AAOS Comprehensive Orthopaedic Review

Section 9 - Hand and Wrist

Chapter 86. Bone and Soft-Tissue Tumors of the Hand and Forearm

I. Benign Soft-Tissue Tumors

A. Lipoma

1. Lipomas commonly occur subcutaneously or adjacent to nerves in the hand.

2. Deep masses should be assessed with MRI (

Figure 1).

3. Marginal excision is appropriate surgical treatment.

4. Recurrence is infrequent.

B. Lipofibromatous hamartoma

1. Peripheral nerve lesion most common in the median nerve when seen in the hand

2. May be associated with macrodactyly

3. Symptoms of nerve compression may be seen that respond short-term to decompression.

4. Natural history of this lesion is slow, progressive deterioration in nerve function.

5. Surgery is usually limited to biopsy of a nerve branch to establish diagnosis and nerve decompression.

6. Resection and nerve grafting has been suggested when this lesion is identified in children.

C. Giant cell tumor of tendon sheath

1. Second most common soft-tissue tumor seen in the hand, following ganglion cyst

2. Lesions are not always associated with the tendon sheath.

3. Most commonly seen on the palmar surface of the fingers, near the distal interphalangeal joint

4. Physical examination findings: firm, nodular, does not transilluminate

5. Pressure-type bone erosion may be seen in approximately 5% of patients.

6. Extension into joints and deep to the volar plate is common and may account for recurrence.

[Figure 1. A, Axial T1-weighted MRI scan of the hand of an elderly man shows a large, multilobulated, soft-tissue mass involving the thenar space, as well as both the flexor and extensor compartments with communication through the interosseous ligaments between the third and fourth metacarpals. The areas of high signal intensity (arrows) are diagnostic for lipoma. B, Diagram of same cross section shown in A with structures labeled.]

[

Figure 2. Oblique (left) and AP (right) views (A) and lateral oblique view (B) of the hand of a 22-year-old woman who presented with pain in the hand following a punch to an inanimate object. Lucency in the fifth metacarpal is well-defined and is a classic characteristic of an enchondroma.]

7. Marginal excision is appropriate surgical treatment.

8. Recurrence rate is approximately 30%.

D. Schwannoma

1. Most common nerve tumor affecting the upper extremity.

2. Most commonly occurs on the flexor surfaces

3. Most commonly seen as a firm, painless mass except with direct contact, which may produce Tinel sign

4. Marginal excision under magnification is appropriate surgical treatment.

5. Risk of permanent neurologic deficit following surgery is approximately 5%

E. Digital fibroma of infancy

1. Aggressive fibrous soft-tissue tumor of the digit most commonly occurring in infants younger than 1 year

2. Surgery may be indicated for progressive enlargement, contracture, or deformity.

3. Spontaneous resolution may be seen.

4. Recurrences frequent following marginal excision.

5. Wide excision and skin grafting may provide best local control.



II. Benign Bone Tumors

A. Enchondroma

1. Most common primary bone tumor arising in the hand (Figure 2)

2. The proximal phalanx is the most common location.

3. Surgery is indicated when the diagnosis is in question or when pathologic fracture has occurred or is at risk.

4. Surgical treatment is curettage with or without bone grafting, either autologous or allograft.

5. Enchondromas occasionally undergo malignant transformation to chondrosarcomas.

6. In Maffucci syndrome, multiple enchondromatosis is seen on plain radiographs with hemangiomas present as well (may be associated with phleboliths on plain radiographs); risk of bone and soft-tissue sarcomas is high.

B. Periosteal chondroma

1. Benign surface cartilage lesion most commonly seen in phalanges or metacarpals

2. Radiographic appearance demonstrates typical "saucerization" of the cortex with a thin rim of bone along the exterior surface of the lesion.

3. Treatment is marginal excision including a small amount of surrounding periosteum.

C. Osteochondroma

1. Most commonly seen at the distal aspect of the proximal phalanx

2. May be seen in subungual region and produce nail deformity

3. Marginal excision may be indicated when lesions are painful or result in deformity or limitation in range of motion.

D. Bizarre periosteal osteochondromatous proliferation (Nora lesion)

1. Surface proliferation of bone and cartilage arising from phalanx or metacarpal with aggressive radiographic and histologic appearance

2. May be confused with parosteal osteosarcoma

3. Surgical treatment is marginal excision.



III. Malignant Soft-Tissue Tumors

A. Squamous cell carcinoma

1. Risk factors include sun exposure/ultraviolet radiation as well as conventional radiation exposure, immunocompromised state, or prior transplantation.

2. The dorsum of the forearm and hand are common locations for this lesion.

3. Treatment is wide excision and possible skin grafting.

B. Subungual melanoma

1. This lesion may be seen in the nail bed of the thumb digits.

2. Biopsy-proven invasive lesions are best treated with sentinel lymph node biopsy and amputation at the interphalangeal joint.

C. Epithelioid sarcoma

1. Probably the most common soft-tissue sarcoma that arises in the hand

2. Long-standing finger nodule with recent ulceration is a common clinical presentation.

3. Lesion is unique for its propensity to spread proximally along tendons and tendon sheaths and to lymph nodes.

D. Synovial sarcoma

1. Lesions most commonly arise in close proximity to joints or tendon sheaths.

2. Intraneural lesions may be seen and are usually associated with pain.

3. Soft-tissue calcifications may be present on plain radiographs in approximately 20% of patients.



IV. Malignant Bone Tumors

A. Chondrosarcoma

1. Most common primary malignant bone tumor that arises in the hand

2. Most commonly seen in the proximal phalanx or metacarpal

B. Acral metastasis

1. Metastasis to the hand is most commonly seen in the setting of lung or breast cancer.

2. Usually seen late in the course of the disease but may be the presenting symptom.

3. Local treatment is aimed at controlling pain and maximizing function.



Top Testing Facts

1. Ganglions and giant cell tumors are the first and second most common soft-tissue tumors in the hand, respectively.

2. Enchondromas are the most common primary bone tumors in the hand.

3. Epithelioid sarcomas are the most common soft-tissue sarcomas arising in the hand.

4. Schwannomas are the most common nerve tumors in the upper extremity and they most commonly occur on the flexor surfaces.

5. Chondrosarcomas are the most common primary malignant bone tumors arising in the hand.

6. The most common acral metastases are seen in the setting of lung or breast cancer.

7. There are well-documented instances of malignant transformation of enchondromas to chondrosarcomas.

8. With Maffucci syndrome, multiple enchondromas and hemangiomas are seen on radiographs; risk of sarcoma is high.

9. Treatment for squamous cell carcinoma of the hand is wide excision and coverage (possible skin grafting).

10. Soft-tissue calcifications may be seen in synovial sarcomas.



Bibliography

Athanasian EA: Aneurysmal bone cyst and giant cell tumor of bone of the hand and distal radius. Hand Clin 2004;20:269-281.

Brien EW, Geer RJ, Caldwell G, Brennan MF, Healey JH: Treatment of soft-tissue sarcomas of the hand. J Bone Joint Surg Am 1995;77:564-571.

Lopez-Barea F, Rodriguez-Peralto JL, Garcia-Giron J, Guemes-Gordo F: Benign metastasizing giant-cell tumor of the hand: Report of case and review of the literature. Clin Orthop Relat Res 1992;274:270-274.

Mankin HJ, Lange TA, Spanier SS: The hazards of biopsy in patients with malignant primary bone and soft-tissue tumors. J Bone Joint Surg Am 1982;64:1121-1127.

Nahra ME, Bucchieri JS: Ganglion cysts and other tumor related conditions of the hand and wrist. Hand Clin 2004;20: 249-260.

Saint-Cyr M, Langstein HN: Reconstruction of the hand and upper extremity after tumor resection. J Surg Oncol 2006;94: 490-503.



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