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

210 citationsUpdated Nov 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, enchondromas, and cutaneous neoplasms. Definitive diagnosis requires pathologic evaluation, as MRI findings alone are not definitive [1]. An orderly protocol is essential for appropriate diagnosis and surgical staging [16]. Biopsies for suspected malignant primary bone and soft-tissue tumors must be planned with the same rigor as definitive surgery [44]. 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 [44, 58]. For brachial plexus tumors suspected to be malignant, biopsies should be performed before starting definitive treatment [2].

Management strategies vary significantly by histology and symptom status. Asymptomatic benign tumors of the brachial plexus should be observed [2]. Surgical excision is the primary treatment for symptomatic perionychium tumors [5], intravenous pyogenic granuloma of the hand [46], extraskeletal chondroma causing trigger finger [175], and pigmented onychomatricoma [145]. For malignant peripheral nerve sheath tumours, complete surgical excision with clear margins is the only curative treatment [35]. Wide surgical excision remains the standard of care for definitive treatment of synovial sarcoma [59], with adjuvant radiation utilized for larger and deeper lesions [59]. Tumor resection is the preferred option for tumor-induced osteomalacia other than tumor curettage surgery [40].

Prognosis and long-term outcomes depend on tumor type and treatment adequacy. Schwannomas treated by excision typically have few long-term deficits [1], whereas neurofibroma excision carries a higher risk of nerve dysfunction and pain worsening [1]. Prognosis for malignant peripheral nerve sheath tumours remains poor, particularly for patients with neurofibromatosis [35]. Long-term clinical follow-up of 10 years is advised for soft-tissue sarcoma of the hand even with adequate oncologic treatment [11]. The absence of disease on MRI should not be the sole criterion for determining whether repeat resection is performed after marginal excision of unsuspected soft tissue sarcomas [10]. Management of benign tumors emphasizes balancing tumor recurrence risk and postoperative functionality within the context of histologic diagnoses and surgical approaches [182]. Knowledge regarding treatment of skin tumors of the hand and upper extremity is often based on case reports, small case series, and retrospective reviews rather than controlled trials [43].

Anatomy & Pathophysiology

Bony Anatomy

The hand and wrist skeleton comprises 27 bones, 19 of which are long bones [27]. The hand skeleton is organized into five rays, each forming a polyarticulated chain of metacarpals and phalanges [27]. The base of each metacarpal articulates with the distal carpal row, while the carpus articulates with the forearm via its proximal row [27]. 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 [27]. The radial ray is the shortest, consisting of a metacarpal and two phalanges, and continues the external carpal column formed by the scaphoid and trapezium [27]. The trapezium is angled anteriorly out of the carpal plane, creating an approximately 45-degree angle between the first and second metacarpals in the sagittal plane [27]. The thumb metacarpal is the shortest, whereas the index metacarpal is the longest [27]. The proximal and middle phalanges of the long and ring fingers are longer than those of the index finger [27]. Epiphyseal plates are located at the proximal ends of the phalanges and the first metacarpal, and at the distal ends of the other metacarpals [27].

The hand skeleton presents a longitudinal and transverse concavity, forming a cup shape with palmar concavity when the thumb is placed next to the index finger [27]. The transverse axis of the palm corresponds to the metacarpophalangeal articulations and is oblique, positioned more distally at the index metacarpophalangeal joint and more proximally at the fifth metacarpophalangeal joint [27]. This transverse palmar axis forms an acute angle of approximately 75 degrees with the longitudinal axis [27]. The index metacarpal is the most firmly fixed [92]. The ring metacarpal exhibits about 10 degrees of mobility in flexion and extension, while the fifth metacarpal has a range of flexion–extension of approximately 20 degrees [92]. The second through fifth metacarpals are bound together by the deep transverse intermetacarpal ligament, also known as the interglenoid ligament [92]. This ligament ties together the anterior glenoid ligaments of the metacarpophalangeal articulations, known as the volar plates [92]. The metacarpophalangeal joints serve as the keystones of the hand’s longitudinal arches [92]. The thick anterior glenoid capsules, or volar plates, of these joints prevent hyperextension [92]. Stability of the metacarpophalangeal joints is essential for supporting both the longitudinal and transverse metacarpal arches [92].

Muscular Anatomy

Control of digital posture requires a complex balance of extrinsic and intrinsic muscle forces [24]. Extrinsic muscles originate outside the hand and insert on the hand or carpus, functioning as either flexors or extensors [24]. Intrinsic muscles have both origin and insertion within the hand and contribute to both digital flexion and extension [24]. The extrinsic extensors run through six fibroosseous retinacular compartments at the wrist level [24].

First Compartment: Contains the abductor pollicis longus and extensor pollicis brevis [24]. The abductor pollicis longus inserts at the base of the thumb metacarpal and radially abducts the thumb [24]. The extensor pollicis brevis inserts on the dorsum of the proximal aspect of the thumb’s proximal phalanx and actively extends the thumb metacarpophalangeal joint [24].

Second Compartment: Contains the extensor carpi radialis longus and extensor carpi radialis brevis [24]. The extensor carpi radialis longus inserts on the index metacarpal, dorsiflexing and radially deviating the wrist [24]. The extensor carpi radialis brevis inserts into the base of the middle metacarpal and provides balanced wrist dorsiflexion [24].

Third Compartment: Contains the extensor pollicis longus [24]. This tendon runs longitudinally down the forearm, turns abruptly radialward about Lister tubercle, and inserts on the distal phalanx [24]. It provides forceful extension of the thumb interphalangeal joint [24]. The oblique course of the extensor pollicis longus tendon provides a substantial adduction component to its pull [24].

Fourth Compartment: Contains the extensor indicis proprius, which lies deep to the four tendons of the extensor digitorum communis [24]. The extensor indicis proprius inserts on the index finger ulnar to the extensor digitorum communis [24].

Fifth Compartment: Contains the extensor digiti quinti [24]. The extensor digitorum communis inserts on the index, middle, ring, and, in some cases, little fingers [24]. The extensor digiti quinti tendon inserts on the little finger ulnar to the extensor digitorum communis insertion [24].

Sixth Compartment: Contains the extensor carpi ulnaris, which inserts at the base of the little finger metacarpal and provides wrist extension and ulnar deviation [24]. The principal bony insertion of the extrinsic digital extensors is on the dorsal proximal aspect of the middle phalanx [24]. The extensor digitorum communis tendons of the middle, ring, and little fingers are tethered together by juncturae tendinum over the dorsum of the hand proximal to the metacarpophalangeal joint [24].

Metacarpophalangeal joint extension is provided by extrinsic extensor force transmitted through the sagittal bands [24]. Distal interphalangeal joint extension is achieved through conjoined lateral bands composed of tendinous slips from extrinsic and intrinsic tendons [24]. The digital extensor tendons are stabilized over the mid-line of the metacarpophalangeal joint by their attachment to sagittal band fibers [24]. These fibers insert onto the volar proximal phalanx and onto the lateral borders of the volar plate [24]. The sagittal bands form a sling that allows proximal extrinsic extensor tension to be transmitted to the proximal phalanx, permitting metacarpophalangeal joint extension without a tendinous insertion onto the proximal phalanx [24]. They keep the extrinsic extensor as far as possible away from the center of rotation of the metacarpophalangeal joint, giving it the greatest mechanical efficiency [24]. Rupture or attenuation of the sagittal band fibers allows the extrinsic extensor tendon to sublux to the ulnar side of the metacarpal head, causing ulnar deviation of the finger [24].

The extrinsic finger flexors are the flexor digitorum profundus and the flexor digitorum superficialis [24]. The flexor digitorum profundus inserts on the proximal volar aspect of the distal phalanx and flexes the distal interphalangeal joint as well as the proximal interphalangeal and metacarpophalangeal joints [24]. The flexor digitorum superficialis acts as a flexor of the proximal interphalangeal and metacarpophalangeal joints [24].

There are seven interosseous muscles: four dorsal and three volar [84]. The anatomic axis of the hand coincides with the axis of the third metacarpal [84]. The dorsal interossei are abductors [84]. They lie to the radial side of the index and middle fingers and the ulnar side of the middle and ring fingers [84]. The little finger is abducted by the abductor digiti quinti [84]. The volar interossei are adductors and lie to the ulnar side of the index finger and the radial side of the ring and little fingers [84]. The middle finger has two dorsal interossei (abductors) and no volar interossei (adductors) because the central axis of the hand lies within it [84]. Each dorsal interosseous muscle, with the exception of the third, has two muscle heads [84]. The superficial head arises from the shaft of the contiguous metacarpals and is inserted by a medial tendon onto the lateral tubercle of the base of the proximal phalanx, abducting and weakly flexing the proximal phalanx [84]. The deep head forms a lateral tendon, or lateral band, at the level of the MP joint, flexing and weakly abducting the proximal phalanx while extending the middle and distal phalanges [84]. Transverse fibers arch dorsally from each lateral band to join each other over the dorsum of the finger, flexing the proximal phalanx [84]. Oblique fibers (spiral fibers) from the lateral bands sweep over the distal third of the proximal phalanx to insert onto the lateral tubercles at the base of the middle phalanx, extending the middle phalanx (PIP joint) [84]. The lateral bands are joined by the lateral slips of the extensor tendon to form the conjoined lateral band [84]. The two conjoined lateral bands to each finger unite at the distal third of the middle phalanx to form the terminal tendon, which inserts at the base of the distal phalanx to extend it [84].

The flexor digiti quinti brevis is structurally and functionally similar to the deep head of the dorsal interossei, forming the ulnar lateral band of the little finger [84]. The three volar interossei arise from adjacent surfaces of contiguous metacarpal shafts [84]. Each volar interosseous muscle has only one muscle head and none of them insert onto the proximal phalanx [84]. The volar interossei form the ulnar lateral band of the index finger and the radial lateral band of the ring and little fingers [84]. The abductor digiti quinti and flexor digiti quinti brevis are similar in structure and function to the superficial and deep heads of the dorsal interossei, respectively, and arise from the fifth metacarpal [84]. The abductor digiti quinti inserts onto the ulnar lateral tubercle at the base of the proximal phalanx of the little finger [84]. The flexor digiti quinti forms the ulnar lateral band [84]. The opponens digiti quinti arises from the pisohamate ligament and the hook of the hamate and inserts onto the ulnar side of the diaphysis of the fifth metacarpal, flexing and supinating the fifth metacarpal [84].

The flexor digitorum profundus originates from the proximal ulna and the interosseous membrane [108]. In the forearm, it divides into a radial component supplying the index finger and an ulnar component supplying the middle, ring, and little fingers [108]. The flexor digitorum profundus and flexor pollicis longus muscles form the deep compartment of the volar forearm [108]. Their tendons occupy the floor of the carpal tunnel [108]. The tenosynovial sheath of the flexor pollicis longus is continuous with the radial bursa [108]. The tenosynovial sheath to the little finger is continuous with the ulnar digital bursa [108]. In some patients, the radial and ulnar bursae communicate, allowing a horseshoe abscess to spread between the thumb and little finger if infection occurs in the flexor tendon sheath of either digit [108]. The lumbricals originate from the radial side of the index, middle, ring, and little fingers in the palm [108]. The profundus tendon passes through the bifurcation of the flexor digitorum superficialis before inserting into the proximal palmar base of the distal phalanx [108]. The innervation of the flexor digitorum profundus of the index and middle fingers is through the anterior interosseous branch of the median nerve [108]. The profundus of the ring and little fingers is innervated by the ulnar nerve [108].

The flexor digitorum superficialis has two heads: a radial head originating from the proximal shaft of the radius, and a humeral ulnar head originating from the medial humeral epicondyle and coronoid process of the ulna [108]. Each digit has a corresponding independent superficialis muscle [108]. As the superficialis tendons pass through the carpal tunnel, the tendons of the middle and ring fingers are more superficial and central than those of the index and little fingers [108]. In the proximal aspect of the finger, the flexor digitorum superficialis tendon bifurcates around the flexor digitorum profundus at the beginning of the A2 pulley [108]. The flexor digitorum superficialis tendon slips reunite distally at the Camper chiasm, with approximately half of the fibers staying on the ipsilateral side and half crossing to the contralateral side of the finger [108]. The tendon inserts via radial and ulnar slips into the proximal metaphysis of the middle phalanx [108]. The entire flexor digitorum superficialis muscle receives innervation from the median nerve [108]. The primary function of the flexor digitorum superficialis is digital flexion at the proximal interphalangeal joint [108].

The flexor pollicis longus originates from two heads: a radial head from the proximal radius and interosseous membrane, and an accessory head from the coronoid process of the ulna and medial epicondyle of the humerus [108]. In the palm, the flexor pollicis longus tendon transverses between the abductor pollicis brevis and the flexor pollicis brevis [108]. It inserts into the proximal base of the thumb distal phalanx [108]. The flexor pollicis longus is innervated by the anterior interosseous branch of the median nerve [108]. It flexes both the interphalangeal and metacarpophalangeal joints of the thumb [108].

Tendon Sheaths and Gliding Mechanisms

As the flexor tendons pass distal to the metacarpal neck, they enter the fibroosseous tunnel, or digital flexor sheath [108]. The fibroosseous tunnel extends distally to the proximal aspect of the distal phalanx [108]. The tendinous sheath consists of annular pulleys, which provide mechanical stability, and cruciate pulleys, which provide flexibility [108]. The first, third, and fifth annular pulleys (A1, A3, and A5) are located over the metacarpophalangeal, proximal interphalangeal, and distal interphalangeal joints, respectively [108]. The second and fourth pulleys (A2 and A4) are situated over the middle portion of the proximal and middle phalanges [108]. The A2 and A4 pulleys are the most essential in maintaining the mechanical advantage of the flexor tendons [108]. The tenosynovium that lines the fibroosseous tunnel supplies both nutrition and lubrication to the poorly vascularized flexor tendons [108]. Proximal to the sheath, the tendons are well vascularized by the peritenon [108]. Within the sheath, tendon vascularity is supplied via the vincula system: the vinculum longus and brevis [108]. Most structures in the hand glide in relation to neighboring structures [110].

Classification

General Principles and Staging Systems

The basis of all staging systems includes the grade of the tumor, size of the tumor, local extent, and presence of metastases [150]. An orderly protocol for appropriate diagnosis and surgical staging of tumors is essential [16]. Treatment must take into consideration the location and size of the tumor, histologic grade, clinical behavior, and potential for metastases [28]. The lungs are the most common site for metastases from bone and soft-tissue sarcomas due to metastasis via the bloodstream [150]. Classification of tumors is increasingly based on molecular characteristics, which improves diagnostic accuracy for tumors difficult to classify on morphology alone [19].

Enneking: The Enneking staging system classifies primary bone tumors based on grade (high or low), presence of metastases, and whether the tumor is intracompartmental or extracompartmental [150]. In this system, 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 [150]. For soft-tissue sarcomas, the Enneking staging system is based primarily on grade, location within the anatomic compartment, and metastases [52].

AJCC: The American Joint Committee on Cancer (AJCC) staging system for soft-tissue sarcomas is based on tumor size and grade, lymph node involvement, and the presence or absence of distant metastases (TNM) [52]. 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 [52]. The AJCC staging system has widespread acceptance due to its multidisciplinary nature and ability to facilitate more nuanced prediction of prognosis compared to the MSTS system [159]. In the TNM staging system for soft-tissue sarcomas, size is dichotomized as small (<5 cm) or large (>5 cm) [150]. In the TNM staging system for bone sarcomas, size is dichotomized as small (<8 cm) or large (>8 cm) [150].

MSTS: The Musculoskeletal Tumor Society (MSTS) staging system is considered valid for primary malignant tumors of the upper extremity to permit statistical comparisons [155].

Benign Bone Tumors

Benign bone lesions are classified into three stages: stage 1 (latent), stage 2 (active), and stage 3 (locally aggressive) [70]. Benign bone tumors can be characterized by location and tissue type, including cartilage tumors, cystic lesions, osteogenic tumors, and miscellaneous lesions [70].

Enneking (Benign): In the Enneking staging system for benign tumors, a stage 1 tumor is latent, meaning it does not progress or heals spontaneously [71]. A stage 2 tumor is active, meaning it progresses but respects natural barriers such as the bone cortex [71]. A stage 3 tumor is aggressive, meaning it progresses and will ultimately destroy natural barriers [71]. Enchondromas are usually stage 1 but are occasionally stage 2 according to the Enneking staging system for benign tumors [71]. The Enneking clinico-radiological classification for benign bone tumors includes stage 1 (latent), stage 2 (active), stage 3 (aggressive), and stage IV (malignant) [38]. An Enneking stage 1 benign bone tumor is confined totally by bone, asymptomatic, inactive on bone scan, and histologically benign [38]. An Enneking stage 2 benign bone tumor has an expanded cortex with no breakthrough, is symptomatic, active on bone scan, and histologically benign [38]. An Enneking stage 3 benign bone tumor is a rapidly growing mass with cortical perforation and soft tissue mass, may metastasize, is symptomatic, and shows extensive activity on bone scan [38]. An Enneking stage IV benign bone tumor refers to a sarcomatous lesion contiguous with a benign giant cell tumor [38].

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) [38]. A Campanacci grade 1 GCT lesion has a well-defined margin and an intact cortex [38]. A Campanacci grade 2 GCT lesion has a relatively well-defined margin, no radiopaque rim, and a thinned and moderately expanded cortex [38]. A Campanacci grade 3 GCT lesion has indistinct borders and cortical destruction [38]. The Campanacci grading system for GCT is similar to the Enneking classification for benign bone tumors overall [38]. A novel GCT grading system demonstrates excellent interrater agreement and classifies more nonrecurrent curetted tumors as low grade compared with the Campanacci classification [198].

Malignant Bone Tumors

Enneking (Malignant): Nonmetastatic low-grade chondrosarcomas are considered stage I neoplasms according to the Enneking system [71]. Nonmetastatic intermediate- and high-grade chondrosarcomas are stage II according to the Enneking system [71]. Metastatic chondrosarcomas are stage III according to the Enneking system [71]. Chondrosarcomas are subclassified as stage A or stage B based on whether they are located within the bone or extend outside the bone [71]. A low-grade intramedullary chondrosarcoma without metastases is classified as stage IA [71]. A high-grade nonmetastatic chondrosarcoma with an associated soft tissue mass is classified as stage IIB [71]. High-grade malignant primary bone lesions typically undergo neoadjuvant therapies and surgery, while low-grade lesions are usually managed with surgery alone [42].

Soft-Tissue Tumors

The World Health Organization classifies soft-tissue sarcomas into 12 different groups based on tissue type [52]. Soft-tissue sarcomas are staged via the American Joint Committee on Cancer (AJCC) or Enneking staging systems [52]. The heterogeneous nature of sarcoma behavior limits the prognostic value of staging systems when compared with other cancers [52].

AJCC 7th Edition: The AJCC 7th edition anatomical classification for soft-tissue sarcomas includes stages IA, IB, IIA, IIB, III, and IV based on tumor size (T), lymph node status (N), metastasis status (M), and histologic grade (G) [236]. Tumor features (T) in the AJCC 7th edition are defined as T1 for small (<5 cm) and T2 for large (>5 cm), with 'a' indicating superficial location above fascia and 'b' indicating deep to fascia [236]. Lymph node characteristics (N) in the AJCC 7th edition are defined as N0 for no evidence of lymph node metastases and N1 for presence of lymph node metastases [236]. Distant metastases status (M) in the AJCC 7th edition is defined as M0 for no evidence of distant metastases and M1 for presence of distant metastases [236]. Histologic grade (G) in the AJCC 7th edition is defined as G1 for low grade, G2 for intermediate grade, G3 for high grade, and GX for indeterminate grade [236]. In the AJCC 7th edition classification, Stage IA includes T1a or T1b tumors with N0, M0, and G1 or GX grade [236]. In the AJCC 7th edition classification, Stage IIA includes T1a or T1b tumors with N0, M0, and G2 or G3 grade [236]. In the AJCC 7th edition classification, Stage III includes T2a or T2b tumors with N0, M0, and G3 grade, or any T with N1, M0, and any G grade [236]. In the AJCC 7th edition classification, Stage IV includes any T, any N, and M1 with any G grade [236].

UICC: The Union for International Cancer Control (UICC) classification defines R0 as no tumor remnant with clear microscopic margins [236]. The Union for International Cancer Control (UICC) classification defines R1 as the presence of microscopic tumor remnant on a surgical margin [236]. The Union for International Cancer Control (UICC) classification defines R2 as the presence of macroscopic tumor remnant during surgery [236].

Specific Tumor Classifications

Vascular: Vascular bone tumors are classified into hemangioma, hemangioendothelioma, angiosarcoma, and their epithelioid variants [17]. The international classification distinguishes between vascular tumors and malformations, which have radically opposite evolutive profiles [23]. Vascular tumors of the hand are classified into benign, congenital, and malignant categories [170]. Vascular leiomyomas in the hand have three pathologic types: solid, cavernous, and venous type tumors [165].

Other Considerations: The PUMCH classification for extremity and pelvic hemophilic pseudotumors is based on anatomic pathology and surgical strategy [193]. 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 [197]. Lipoblastoma and infiltrating lipoma are classified as benign fatty tumors despite their rapid growth [161]. Neurilemmomas and neurofibromas are commonly associated in Von Recklinghausen's disease but have not been reported in association outside this syndrome [162]. Confirming PIK3CA mutation status allows for more accurate diagnosis and classification of isolated macrodactyly based on its molecular pathogenesis [200]. A lesion previously considered malignant is now classified as an intermediate type of tumor to better describe its clinical indolent behavior [36].

Clinical Presentation

General Diagnostic Principles

A thorough history and physical examination, followed by appropriate imaging studies, establishes a correct diagnosis and determines treatment recommendations for soft-tissue tumors in children [21]. The history must elicit the duration of symptoms and accurately identify the size and location of the mass [94]. For benign-appearing hand and finger tumors, a pertinent history, physical examination, and plain radiographs are frequently sufficient to determine diagnosis and treatment [120, 134]. Clinical diagnosis of tumorous conditions is accurate in 94% of cases [105]. Awareness of tumor characteristics, including updated knowledge of the nature and demographic characteristics of hand tumors, is crucial for accurate diagnosis and appropriate treatment [79, 6]. History, clinical examination, imaging, and histopathological examination are essential for diagnosing rare tumors [3]. Tumor-like conditions of the hand and upper extremity typically present as masses that can be confused with more serious conditions; diagnosis often relies on clinical examination and imaging, with excisional biopsy required for definitive diagnosis to guide treatment [80]. Clinical and pathologic diagnosis may be difficult for granular cell tumors in the hand [88]. Shared clinical, epidemiological, and imaging characteristics with other soft tissue tumors create diagnostic difficulties [91]. Diagnostic delay or inappropriate treatment may result if patients do not receive appropriate follow-up, biopsy (usually open), or referral when the diagnosis is in doubt [29]. 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 uncommon tumors in adult patients is essential to prevent mistaken diagnosis and subsequent extensive workup [101].

Bone Tumors

Patients with bone tumors most frequently present with pain [107]. Pain may initially be activity-related, but patients with bone malignancy often complain of progressive pain at rest and at night [107]. Patients with benign bone tumors may also have activity-related pain if the lesion is large enough to weaken the bone [107]. Other benign lesions, most notably osteoid osteoma, may cause night pain initially [107]. 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 [26]. 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 [104]. A solitary bone lesion in a patient with or without a history of cancer should be biopsied to obtain an accurate diagnosis [163]. Presuming a solitary lesion is a bone metastasis in an older patient may lead to the wrong operation, cause extensive contamination, and potentially compromise the life or limb of the patient if the lesion is actually a primary sarcoma of bone [163]. 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 [28]. If a more aggressive process is present, causing considerable pain, inflammation, tumor enlargement, or bony destruction, further diagnostic and staging studies are warranted before biopsy or a definitive surgical procedure [120, 134].

Soft Tissue Tumors

Patients with soft-tissue tumors rarely complain of pain but more often complain of a mass [107]. Exceptions to this rule are patients with nerve sheath tumors who have pain or neurologic signs [107]. Soft tissue sarcomas are malignant tumors that require prompt recognition, typically presenting as enlarging painless masses greater than 5 cm [87]. Pain, or lack thereof, is not indicative of malignancy in soft-tissue tumors [33]. 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, because of the relatively high probability that the mass is malignant [96]. Benign peripheral nerve tumors encompass a wide range of neoplasms and non-neoplastic tumor-like lesions [20]. Some benign peripheral nerve tumors have different levels of malignant potential, thus treating physicians should be aware of this to avoid possible pitfalls with devastating outcomes [20]. The tumor presented with characteristics similar to other intraneural tumors (slow growing, without symptoms of neuropathy) making preoperative diagnosis difficult [82]. 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 [109]. Diagnosis and management of neurofibromas affecting digital nerves are both difficult and demanding [85]. Definitive diagnosis of nerve tumors requires pathologic evaluation as MRI is not definitive [1]. Schwannomas treated by excision typically have few long-term deficits, whereas neurofibroma excision carries a higher risk of nerve dysfunction and pain worsening [1]. Benign tumors that are asymptomatic should be observed, while biopsies should be performed for tumors suspected to be malignant before starting definitive treatment [2]. Treatment should not begin until a neoplasm has been diagnosed definitively and the results of all preoperative studies, including biopsy, are verified [4]. The assumption that a problem can be “handled” by relatively simple extirpation in the absence of complete diagnostic data is neither justified nor defensible [4]. The risks associated with soft tissue extension from a tumor-contaminated field are too great [4]. 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]. Despite the benign nature of these tumors, multidisciplinary care is needed to provide combined treatment options [32]. Patients with GCT should be followed indefinitely, and referred promptly if new symptoms, particularly pain, emerge [83]. Signs of malignancy, such as rapid growth, increasing pain, or other atypical features, mandate a thorough workup, and surgical management should be considered [95]. Vigilance in monitoring for signs of malignancy is encouraged, even for seemingly benign tumors [95]. 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]. Surgical excision is the primary treatment for symptomatic tumors, with recurrence rates being rare following complete removal [5]. 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 [106]. The authors believe that recurrent symptoms following tumor excision most likely are due to the presence of a previously undetected tumor [22].

Glomus Tumors

Glomus tumors can present with variable pain, and a high index of suspicion is needed for diagnosis [25]. The suspicion of glomus tumor is based on clinical examination, primarily searching for localized pain [93]. Glomus tumors of the hand are well recognized for their diagnostic difficulty and the frequent delay between symptom onset and definitive treatment [31]. Previous studies have reported prolonged diagnostic delays, often extending over several years, largely due to the small size of the tumor and its nonspecific clinical presentation [31]. In the present study, a similarly prolonged delay was observed, with a mean duration of 8.6 years, indicating that delayed diagnosis remains a persistent clinical problem [31]. Glomus tumors most commonly arise in the subungual region [31]. In the present study, no clear difference in diagnostic delay was observed between subungual and non-subungual tumors [31]. The duration of symptoms was comparable between the two groups; however, this finding should be interpreted with caution, given the small sample size and the descriptive nature of the analysis [31]. These results suggest that factors other than tumor location, including clinical presentation, may influence the time to diagnosis [31]. MRI is commonly used in the evaluation of suspected glomus tumors and facilitates the detection and localization of small soft tissue lesions [31]. Its usefulness extends beyond typical subungual lesions, as it can detect tumors in non-subungual or atypical locations and may also aid in screening for multifocal disease [31]. MRI provides important information regarding tumor size, depth, and precise anatomical location, thereby contributing to accurate localization and surgical planning [31]. MRI findings are not always specific to glomus tumors, and the characteristic signal patterns may overlap with those of other benign soft tissue tumors of the hand [31]. Small lesions or tumors in certain anatomical locations may not be detected [31]. Of the 11 cases examined, there were nine cases of tumors located in the upper and lower limbs; patients presented with pain which had lasted for a few months to several years (mean duration: 14 years) prior to being admitted for surgery [113]. The patient was able to indicate the presumed tumor location due to the presence of pain at that site, which was confirmed by clinical inspection in 4 out of the 9 abovementioned cases [113]. The tumor appeared as a cutaneous mass with a dark red coloration of the skin in 2 out of the 4 clinically-confirmed cases [113]. Pain was initiated by touch in 7 out of the 9 cases, and also by cold in 8 out of the 9 cases [113]. In cases 10 and 11, the duration of symptoms was very long (20 and 25 years respectively) due to misdiagnosis related to the atypical site of the lesions [113]. A definitive diagnosis was established after the appearance of a subcutaneous tumefaction [113]. In the patient with type I neurofibromatosis who presents with a painful subcutaneous lesion, the diagnosis of glomus tumor must be considered [97]. Glomus tumor should be included in the differential diagnosis in neurofibromatosis patients who present with a painful lesion of the hand or finger [102].

Imaging and Classification

Plain radiographs are an excellent starting point for most bone tumors [33]. MRI is often helpful for soft-tissue tumors, bone masses with a soft-tissue component, and any malignant tumor [33]. Sonography provides images from a pattern of echoes transmitted and received [28]. This technique generally conveys less detail concerning precise anatomic relationships and tumor margins than CT and MR images [28]. Echo patterns of solid masses are nonspecific; however, deep, fluid-filled lesions (for example, ganglion cysts) can be easily differentiated from solid tumors [28]. Sonography is usually much less expensive than CT or MR imaging [28]. Classification of tumors is increasingly based on the molecular characteristics of tumor types, which improves diagnostic accuracy for tumors that have been difficult to classify on the basis of morphology alone [19]. The authors propose a classification scheme for vascular bone tumors that includes hemangioma, hemangioendothelioma, angiosarcoma, and their epithelioid variants [17]. Distinguishing soft-tissue and bone tumors is a key starting point to the treatment of a patient with a musculoskeletal tumor [33]. It is important to understand when various imaging techniques would be most helpful [33]. Understanding the key principles for performing a safe and effective musculoskeletal biopsy is paramount to care of patients with musculoskeletal tumors [33]. The biopsy of tumors must be taken quite seriously, as performing a poor biopsy can lead to devastating consequences for patients [33]. One can diagnose the vast majority of patients without invasive testing with the appropriate use of radiographs and judicious use of advanced imaging modalities [33]. When clinical presentation and imaging fail to provide an answer, biopsy often with molecular testing is an extremely useful tool [33]. Local imaging studies, including bone scans, angiograms, CT, and MRI, are helpful in surgical planning [120, 134]. 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 [120, 134]. The diagnostic strategy to be used for a bone tumor depends on the ability of the clinician to make an accurate differential diagnosis on the basis of clinical information and plain radiographs [96]. The clinician must be able to classify the patient as having a non-progressive or a progressive primary benign bone tumor, a primary malignant bone tumor, or a metastatic bone tumor [96]. Only after assignment to one of these four categories can an effective diagnostic strategy ensue [96]. If the 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]. Plain radiographs are the most useful modality to determine whether the mass is arising in the soft tissues or in bone [116]. Magnetic resonance imaging gives the best resolution both to characterize the lesion and to define the anatomical features [116]. The biopsy must be planned carefully to avoid complications [116]. Soft-tissue lesions should be reviewed closely by an experienced musculoskeletal pathologist [116]. Treatment is based on the biological activity of the particular lesion [116]. A careful, coordinated evaluation, involving the physician, radiologist, and pathologist, is necessary to avoid incorrect diagnoses and inappropriate treatment [116]. Correct treatment must always take into consideration the location and size of the tumor, the histologic grade and clinical behavior, and the potential for metastases [28]. Comparison of survival rates resulting from various surgical treatments is difficult due to the small numbers of cases of anyone tumor type, varying histologic and anatomic gradings or stagings, and differences in surgical nomenclature used to describe techniques of tumor excision [28]. A classification scheme based on a modified staging system for bone sarcomas was established by the American Joint Committee on Cancer and End Results Reporting in 1977 [28]. It was proposed by W. F. Enneking at the University of Florida and agreed upon by the Musculoskeletal Tumor Society in 1979 [28]. According to the University of Florida group, a good surgical staging system would be one that: (1) [28]. Uniform use of the Enneking terminology, as recommended by the MSTS, provides such a means of communication [4]. Hand surgeons who undertake management of a neoplasm must have a thorough understanding of the tumor’s biologic behavior, its patterns of local and metastatic spread, and its response to radiotherapy and chemotherapy [4]. Radiation therapy and chemotherapy may allow the surgeon to use less radical tumor margins so that amputation can be avoided and function maintained [4]. The need for unnecessary sacrifice of tissue must be balanced against the need for adequate resection because local recurrence of sarcoma sometimes carries a grave prognosis [4]. In the management of malignancies of the upper limb, preservation of function is secondary to eradication of the disease process [4]. Nonaggressive, benign neoplasms are usually removed simply by marginal or intralesional operations such as curettage [4]. If there is a question about the adequacy of resection of the tissue margins and the diagnosis is already confirmed, a frozen section may be of great assistance for intraoperative sampling of the margins [4]. Age may be the most important information obtained in the history, however, because most benign and malignant musculoskeletal neoplasms occur within specific age ranges [107]. Although some tumors show a sex predilection (e.g., female predominance with giant cell tumors), this is rarely of diagnostic significance [107]. Race likewise is of little significance, with the exception that Ewing sarcoma is exceedingly rare in individuals of African descent [107]. Family history occasionally can be helpful, as in cases of multiple hereditary exostosis (autosomal dominant inheritance) and neurofibromatosis (autosomal dominant inheritance) [107]. Patients may present to the orthopaedic oncologist with pain, a mass, or an abnormal radiographic finding detected during the evaluation of an unrelated problem [107]. An adequate history and physical examination are the first steps in evaluating a patient with a musculoskeletal tumor [107]. The current concepts and difficulties in diagnosing these tumours are discussed [114]. It must be considered in the differential diagnosis of tumors of the extremities [103]. Awareness of tumor features may help physicians with diagnosis, and awareness of recurrence rates is important when counseling patients [111]. This review aims to provide a comprehensive picture of benign bone tumors frequently encountered in clinical practice, covering their classification, clinical presentation, imaging features, and therapeutic options based on the best-published literature [8]. This chapter provides a comprehensive overview of the demographics, clinical presentation, imaging, pathology, and treatment outcomes for various benign bone tumors and reactive lesions, including osteoid osteoma, osteoblastoma, and giant cell tumors [115]. The aim of this work was to review the principles for diagnostic and therapeutic management of hand tumors and the most commonly encountered clinical entities to limit the risk of errors and to optimize their care [30]. It is important to be familiar with the wide variety of benign tumors that may present in everyday hand surgery practice [95]. The treatment of many of these tumors in the hand is based on studies with

Investigations

General Principles and 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 [18]. Patients often have difficulty accurately describing their symptoms and may incorrectly attribute pathology to a perceived deficit, whether real or imagined [18]. The task of the clinician is to combine patient history with a careful physical examination to pinpoint or at least narrow the scope of possible pathologic processes [18]. History, clinical examination, imaging, and histopathological examination are crucial in the diagnosis of rare nerve tumors such as ancient schwannomas [3]. Updated knowledge and understanding of the nature and demographic characteristics of tumorous conditions of the hand is crucial for accurate diagnosis and appropriate treatment [6]. The principles for diagnostic and therapeutic management of hand tumors aim to limit the risk of errors and optimize care [30]. Once diagnosed, pediatric soft tissue tumor cases should be discussed at a tumor board meeting [7].

Imaging Modalities

Plain radiography: Plain radiographs are diagnostic for most bony lesions [123]. For all tumor types, plain radiographs were frequently rated as helpful studies [124]. 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 computed tomography and magnetic resonance imaging may be necessary in some cases [181]. 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]. However, 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 [172].

MRI: MRI is the preferred modality for the staging of soft tissue tumors and for evaluating patients for tumor recurrence [127]. MRI is to be preferred over CT in the preoperative evaluation of soft-tissue tumors due to better spatial orientation and sharper contrast between tumor and adjacent tissues [151]. Magnetic resonance imaging may be necessary to help differentiate a benign soft-tissue lesion from the rare malignant neoplasm [123]. For all tumor types, MRI was frequently rated as a helpful study [124]. In the coronal plane, magnetic resonance imaging accurately defined the intramedullary abnormality and provided greater interpretive certainty than computerized tomography or scintigraphy in defining the true proximal and distal extent of a tumor [180].

For specific tumor types, MRI was able to identify tumor type and determine tumor resectability in 80% of cases for single benign peripheral nerve tumors [45]. Ultrasound and MRI confirmed the neural nature of tumors in 90% of cases for single benign peripheral nerve tumors [45]. 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 [56]. MRI provides important information regarding tumor size, depth, and precise anatomical location for glomus tumors, contributing to accurate localization and surgical planning [31]. However, MRI findings are not always specific to glomus tumors, and characteristic signal patterns may overlap with those of other benign soft tissue tumors of the hand [31]. MRI confirmed the diagnosis and extent of the lesions in a case of glomus tumor of the hand [122]. Imaging examinations, particularly ultrasound and MRI, should be performed to support the diagnosis of glomus tumors [137]. High-resolution MRI is recommended for glomus tumors to ensure complete excision and decrease recurrence rates [154]. MRI examinations depicted glomus tumors in excellent detail and facilitated diagnosis in patients with atypical presentation [185].

MR imaging has become the modality of choice in evaluating synovial tumors and tumor-like lesions, as a presumptive diagnosis can be made in most cases [140]. A preoperative diagnosis based on radiographic and MRI characteristics of calcifying aponeurotic fibroma may prevent unnecessary biopsy [126]. The diagnosis of parosteal osteochondromatous proliferation (Nora's tumor) relies on radiological examination, ideally supplemented by MRI, and is confirmed by histological examination [164]. 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 [168]. Magnetic resonance imaging was helpful in delineating the lesion of tumoral calcinosis of the triangular fibrocartilage complex, but could not identify its nature [148]. MRI did not recognize the intravascular nature of an intravascular myopericytoma of the superficial palmar arch [188].

CT: For all tumor types, CT was frequently rated as a helpful study [124]. Improved investigation techniques, particularly the bone scan and tomography, have improved the diagnostic accuracy of osteoid osteoma, and a CT scan is useful for precise localization of the lesion [171].

Ultrasound: Ultrasound is congruent in diagnosis and size estimation when compared to MRI for subungual glomus tumors and subungual myxoid cysts [187].

Other Considerations: MRI should be used as a complementary method, while angiography remains necessary for specific cases involving highly vascularized tumors [152]. A high level of suspicion and histological study are required when there is an underlying history of malignancy [168].

Treatment

General Principles and Multidisciplinary Care

Management of musculoskeletal tumors requires an orderly protocol for diagnosis and surgical staging [16]. Multidisciplinary collaboration is mandatory for optimal patient care, particularly in adult soft-tissue sarcomas where coordinated work among trained pathologists, radiologists, medical oncologists, radiation oncologists, and surgeons is essential [73]. This multidisciplinary approach is also required for musculoskeletal desmoid tumors despite their benign nature [32], spine tumors requiring dynamic individualized follow-up [229], and malignant hand tumors managed by orthopaedic surgeons, medical oncologists, and radiation oncologists at cancer centers [234]. Treatment at specialized high-volume centers improves overall survival and reduces local recurrences for adipose tumors in the limbs [130] and provides slightly better overall survival for primary malignant bone tumors compared to low-volume facilities [173]. The field continues to advance through such multidisciplinary collaboration [204].

Biopsies must be performed for tumors suspected to be malignant before definitive treatment begins [2], and the biopsy procedure should be planned with the same care as definitive surgery [44]. Asymptomatic benign tumors are observed [2]. Surgery is an essential component of pediatric soft tissue tumor treatment, timed appropriately after potential systemic or local therapy [7].

Surgical Management: Benign Tumors

Indications: Surgery is indicated for symptomatic benign tumors, including those causing pain or functional impairment [5, 233]. Benign hand tumors in the distal upper limb have a clear indication for surgery based on patient-rated outcome measures [212]. For benign lesions with pathologic fractures, prompt simultaneous management of the fracture and tumor is required to minimize recurrence and maximize recovery [199]. Conservative management may be justified for juvenile aponeurotic fibroma if patients do well, though surveillance is warranted to identify recurrence or metastasis early [61].

Surgical Approach / Technique: Surgical excision is the primary treatment for symptomatic perionychium tumors [5], intravenous pyogenic granuloma of the hand [46], and leiomyoma of the hand, which is uniformly curative as metastasis has never been reported [153]. Local excision is satisfactory for Nora’s lesion [169] and low-grade myofibroblastic sarcoma of the distal phalanx, provided free surgical margins are achieved and long clinical follow-up with imaging is maintained [14]. Curettage is the standard of care for symptomatic enchondromas of the hand, with most patients returning to full function [203]. For invasive skin tumors of the hand, surgical excision remains primary, though nonsurgical options exist for unresectable or low-grade cancers [158]. Surgical treatment of benign soft tissue chondromatous lesions of the hand should be locally extensive but conservative, even in cases of recurrence [201]. Complete en bloc excision is recommended for osteoid osteoma of the clavicle when conservative therapy fails [196].

Other Considerations: Early surgical treatment of enchondromas in Ollier Disease may limit growth of large lesions and facilitate future surgery, though longer-term follow-up is needed to confirm absence of recurrence and malignant transformation [54]. At 18 months following resection of fibrous hamartoma of the wrist in infancy, there is no evidence of recurrence [50]. Juvenile aponeurotic fibroma should be treated conservatively by repeated surgical removal rather than radical excision [191]. 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 [211]. Tumoral resection of lipofibromatous hamartoma of the radial nerve is justified only in the case of complete preoperative deficit [206]. Most osteochondromas in the upper limb are treated similarly to those in other skeletal regions, with surgical removal necessary only if pain or functional impairment occurs [233]. 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 versus 70% improvement rates in patients undergoing concurrent limb-lengthening procedures [233]. Forearm lengthening surgery for osteochondromas is associated with fixator use and severe complications, with four out of six patients requiring unplanned reoperations [233]. The non-surgical group for forearm osteochondromas had more significant functional and cosmetic deficits compared to the surgical group [233]. Benign tumors of the brachial plexus were treated with microsurgical techniques [223]. Secondary brachial plexus tumors were treated with neurolysis to palliate pain [223]. Surgery improves the quality of life in malignant brachial plexus tumor cases [223]. The average time between onset of symptoms and surgery for glomus tumors of the fingers was 8 years 6 months [228]. The VAS average for preoperative pain for glomus tumors was 8.7, whereas it was 0.8 postoperatively [228]. The cessation of pain was immediate in all patients at the postoperative consultation for glomus tumors [228].

Surgical Management: Malignant Tumors

Indications: Wide tumor resection aimed at achieving R0 surgical margins remains the cornerstone of management for adult soft-tissue sarcomas [73]. 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 [35]. Treatment of localized malignant peripheral nerve sheath tumours is surgical excision with curative intent; however, the prognosis remains poor [234]. Malignant peripheral nerve sheath tumours have not been shown to be particularly radio- or chemo-sensitive; therefore, wide excision is the mainstay of treatment [234]. The 5-year survival rate for malignant peripheral nerve sheath tumours is approximately 40% [234]. Risk factors for malignant peripheral nerve sheath tumours include proximal location of the tumors, age older than 60 years, size greater than 5 cm, and the presence of NF1 [234].

Surgical Approach / Technique: Malignant primary bone tumors are rare and varied, requiring management tailored to specific tumor types [42]. High-grade malignant primary bone lesions typically undergo neoadjuvant therapies and surgery [42], while low-grade malignant primary bone lesions are usually managed with surgery alone [42]. 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 [147]. Surgical resection is the main treatment for intraperitoneal extraosseous osteosarcoma and the prognosis is poor [144]. 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 [146]. In the setting of nonmetastatic angiosarcoma in the upper extremity, amputation is the treatment of choice; however, this aggressive tumor uniformly has a poor survival rate [136]. 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 [129]. The early treatment that will increase survival prospects and may improve the chances of cure for sub-ungual melanoma is rarely achieved [166]. 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 [219]. 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 [207]. Primary tumors of the spine are best treated with an en bloc resection to minimize risk of local and distant metastases [229]. Surgery remains to be the first line of treatment for more systemically and locally aggressive cancer of the spine [229]. With impending neurologic compromise, more urgent need for surgery is recommended for spine tumors [229]. Malignant brachial plexus tumors were treated with large extirpations [223]. Aggressive surgical resection is highlighted for non-sacral pelvic Ewing sarcoma [204].

Other Considerations: There are four basic principles or goals of limb-sparing procedures: (1) Local recurrence should be no greater and survival no worse than with amputation, (2) The procedure, or treatment of its complications, should not delay adjuvant therapy, (3) Reconstruction should be enduring and not associated with a large number of local complications requiring secondary procedures and frequent hospitalizations, and (4) Function of the limb should approach that obtained by amputation [220]. Before consideration of limb preservation, the patient needs to be appropriately staged and assessed through a multidisciplinary approach [220]. An experience with multimodal therapy for sarcomas of the forearm and hand demonstrated a 91% survival rate, with an 8% rate of local recurrence [142]. No significant survival benefit was identified for individual tumors in the context of improved survival for sarcomas of the wrist and hand [156]. 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 [217].

Adjuvant and Non-Operative Therapies

Non-Operative: Treatment options for non-melanotic skin tumors of the upper extremity include surgical excision, destructive techniques, and medical therapies, with cure rates varying by tumor type, size, and histologic subtype [9]. Upfront surgery is not advantageous compared to more conservative treatments such as observation or medical treatment for patients with desmoid tumors [202]. 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 [138]. Unicameral bone cysts are often asymptomatic unless a pathologic fracture has occurred, and two thirds of patients present with fractures that can stimulate the cyst to heal [68]. Unicameral bone cysts in the flat bones are usually asymptomatic, found incidentally, and rarely fracture [68]. Unicameral bone cysts are most active during skeletal growth and usually heal spontaneously at maturity [68].

Adjuvant: Adjuvant radiation therapy is probably beneficial for local control of extra-abdominal desmoid tumors, but the precise indication for its use is not well defined [208]. Radiation therapy, either preoperatively or postoperatively, is recommended for high-grade, large, and deep-seated adult soft-tissue sarcomas [73]. To date, chemotherapy has marginal efficacy in the management of localized adult soft-tissue sarcoma disease and is considered in high-risk patients with large, deep, and high-grade lesions [73]. Multidrug neoadjuvant chemotherapy is usually initiated as appropriate after histologic diagnosis and staging for malignant bone tumors [220]. Chemotherapy helps control systemic disease by attacking micrometastases, dramatically increasing overall survival rates [220]. Neoadjuvant therapy also "sterilizes" the reactive zone around the tumor by destroying microscopic disease at the periphery of the primary lesion, thus facilitating resection [220]. The effectiveness of the chemotherapy regimen correlates with the extent of tumor necrosis [125].

Other Considerations: Other than tumor curettage surgery, tumor resection is the preferred option for tumor-induced osteomalacia [40]. Three treatment modalities are reported to be of value in the management of fibromatosis: surgery, irradiation, and drug treatment (chemotherapy or hormone therapy) [222]. There is controversy in the literature as to the appropriate treatment for fibromatosis, and this uncertainty is emphasized in the upper extremity where there is limited tissue present to provide for margins of resection [222]. 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 [222]. The benefits of radiation for fibromatosis are controversial [222]. Radiation treatment is withheld for patients whose initial tumors are located in sites that will not cause a major functional loss if the lesion recurs [222]. The use of preoperative irradiation offers potential benefit for tumors encompassing the brachial plexus [222]. The role of percutaneous sclerotherapy for benign bone tumors is highlighted in recent literature [204]. The prognostic value of preoperative imaging in osteosarcoma and chondrosarcoma is highlighted in recent literature [204]. The optimum treatment for Grade III giant-cell tumors of bone and patients with local recurrence is unclear [160]. Previous studies have regarded persistent bony defects as evidence of complete excision without recurrence for solitary enchondroma at the hand, however, in view of the slow asymptomatic growth of this tumour this opinion is incorrect [218]. Clinical and radiologic follow-up for malignant bone tumors treated with frozen autograft was done monthly for 6 months, then every 3 months for the next 12 months, then biannually until the end of the follow-up period [210]. Early mobilization was allowed in all patients treated with frozen autograft for malignant bone tumors [210]. For patients with lesions in the lower limb treated with frozen autograft, graduated weightbearing was allowed 12 weeks postoperatively [210]. Clinicians should note that elbow tumours present with unexplained and unremitting non-mechanical pain, swelling or fracture, and early specialist referral is recommended [209].

Complications

Nerve Tumors

Schwannoma: Enucleation is the standard procedure for resectable schwannomas [12], typically resulting in few long-term deficits [1]. Neurofibroma: These lesions require prolonged monitoring due to the possibility of recurrence [41]. For unresectable neurofibromas, epineurotomy is performed to prevent further deterioration [12]. Glomus Tumor: Recurrent symptoms following excision are most likely due to the presence of a previously undetected tumor [22]. Specifically, recurrent symptoms from digital glomus tumors may be attributed to small, synchronous, satellite lesions not detected during the original surgery rather than inadequate resection [240]. Fibrolipomatous Hamartoma: This lesion of the median nerve may remain unchanged in size and asymptomatic at 30 months of follow-up [64, 65].

Bone Tumors

Malignant Transformation: Secondary malignant tumors can develop up to twenty-five years after the initial presentation of a benign giant-cell tumor [69]. Malignant giant-cell tumors of bone are often secondary to previous therapeutic irradiation received four to thirty-nine years earlier [255]. Recurrence: Recurrence of extraskeletal soft tissue chondrosarcoma has been reported as early as two months and as late as fifteen years after the primary operation [76]. Recidivism of indolent myxomas of the hand suggests that long-term follow-up is required to monitor for local recurrence [190]. Treatment Outcomes: No tumor recurrence was found during more than 10 years of follow-up for benign bone tumours treated with sintered bone implantation [48]. No local recurrences were observed at a mean follow-up period of 54 months for giant cell tumors of the hand treated with curettage, cryosurgery, and cementation [66]. No local recurrence was observed at one year of follow-up for soft tissue chondromes of the hand [49]. No recurrence was observed at follow-up 1 to 8 years after surgery for subscapular elastofibroma [194]. No recurrence was observed at 5-year follow-up for desmoid tumors of the hand [192]. Pediatric Considerations: Benign bone tumors in children may simulate malignancy and require referral to an orthopaedic oncologist [26].

Malignant and Soft-Tissue Tumors

Diagnostic and Prognostic Factors: Diagnostic delay or inappropriate treatment may result if patients do not receive appropriate follow-up, biopsy, or referral when the diagnosis is in doubt [29]. Hand metastases can be the first sign of malignant disease [55]. 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 [67]. Squamous Cell Carcinoma: Recurrence of squamous cell carcinoma of the hand mostly occurs within 15 months, though it has been reported as late as 9 years post-operation [226]. Metastases followed disease recurrence in 67% of patients with squamous cell carcinoma of the hand [226]. Epithelioid Sarcoma: This tumor of the hand is characterized by recurrence and repeated operations, with diagnosis often made only after three or four excisions or after death [215]. Treatment failures include local recurrence, regional metastases, and distant metastases [72]. No evidence of local recurrence or metastatic dissemination was observed after 2 years of follow-up for epithelioid sarcoma in the median nerve [195]. Neurofibrosarcoma: Early diagnosis and radical surgical excision offer the best chance of long-term survival from neurofibrosarcoma of the upper extremity [74]. Five patients died of metastases an average of 3 years after diagnosis in a series of neurofibrosarcomas of the upper extremity [74]. Other Malignancies: Short-term prognosis for metastatic tumours of the hand is poor with a median survival of 7 months [55]. Long-term clinical follow-up of 10 years is advised for soft-tissue sarcomas of the hand as they should not be deceived by their smaller size [11]. Regular oncologic follow-up is necessary for primary malignant bone tumors of the hand [37]. Long-term follow-up is indicated for granular cell schwannoma of the hand due to reported cases of malignant or locally invasive behavior [53]. Recurrence is unlikely at 5-year follow-up for Merkel cell tumors of the hand [189]. Pediatric Soft Tissue Tumors: Regrowth of tumor or persistent disease occurred in 72% of patients with pediatric soft tissue tumors classified as lipofibromatosis [249]. No tumor has given rise to metastases in the reported series of pediatric soft tissue tumors with an admixture of adipose tissue and fibroblastic elements [249].

Recovery

Other Considerations: The evidence base for hand and upper-extremity tumors does not provide specific timelines for light activity, full activity, or complete recovery phases. Consequently, standard return-to-work or sport timelines cannot be established from this data. Recovery outcomes are instead characterized by long-term surveillance requirements, functional preservation metrics, and recurrence monitoring intervals.

Nerve Tumors

Neurofibroma excision carries a higher risk of nerve dysfunction and pain worsening [1]. Enucleation is performed for resectable tumours (schwannomas) [12], while epineurotomy is performed for unresectable tumours (neurofibromas) to prevent further deterioration [12]. Neurofibromas require prolonged monitoring due to possible recurrence [41]. The possibility of sequential appearance of neurilemomas indicates that routine follow-up examination of patients is important [257]. In cases of segmental neurofibromatosis, postoperative grip strength and movements were normal, and no tumour recurrence was found at 1-year follow-up [183]. For a fibrolipomatous hamartoma of the median nerve, the size was unchanged and the patient was without symptoms at follow-up 30 months later [64, 65]. The long clinical course of a cellular schwannoma (60 years) showed a lack of any significant growth or evidence of metastases [77].

Benign Bone Tumors

Longitudinal radiographs allowed observation of the timing of the first appearance and the potential for resolution for each benign bone tumor type in asymptomatic children [13]. No tumour recurrence has been found during more than 10 years of follow-up for benign bone tumours treated with sintered bone implantation [48]. All patients with enchondromas treated with curettage and cemented internal fixation returned to their presurgical functional capability within 4 weeks, and none had local tumor recurrence at the most recent follow-up [179]. The benign clinical course of chondromyxoid fibroma, as emphasized in the original series and by follow-up studies, makes the differentiation from chondrosarcoma important [75]. At a mean follow-up period of 54 months there were no local recurrences for giant cell tumors treated with curettage, cryosurgery, and cementation [66]. The patient with a giant cell tumor of the thumb metacarpal had 30° motion in both radial and palmar abduction and 90° flexion-extension at the interphalangeal joint of the thumb [34]. Range of motion of the nonamputated fingers was scored as normal and the patient showed a high degree of satisfaction following treatment for deforming chondrosarcoma secondary to long-term enchondromatosis [15]. Eight recurrences were noted (8.3%) in a review of 96 patients with giant cell tumors of the synovial sheaths, occurring in seven patients, with a mean recurrence delay of 2.75 years [277]. The rate of benign neoplasms was significant at >5 years follow-up duration (P = .016, 95% CI 0.001-0.005) but was not significant at 1 year or 1 to 5 years [280].

Soft Tissue and Other Benign Tumors

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 [47]. At 18 months following resection of a fibrous hamartoma of the wrist, there is no evidence of recurrence of the tumor [50]. At one year of follow-up, there was no local recurrence for chondrome des parties molles de la main [49]. 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 [276]. The patient with subungual keratoacanthoma is free of disease at 5½ years follow-up [275].

Malignant Tumors and Sarcomas

Even with adequate oncologic treatment, long-term clinical follow-up (10 years) is advised for soft-tissue sarcoma of the hand as they should not be deceived by their smaller size [11]. Long-term follow-up is indicated for granular cell schwannoma due to a few cases of malignant or locally invasive behavior reported [53]. Since recurrence has been reported as early as two months and as late as fifteen years after the primary operation for extraskeletal soft tissue chondrosarcoma, all patients should be carefully followed [76]. A multi-disciplinary approach with long-term follow-up is recommended to monitor for local recurrence of intraosseous myoepithelioma [184]. Grip strength recovered to 70% of the normal side following treatment for primary osteogenic sarcoma of a finger proximal phalanx [57]. At the 5-year follow-up, the patient with a well-differentiated liposarcoma in the digit showed no evidence of local recurrence or metastasis, with no loss of hand function [62]. After aggressive surgical management with negative margins for epithelioid sarcoma of the hand, 71% of the patients were alive without evidence of disease at the last followup, with a 5- and 10-year survivorship of 85% [72]. Early diagnosis and radical surgical excision offer the best chance of long-term survival from neurofibrosarcoma, a highly lethal cancer [74]. A secondary malignant tumor can develop as long as twenty-five years after the initial presentation of a benign giant-cell tumor [69].

Metastatic Tumors

Short-term prognosis for metastatic tumours of the hand was poor with a median survival of 7 months, and in two patients, hand metastases were the first sign of malignant disease [55]. A history of extensive recurrent disease well removed from the primary tumor is associated with a poor prognosis for extensive cutaneous metastatic breast carcinoma of the hand and upper extremity [67]. 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 [248]. The prognosis for patients with hand metastases is generally poor, with 85% dead at 1 year and 96% at 2 years [273]. Despite the tumor's initial response, intracranial metastases occurred 7 months later, and the patient died 15 months after presentation for a congenital primitive neuroectodermal tumor of the hand [262]. The prognosis for 5-year disease-free survival for sweat gland carcinoma of the hand is less than 30% [271].

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] This review aims to provide a comprehensive picture of benign bone tumors frequently encountered in clinical practice, covering their classification, clinical presentation, imaging features, and therapeutic options based on the best-published literature. [8] (10.3390/jcm11030699)
• [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)
• [L5] It is essential to follow an orderly protocol for appropriate diagnosis and surgical staging of tumors. [16] (10.1016/s0363-5023(83)80277-9)
• [L4] The authors propose a classification scheme for vascular bone tumors that includes hemangioma, hemangioendothelioma, angiosarcoma, and their epithelioid variants. [17] (10.1007/s00256-012-1510-6)
• [L5] Classification of tumors is increasingly based on the molecular characteristics of tumor types, which improves diagnostic accuracy for tumors that have been difficult to classify on the basis of morphology alone. [19] (10.1097/pap.0000000000000284)
• [L5] [20] (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. [21] (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. [22] (10.1016/s0363-5023(79)80076-3)
• [L5] The international classification distinguishes between vascular tumors and malformations, which have radically opposite evolutive profiles. [23] (10.1016/j.main.2007.10.004)
• [Paper] Glomus tumors can present with variable pain, and a high index of suspicion is needed for diagnosis. [25] (10.7759/cureus.30038)
• [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. [26] (10.5435/00124635-199911000-00004)
• [L5] [28] (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. [29] (10.1097/blo.0b013e31815953a7)
• [L5] The aim of this work was to review the principles for diagnostic and therapeutic management of hand tumors and the most commonly encountered clinical entities to limit the risk of errors and to optimize their care. [30] (10.1016/j.otsr.2021.103153)
• [Paper] [31] (10.7759/cureus.107434)
• [L5] Despite the benign nature of these tumors, multidisciplinary care is needed to provide combined treatment options. [32] (10.5435/00124635-200804000-00002)
• [L5] The patient had 30° motion in both radial and palmar abduction and 90° flexion-extension at the interphalangeal joint of the thumb. [34] (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. [35] (10.1177/17531934241238739)
• [L4] It is now classified as an intermediate type of tumor, not a malignancy, that better describes its clinical indolent behavior. [36] (10.1055/s-0038-1675550)
• [L4] Regular oncologic follow-up is necessary. [37] (10.1016/s0363-5023(89)80054-1)
• [Paper] [38] (10.1051/sicotj/2017041)
• [L4] Other than tumor curettage surgery, tumor resection is the preferred options for these tumors. [40] (10.1186/s12891-015-0496-3)
• [L4] Neurofibromas require prolonged monitoring due to possible recurrence. [41] (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. [44] (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. [45] (10.1016/j.hansur.2016.10.098)
• [L5] Surgical excision is the treatment of choice and the prognosis is excellent. [46] (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. [47] (10.1016/s0363-5023(97)80139-6)
• [L4] No tumour recurrence has been found during more than 10 years of follow-up. [48] (10.1016/s0266-7681(99)90055-4)
• [L5] At one year of follow-up, there was no local recurrence. [49] (10.1016/j.main.2004.04.008)
• [L5] At 18 months following resection, there is no evidence of recurrence of the tumor. [50] (10.1016/s0363-5023(86)80235-0)
• [L4] Long-term follow-up is indicated due to a few cases of malignant or locally invasive behavior reported. [53] (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. [54] (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. [55] (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. [56] (10.1016/j.hansur.2016.08.001)
• [L5] Grip strength recovered to 70% of the normal side. [57] (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. [58] (10.2106/00004623-198264080-00002)
• [L5] Wide surgical excision remains the standard of care for definitive treatment with adjuvant radiation utilized for larger and deeper lesions. [59] (10.3390/curroncol28030177)
• [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. [61] (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. [62] (10.1142/s242483551872027x)
• [L4] The absence of neurofibroma and NF2 criteria makes schwannomatosis a diagnosis of exclusion. [63] (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. [64] (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. [65] (10.1016/0266-7681_87_90018-0)
• [L4] At a mean follow-up period of 54 months there were no local recurrences. [66] (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. [67] (10.1016/j.jhsa.2006.10.022)
• [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. [69] (10.2106/00004623-198971050-00019)
• [L5] [70] (10.5435/jaaos-d-23-01020)
• [L5] [71] (10.5435/00124635-200009000-00003)
• [L4] [72] (10.1097/01.blo.0000150317.50594.96)
• [L5] [73] (10.2106/jbjs.21.00196)
• [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)
• [Paper] In the patient with type I neurofibromatosis who presents with a painful subcutaneous lesion, the diagnosis of glomus tumor must be considered. [97] (10.1097/gox.0000000000000144)
• [L5] Awareness of this uncommon tumor in adult patients is essential to prevent mistaken diagnosis and subsequent extensive workup. [101] (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. [102] (10.1016/j.jhsa.2013.05.025)
• [L5] It must be considered in the differential diagnosis of tumors of the extremities. [103] (10.1016/j.main.2012.07.007)
• [L4] The clinical diagnosis of the tumorous condition was accurate in 94% of the cases. [105] (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. [106] (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. [109] (10.1177/1753193408092039)
• [L4] Awareness of tumor features may help physicians with diagnosis, and awareness of recurrence rates is important when counseling patients. [111] (10.1177/1558944720922921)
• [L4] [113] (10.1016/s1297-3203(00)73487-1)
• [L5] The current concepts and difficulties in diagnosing these tumours are discussed. [114] (10.1016/j.jhse.2007.04.001)
• [L5] [116] (10.2106/00004623-199601000-00018)
• [L4] MRI confirmed the diagnosis and extent of the lesions. [122] (10.1016/j.hansur.2017.10.207)
• [L5] Plain radiographs are diagnostic for most bony lesions, whereas magnetic resonance imaging may be necessary to help differentiate a benign soft-tissue lesion from the rare malignant neoplasm. [123] (10.5435/00124635-200303000-00007)
• [L4] For all tumor types, MRI, CT, and plain radiographs were frequently rated as helpful studies. [124] (10.5435/jaaosglobal-d-25-00015)
• [L5] The effectiveness of the chemotherapy regimen correlates with the extent of tumor necrosis. [125] (10.5435/jaaos-21-08-480)
• [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. [126] (10.1016/j.jhsa.2006.04.011)
• [L4] Magnetic resonance imaging is the preferred modality for the staging of soft tissue tumors and for evaluating patients for tumor recurrence. [127] (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. [129] (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. [130] (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. [136] (10.1177/1558944717702466)
• [L5] Imaging examinations, particularly ultrasound and MRI, should be performed to support the diagnosis. [137] (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. [138] (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. [139] (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. [140] (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. [142] (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. [143] (10.1016/j.jhsa.2008.05.033)
• [Case_report] Surgical resection is the main treatment and the prognosis is poor. [144] (10.1186/s12891-020-03429-5)
• [L5] Surgical resection of the tumor is the treatment of choice. [145] (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. [146] (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. [147] (10.1016/s0363-5023(09)90033-8)
• [L5] Magnetic resonance imaging was helpful in delineating the lesion, but could not identify its nature. [148] (10.1016/s0363-5023(97)80177-3)
• [L3] 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. [151] (10.1007/bf00395801)
• [L4] MRI should be used as a complementary method, while angiography remains necessary for specific cases involving highly vascularized tumors. [152] (10.1007/bf00395793)
• [L5] Treatment is complete surgical excision, which is uniformly curative, as this tumor has never been reported to metastasize. [153] (10.1007/s11552-008-9143-x)
• [Case_report] They recommend using high-resolution MRI and ensuring complete excision to decrease recurrence rates. [154] (10.1111/sae.12041)
• [L4] The study suggests that the Musculoskeletal Tumor Society staging system is valid and should be applied to these infrequent malignancies to permit future statistical comparisons. [155] (10.1016/s0363-5023(85)80155-6)
• [L2] No significant survival benefit was identified for individual tumors. [156] (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. [157] (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. [158] (10.5435/jaaos-d-14-00040)
• [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. [159] (10.5435/jaaos-d-17-00055)
• [L4] The optimum treatment for Grade III lesions and patients with local recurrence is unclear. [160] (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. [161] (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. [162] (10.1016/0266-7681(88)90063-0)
• [L4] The diagnosis relies on radiological examination, ideally supplemented by MRI, and is confirmed by histological examination. [164] (10.1016/j.main.2005.08.014)
• [L4] There are three pathologic types—solid, cavernous, and venous type tumors. [165] (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. [166] (10.1016/0266-7681(90)90003-m)
• [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. [168] (10.1016/j.jhsa.2020.03.014)
• [L5] Local excision of the tumour appears to be a satisfactory method of treatment. [169] (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. [170] (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. [171] (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. [172] (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. [173] (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. [174] (10.1016/j.jhsa.2010.08.014)
• [L4] Ultrasound is an acceptable imaging modality for diagnosis, and surgical excision is the treatment of choice. [175] (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. [179] (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. [180] (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. [181] (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. [182] (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. [183] (10.1177/1753193413517807)
• [L4] A multi-disciplinary approach with long-term follow-up is recommended to monitor for local recurrence. [184] (10.1177/1753193416676229)
• [L4] The MRI examinations depicted the tumors in excellent detail and facilitated diagnosis in the two patients with atypical presentation. [185] (10.1016/0363-5023(92)90353-q)
• [Paper] US is congruent in diagnosis and size estimation when compared to MRI. [187] (10.1159/000530397)
• [L5] It is also concerning that MRI did not recognise the intravascular nature of the lesion. [188] (10.1016/j.jhsb.2007.01.011)
• [L4] With both patients at 5-year follow-up, recurrence is unlikely. [189] (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. [190] (10.1053/jhsu.2000.9412)
• [L5] It should be recognized and treated conservatively by repeated surgical removal rather than radical excision. [191] (10.1016/s0363-5023(77)80120-2)
• [L4] A 5-year follow-up showed no recurrence. [192] (10.1016/s0363-5023(83)80064-1)
• [L4] The PUMCH classification is based on the anatomic pathology and surgical strategy for HPTs. [193] (10.2106/jbjs.22.00781)
• [L4] No recurrence was observed at follow-up 1 to 8 years after surgery. [194] (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. [195] (10.1177/15589447211030689)
• [L5] Complete en bloc excision of the lesion has been the recommended treatment for failure of conservative therapy. [196] (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. [197] (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. [198] (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. [199] (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. [200] (10.1177/1753193418770366)
• [L4] Surgical treatment, even of recurrence, should be locally extensive but conservative. [201] (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. [202] (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. [203] (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. [204] (10.2106/jbjs.20.01510)
• [L5] Tumoral resection can only be justified in this benign context in the case of complete preoperative deficit. [206] (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. [207] (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. [208] (10.2106/00004623-199606000-00007)
• [L4] Clinicians should note that elbow tumours present with unexplained and unremitting non-mechanical pain, swelling or fracture, and early specialist referral is recommended. [209] (10.1177/1758573215586151)
• [L4] [210] (10.1097/corr.0000000000002788)
• [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. [211] (10.1016/s0749-0712(21)00222-5)
• [L4] According to the results of PROM, benign hand tumors located within the distal upper limb have a clear indication for surgery. [212] (10.1016/j.jhsa.2015.06.078)
• [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. [215] (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. [217] (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. [218] (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. [219] (10.1016/s1297-3203(01)00023-3)
• [L4] [220] (10.5435/00124635-200301000-00005)
• [L4] [222] (10.1016/0363-5023(94)90283-6)
• [L4] [223] (10.1016/s0266-7681(96)80262-2)
• [L4] [226] (10.1016/s0363-5023(88)80018-2)
• [L4] [228] (10.1016/j.main.2014.07.001)
• [L4] [233] (10.1530/eor-2025-0149)
• [L5] [234] (10.5435/jaaos-d-20-00333)
• [Paper] [236] (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. [240] (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. [248] (10.1016/s0363-5023(09)91071-1)
• [L4] [249] (10.1097/00000478-200011000-00004)
• [L4] [255] (10.2106/00004623-198668070-00016)
• [L4] The possibility of sequential appearance of tumors indicates that routine follow-up examination of patients is important. [257] (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. [262] (10.1016/s0363-5023(97)80140-2)
• [L5] The prognosis for 5-year disease-free survival is less than 30%. [271] (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. [273] (10.1016/0363-5023(94)90189-9)
• [L5] The patient is free of disease at 5½ years follow-up. [275] (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. [276] (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. [277] (10.1016/j.main.2012.10.120)

See Also

• Trigger Finger
• Neuropathy

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