthopaedics And Trauma. STAGING.

[148] MRI and CT in the preoperative evaluation of soft-tissue tumors. Archives of Orthopaedic and Trauma Surgery. 1991. DOI: 10.1007/bf00395801

[149] Role of magnetic resonance imaging in the radiological management of musculoskeletal tumors. Archives of Orthopaedic and Trauma Surgery. 1991. DOI: 10.1007/bf00395793

[150] Leiomyoma of the Hand. HAND. 2008. DOI: 10.1007/s11552-008-9143-x

[151] A Glomus Tumour of the Elbow: A Case Report and Review of the Literature. Shoulder & Elbow. 2014. DOI: 10.1111/sae.12041

[152] Improved Survival for Sarcomas of the Wrist and Hand. The Journal of Hand Surgery. 2006. DOI: 10.1016/j.jhsa.2005.11.005

[153] Giant Lipomatous Tumours of the Hand and Forearm. Journal of Hand Surgery. 2005. DOI: 10.1016/j.jhsb.2005.05.002

[154] Skin Cancer of the Hand. Journal of the American Academy of Orthopaedic Surgeons. 2015. DOI: 10.5435/jaaos-d-14-00040

[155] Patients with benign hand tumors are indicated for surgery according to patient-rated outcome measures. Journal of Plastic, Reconstructive & Aesthetic Surgery. 2017. DOI: 10.1016/j.bjps.2016.12.010

[156] Staging of Bone and Soft-tissue Sarcomas. Journal of the American Academy of Orthopaedic Surgeons. 2018. DOI: 10.5435/jaaos-d-17-00055

[157] Does Curettage without Adjuvant Therapy Provide Low Recurrence Rates in Giant-Cell Tumors of Bone?. Clinical Orthopaedics and Related Research. 2005. DOI: 10.1097/01.blo.0000160024.06739.ff

[158] Two rapidly growing fatty tumors of the upper limb in children: Lipoblastoma and infiltrating lipoma. The Journal of Hand Surgery. 1995. DOI: 10.1016/s0363-5023(05)80051-6

[159] A neurilemmoma and a neurofibroma in the same nerve trunk. The Journal of Hand Surgery: Journal of the British Society for Surgery of the Hand. 1988. DOI: 10.1016/0266-7681(88)90063-0

[161] Prolifération ostéocartilagineuse parostéale bizarre ou tumeur de Nora. À propos de deux cas. Chirurgie de la Main. 2005. DOI: 10.1016/j.main.2005.08.014

[162] Vascular leiomyoma in the hand. The Journal of Hand Surgery. 1987. DOI: 10.1016/s0363-5023(87)80223-x

[163] Sub-ungual melanoma. The Journal of Hand Surgery: Journal of the British Society for Surgery of the Hand. 1990. DOI: 10.1016/0266-7681(90)90003-m

[164] Primitive neuroectodermal tumor of the thumb metacarpal bone: A case report and literature review. The Journal of Hand Surgery. 2003. DOI: 10.1053/jhsu.2003.50056

[165] Unusual Manifestation of Chronic Lymphocytic Leukemia in the Hand. The Journal of Hand Surgery. 2021. DOI: 10.1016/j.jhsa.2020.03.014

[166] Nora’s Lesion. Journal of Hand Surgery. 1997. DOI: 10.1016/s0266-7681(97)80269-0

[167] Vascular tumors. Hand Clinics. 2004. DOI: 10.1016/j.hcl.2004.03.009

[168] Osteoid osteoma of the elbow: A report of six cases. The Journal of Hand Surgery. 1992. DOI: 10.1016/0363-5023(92)90349-t

[169] Osteoid Osteoma of Distal Phalanx of Middle Finger—A Diagnostic Dilemma. The Journal of Hand Surgery (Asian-Pacific Volume). 2016. DOI: 10.1142/s2424835516720140

[171] Is Treatment at a High-volume Center Associated with an Improved Survival for Primary Malignant Bone Tumors?. Clinical Orthopaedics & Related Research. 2019. DOI: 10.1097/corr.0000000000001034

[172] Malignant and Metastatic Tumors of the Hand. The Journal of Hand Surgery. 2010. DOI: 10.1016/j.jhsa.2010.08.014

[173] Trigger Finger Caused by Extraskeletal Chondroma. The Journal of Hand Surgery. 2017. DOI: 10.1016/j.jhsa.2016.10.003

[177] Enchondromas of the hand: Treatment with curettage and cemented internal fixation. The Journal of Hand Surgery. 2002. DOI: 10.1053/jhsu.2002.34369

[178] Magnetic resonance imaging in planning limb-salvage surgery for primary malignant tumors of bone.. The Journal of Bone & Joint Surgery. 1986. DOI: 10.2106/00004623-198668060-00003

[179] Intraosseous growth of giant cell tumors of the tendon sheath (localized nodular tenosynovitis) of the digits: Report of 15 cases. The Journal of Hand Surgery. 1998. DOI: 10.1016/s0363-5023(98)80062-2

[180] Surgical Management of Benign Tumors of the Proximal Fibula. JAAOS: Global Research and Reviews. 2021. DOI: 10.5435/jaaosglobal-d-21-00207

[181] Segmental neurofibromatosis of the upper extremity: a case report. Journal of Hand Surgery (European Volume). 2014. DOI: 10.1177/1753193413517807

[182] Intraosseous myoepithelioma: a rare tumour in the hand. Journal of Hand Surgery (European Volume). 2016. DOI: 10.1177/1753193416676229

[183] Glomus tumor imaging: Use of MRI for localization of occult lesions. The Journal of Hand Surgery. 1992. DOI: 10.1016/0363-5023(92)90353-q

[185] Intravascular Myopericytoma of the Superficial Palmar Arch. Journal of Hand Surgery (European Volume). 2007. DOI: 10.1016/j.jhsb.2007.01.011

[186] Soft-Tissue Osteoma of the Hand: Case Report. The Journal of Hand Surgery. 2006. DOI: 10.1016/j.jhsa.2006.02.003

[187] A Glomus Tumour with Four Recurrences. Journal of Hand Surgery. 1999. DOI: 10.1054/jhsb.1998.0047

[188] Merkel Cell Tumor of the Hand: Report of Two Cases. The Journal of Hand Surgery. 2008. DOI: 10.1016/j.jhsa.2007.12.003

[189] Recurrent myxoma of the hand. The Journal of Hand Surgery. 2000. DOI: 10.1053/jhsu.2000.9412

[190] Juvenile aponeurotic fibroma: A case report. The Journal of Hand Surgery. 1977. DOI: 10.1016/s0363-5023(77)80120-2

[191] Desmoid tumor of the hand—Case report and literature review. The Journal of Hand Surgery. 1983. DOI: 10.1016/s0363-5023(83)80064-1

[192] A Novel Surgical Classification for Extremity and Pelvic Hemophilic Pseudotumors. Journal of Bone and Joint Surgery. 2023. DOI: 10.2106/jbjs.22.00781

[193] Subscapular elastofibroma: A reactive pseudotumor. Journal of Shoulder and Elbow Surgery. 1996. DOI: 10.1016/s1058-2746(05)80008-4

[194] Epithelioid Sarcoma in the Median Nerve of the Hand: A Case Report. HAND. 2021. DOI: 10.1177/15589447211030689

[195] Osteoid osteoma of the clavicle. Journal of Shoulder and Elbow Surgery. 1997. DOI: 10.1016/s1058-2746(97)90009-4

[196] Malignant fibrous histiocytoma of the forearm: Report of a case and review of the literature. The Journal of Hand Surgery. 1977. DOI: 10.1016/s0363-5023(77)80129-9

[197] A New Relevant Integrated Radiologic and Surgical Classification Scheme for Giant Cell Tumors of Bones. JAAOS: Global Research and Reviews. 2025. DOI: 10.5435/jaaosglobal-d-24-00321

[198] Pathologic fractures in primary bone tumors: a paradigm shift in management. Injury. 2025. DOI: 10.1016/j.injury.2025.112853

[199] An investigation of PIK3CA mutations in isolated macrodactyly. Journal of Hand Surgery (European Volume). 2018. DOI: 10.1177/1753193418770366

[200] Benign soft tissue chondromatous lesion of the hand. The Journal of Hand Surgery. 1985. DOI: 10.1016/s0363-5023(85)80169-6

[201] Upfront surgery is not advantageous compared to more conservative treatments such as observation or medical treatment for patients with desmoid tumors. BMC Musculoskeletal Disorders. 2021. DOI: 10.1186/s12891-020-03897-9

[202] Enchondroma of the Hand: Evaluation and Management. Journal of the American Academy of Orthopaedic Surgeons. 2016. DOI: 10.5435/jaaos-d-15-00452

[203] What’s New in Musculoskeletal Tumor Surgery. Journal of Bone and Joint Surgery. 2020. DOI: 10.2106/jbjs.20.01510

[205] Lipofibromatous hamartoma of the radial nerve: An unusual location. Hand Surgery and Rehabilitation. 2017. DOI: 10.1016/j.hansur.2016.05.007

[206] Sweat gland carcinoma of the hand: Two cases of malignant eccrine spiradenoma. The Journal of Hand Surgery. 1984. DOI: 10.1016/s0363-5023(84)80079-9

[207] Local Control of Extra-Abdominal Desmoid Tumors†. The Journal of Bone & Joint Surgery*. 1996. DOI: 10.2106/00004623-199606000-00007

[210] FIBROMATOSES AND RELATED TUMORS OF THE HAND IN CHILDREN. Hand Clinics. 2000. DOI: 10.1016/s0749-0712(21)00222-5

[211] Chapter 145 Benign Soft-Tissue Tumors and Reactive Lesions. 2019.

[213] Epithelioid Sarcoma in the Hand. Journal of Hand Surgery. 1984. DOI: 10.1016/s0266-7681(84)80035-2

[215] Infantile Fibrosarcoma of the Hand: Limb-Sparing Treatment With Modern Targeted Oral Chemotherapy and Conservative Surgical Resection. Journal of Hand Surgery Global Online. 2026. DOI: 10.1016/j.jhsg.2025.100887

[216] Solitary Enchondroma at the Hand. Long-Term Follow-Up Study after Operative Treatment⋆. Journal of Hand Surgery. 2004. DOI: 10.1016/j.jhsb.2003.08.003

[217] Le chondrosarcome de la main. Discussion diagnostique et thérapeutique (à propos de trois cas). Chirurgie de la Main. 2001. DOI: 10.1016/s1297-3203(01)00023-3

[218] Malignant Bone Tumors: Limb Sparing Versus Amputation. Journal of the American Academy of Orthopaedic Surgeons. 2003. DOI: 10.5435/00124635-200301000-00005

[219] Metastatic tumors of the hand. The Journal of Hand Surgery. 1987. DOI: 10.1016/s0363-5023(87)80299-x

[220] Aggressive fibrous tissue lesions in the upper extremity: Treatment and results. The Journal of Hand Surgery. 1994. DOI: 10.1016/0363-5023(94)90283-6

[221] Clinical surgical study of tumors involving brachial plexus. Journal of Hand Surgery. 1996. DOI: 10.1016/s0266-7681(96)80262-2

[224] Squamous cell carcinoma of the hand: Fifty-five case reports. The Journal of Hand Surgery. 1988. DOI: 10.1016/s0363-5023(88)80018-2

[227] Chapter 52 Concepts in Primary Benign, Primary Malignant, and Metastatic Tumors of the Spine. 2020.

[230] The most common bone tumors of the upper extremity in childhood and adolescence and their treatment: a review of the current literature. EFORT Open Reviews. 2026. DOI: 10.1530/eor-2025-0149

[231] Malignant Tumors of the Hand. Journal of the American Academy of Orthopaedic Surgeons. 2020. DOI: 10.5435/jaaos-d-20-00333

[233] Soft tissue sarcomas of the forearm, wrist and hand. Hand Surgery and Rehabilitation. 2017. DOI: 10.1016/j.hansur.2016.12.010

[237] The Anatomic Location of Digital Glomus Tumor Recurrences. The Journal of Hand Surgery. 2010. DOI: 10.1016/j.jhsa.2010.02.019

[245] Metastatic basal cell carcinoma of the upper extremity. The Journal of Hand Surgery. 1992. DOI: 10.1016/s0363-5023(09)91071-1

[246] A Clinicopathologic Study of 45 Pediatric Soft Tissue Tumors With an Admixture of Adipose Tissue and Fibroblastic Elements, and a Proposal for Classification as Lipofibromatosis. The American Journal of Surgical Pathology. 2000. DOI: 10.1097/00000478-200011000-00004

[252] Secondary malignant giant-cell tumor of bone. Clinicopathological assessment of nineteen patients.. The Journal of Bone & Joint Surgery. 1986. DOI: 10.2106/00004623-198668070-00016

[254] Multiplicity of neurilemomas in the upper extremity. The Journal of Hand Surgery. 1987. DOI: 10.1016/s0363-5023(87)80298-8

[259] Congenital primitive neuroectodermal tumor of the hand: A case report. The Journal of Hand Surgery. 1997. DOI: 10.1016/s0363-5023(97)80140-2

[269] Sweat gland carcinoma of the hand (malignant acrospiroma). The Journal of Hand Surgery. 1989. DOI: 10.1016/s0363-5023(89)80018-8

[271] Metastatic transitional cell carcinoma to the hand. The Journal of Hand Surgery. 1994. DOI: 10.1016/0363-5023(94)90189-9

[273] Subungual keratoacanthoma in the hand. The Journal of Hand Surgery. 1989. DOI: 10.1016/0363-5023(89)90074-9

[274] Une localisation inhabituelle d’un lipome ossifié juxtaosseux. À propos d’un cas. Chirurgie de la Main. 2013. DOI: 10.1016/j.main.2013.02.013

[275] Tumeurs à cellules géantes des gaines synoviales de la main : revue de 96 patients a un recul moyen de 12 ans. Chirurgie de la Main. 2012. DOI: 10.1016/j.main.2012.10.120

[277] Multiple schwannomas of the ulnar nerve: A case report. The Journal of Hand Surgery. 1996. DOI: 10.1016/s0363-5023(96)80207-3

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