Malignant tumors comprise 1% to 2% of all hand tumors and are most common in middle-aged and elderly patients.1,2 A multidisciplinary approach involving an orthopedic surgeon, radiologist, pathologist, and radiation oncologist is necessary. The best chance for functional and aesthetic results is with early detection.3
Skin is the only tissue in the hand with an appreciable incidence of malignancy.4 Squamous cell carcinoma is the most common malignancy in the hand, and basal cell carcinoma is the most common malignancy in humans. Other skin tumors include melanomas and nonmelanotic tumors such as sweat gland tumors, Merkel cell carcinoma, dermatofibrosarcoma protuberans, and Kaposi's sarcoma.
Squamous cell carcinoma
Squamous cell carcinoma is the most common malignant skin tumor of the hand and represents 58% to 90% of all hand malignancies.4-8 Squamous cell carcinoma varies in appearance from a small, round, red, slightly elevated lesion to a large ulcerated lesion and is usually located on the dorsum of the hand and fingers, interdigital skin, dorsal wrist, and subungual areas. The incidence of squamous cell carcinomas peaks in the sixth decade with a male to female ratio of 4:1.7-10 Sun and radiation exposure,2-11 immunosuppression, and Marjolin's ulcers (burn wounds) are risk factors for cancer development. Marjolin's ulcer applies to malignant degeneration in chronic ulcers of the skin and sinuses, especially in burn scars.12 Because of the 5.5% to 28% risk for tumor metastasis demonstrated in series restricted to the hand,4,5,7,8 careful examination of regional lymph nodes is imperative.
Excision is curative especially for the early form of squamous cell carcinoma, Bowen's disease, squamous cell carcinoma in situ, and dyskeratotic, scaly, papular lesions.13 Ten percent of these lesions progress to invasive squamous cell carcinoma.14 Small tumors can be excised locally with a margin of normal tissue and usually require skin graft or flap closure. Extensive excision or amputation of digits, rays, or the entire hand may be required in deep, ulcerated, recurrent, or large lesions. Lymph node dissection is required if nodes are palpable or the lesion is recurrent, ulcerated, fixed, or located over moving parts. If surgical excision is inadequate or contraindicated, radiation therapy is palliative.
Squamous cell carcinoma in the nail area may be treated with appropriate amputation. Because of the slow-growing nature of this carcinoma, wide excision is occasionally preferable in cases in which deeper tissues are uninvolved. Reconstruction may be performed with a dermal graft, but if periosteum is involved, then amputation is necessary.
Basal cell carcinoma
Basal cell carcinoma is the second most common malignant tumor of the hand. Basal cell carcinoma presents as a raised erythematous tumor with a pearly border on sun-exposed areas. Although slow growing and similar to benign tumors in behavior, basal cell carcinomas are locally destructive and sometimes metastasize. They primarily occur in middle-aged and elderly patients and have less than a 1% recurrence rate if surgically removed.15,16 Because basal cell carcinomas are most commonly related to sun exposure, avoidance of sun exposure is an excellent preventative measure. Proper treatment of upper extremity lesions is surgical excision with 5 mm of normal marginal tissue. Other modalities include Mohs surgery, radiation, and other forms of destructive therapy.
Malignant melanomas are common skin tumors of the upper extremity in middle-aged patients and frequently involve the fingertips and subungual areas. Malignant melanoma usually presents as a pigmented lesion that may have undergone recent change in size, color, or regularity of border. Certain nevi are at risk, including the giant congenital hair variety and dysplastic nevi. Dysplastic nevi (moles) larger than 5 mm in size are more likely to transform to a malignant melanoma. Sunlight exposure, genetic predisposition, and certain dysplastic nevi are risk factors for the development of malignant melanoma. Pigmented lesions with irregular borders, asymmetry, variation in pigmentation, ulceration, or changes in preexisting pigmented tumor must be investigated.
Acrolentiginous melanomas are found in the palm and nail bed areas. Acrolentiginous melanomas, presenting as flat lesions, grow fast and are found in older patients. Delay in diagnosis occurs due to difficulty in differentiating it from hemorrhage or infection. Linear pigmented nail streaks in subungual locations are seen mainly in black patients and may represent melanocytic striae.
Treatment depends on Clark's classification (Table 1), depth of tumor extension, and lymph node involvement. Finger amputation or ray resection is required in finger involvement. Prognosis is good if superficial lesions are detected early and resected appropriately.13 A 2-cm margin can frequently avoid local recurrence (which is a bad prognostic sign). For tumors smaller than 1 mm thick, a 1-cm margin is justified. Five percent of patients with malignant melanomas develop a second primary melanoma and thorough regular follow-ups are needed.
Table 1. Clark's grading for melanomas
|Level I: Tumor confined to epidermis (in situ)
|Level II: Penetration into the papillary dermis
|Level III: Lesion fills and expands the papillary dermis
|Level IV: Tumor invades the reticular dermis
|Level V: Tumor extends into subcutaneous tissue.
Dermatofibrosarcoma protuberans is a rare tumor of the dermis that occurs in men between the ages of 20 and 40 years.17 Dermatofibrosarcoma protuberans presents as a variably colored, slow-growing nodule involving the subcutaneous tissue. This tumor has a low incidence of metastasis; however, the recurrence rate is high even with wide local excision.
Sweat gland tumors
Sweat gland tumors are rare and present as a slowly enlarging, painless, fixed mass on the fingers of middle-aged and elderly patients. There is histological variation from low malignant carcinoma, digital papillary adenoma, and adenoid cystic carcinoma to higher malignant potential eccrine poroma, malignant cylindroma, and appocrine carcinoma. The prognosis after ray amputation or wide excision depends on lymph node involvement. Lymph nodes must be checked for potential metastases.
Soft tissue sarcomas arise from cells of mesenchymal origin. One-third of the 3,000 extremity sarcomas diagnosed per year are located in the upper extremity; the majority of the sarcomas are not located in the hand or wrist. Most soft tissue sarcomas present as a painless mass.18-21 Occasionally, patients present with either a painful mass or neurologic compromise. Rapid growth is typical of sarcomas. Plain radiographs may help define bone involvement and help differentiate a sarcoma from a primary bone tumor. Magnetic resonance imaging is superior in defining the anatomy of soft tissue tumors, but computed tomography scanning remains a better method of assessing calcification, ossific changes, and cortical involvement. Plain chest radiographs and high-resolution computed tomography are obtained to rule out metastasis to the lungs. Bone scan is generally not indicated because soft tissue sarcomas rarely metastasize to bone. Grade, size, depth, and presence or absence of metastasis have prognostic significance.22
Local treatment of sarcomas includes resection with or without radiation therapy.23 Surgical margins, as defined by Enneking, include intralesional, marginal, wide excision, and radical resection.24,25 Although local control is better with radical resection, there is no improvement in overall survival when compared to wide excision. Eighty to 90% success rates are achieved with the combination of wide excision and radiotherapy.23 Patients with hand sarcomas have a worse prognosis than patients with similar tumors in other extremities, possibly due to the difficulty of achieving a wide enough surgical margin without an amputation in the hand where important nerves, blood vessels, bones, and tendons may need to be sacrificed.
Epithelioid tumors are the most common soft tissue sarcoma of the forearm and hand,26,27 predominantly affecting adolescent and young adult males twice as often as females. Painless and slow growing, epithelioid tumors have a predisposition for fingers, hands, and forearms and can become ulcerated or develop a sinus. Metastases may already be present when diagnosis is made, thus prognosis is guarded and recurrence is possible several years after the initial excision.
There is a high incidence of proximal extension along fascial planes, tendon sheaths, and subcutaneous lymphatics. Treatment of lesions distal to the metaphalangeal (MP) joint and web space is either MP joint disarticulation or ray resection. Epitrochlear and axillary lymph nodes must be considered even in the absence of clinical and radiographical findings. Twenty-five percent of patients develop regional lymph node metastasis. Wide excision including any involved neurovascular structure or amputation is preferred for sarcoma of web space, dorsal, or palmar hand and wrist.
Synovial sarcoma is a common soft tissue sarcoma of the hand.28 Synovial sarcoma occurs in the second to fifth decades and presents as a painless, firm and deep slow-growing mass close to a joint, tendon, or bursa. It is commonly found in carpus but rarely in the fingers. Focal calcification, periosteal reaction, and bone erosion may be present radiographically. Synovial sarcoma appears as a poorly defined mass with a pseudocapsule often attached to tendons, the tendon sheath, or joint capsule with a mix of spindle and epithelial-type cells and different types of monophasic or biphasic cells. Because lymphatic spread is common, wide or radical excision with radiation or chemotherapy is usually recommended. Metastasis is possible even after several years.
Clear cell carcinoma
Clear cell carcinoma is so called because of the round or fusiform cells with clear cytoplasm found on histology. Also called malignant melanoma of soft parts, clear cell carcinoma tends to occur deep in the hand next to tendons and aponeuroses of young female adults. Recurrence and metastasis are common even with wide resection.
Originating from the primordial cell of striated muscle, rhabdomyosarcoma is one of the common soft tissue sarcomas of childhood and is more common in men than women.29 White people are affected three times more often than black people.30,31 The four cell types are botyroid (uncommon in hand), alveolar, pleomorphic, and embryonal.32,34 Rhabdomyosarcoma is slow growing and painless and has the potential for hematogenous spread. Prognosis remains grave even with surgical ablation and adjuvant chemotherapy.
Malignant fibrous histiocytoma
Malignant fibrous histiocytoma is the most common malignant adult soft tissue sarcoma. Its overall incidence has increased because many liposarcomas and fibrosarcomas are now categorized as malignant fibrous histiocytoma.35 Malignant fibrous histiocytoma is typically composed of a pleomorphic cell population of spindle histiocytes and giant cells in a storiform pattern. It typically presents as a painless soft tissue or bone mass usually in a man in the fifth to seventh decade of life. Local recurrence and lymphatic metastases are common. Treatment is wide local excision with radiotherapy. Radical resection of the entire compartment only reduces the rate of local recurrence but not overall survival rates.
Liposarcoma has a variable clinical presentation and histologic appearance. Liposarcoma is uncommon in the hand. The well-differentiated and myxoid forms are low-grade, nonaggressive tumors that rarely metastasize in contrast to the anaplastic pleomophic forms. Wide excision with radiotherapy is the treatment of choice.13
Malignant Vascular Lesions
The two types of malignant hemangiomas, angiosarcoma and Kaposi's sarcoma, are predominantly found in the extremities of white men. Angiosarcomas, which are red, can be cured with wide excision. Kaposi's sarcoma, on the other hand, present as bluish-red to dark-brown plaques or nodules that are painless. Kaposi's sarcoma is frequently present in human immunodeficiency virus-infected patients. Chemotherapy is the best treatment option.13
Malignant Nerve Tumors
Malignant schwannoma is sometimes called neurofibrosarcoma and is the most common malignant nerve tumor. Malignant schwannoma is common in patients with Recklinghausen's disease, occurring 20% of the time in the upper extremity. Patients are usually in their second or third decades. It presents with pain, but early detection is hindered because of its predilection for deep large nerves. Suspicion for malignant degeneration should be high in cases with a rapidly growing neurofibroma, a tumor more than 5 cm in size at diagnosis with ill-defined borders, or a tumor adherent to adjacent soft tissue. Histologically, malignant schwannoma appears fusiform or oblong in shape and has neoplastic Schwann's cells and nerve fascicles. If diagnosis is confirmed by incisional biopsy, then en bloc resection or amputation is necessary.13
- Johnson J, Kilgore E, Newmeyer W. Tumorous lesions of the hand. J Hand Surg Am. 1985; 10:284-286.
- Johnson TM, Rowe DE, Nelson BR, Swanson NA. Squamous cell carcinoma of the skin (excluding lip and oral mucosa). J Am Acad Dermatol. 1992; 26:467-484.
- Fleegler EJ. Skin tumors. In: Green DP, ed. Operative Hand Surgery. New York, NY: Churchill Livingstone; 1993:2173-2195.
- Haber MH, Alter AH, Wheelock MC. Tumors of the hand. Surg Gynecol Osbstet. 1965; 121:1073-1080.
- Ariyan S. Benign and malignant soft tissue tumors of the hand. In: McCarthy JG, May JW, Littler JW, eds. Plastic Surgery. The Hand. Philadelphia, Pa: WB Saunders; 1990(pt 2):5483-5509.
- Chakrabarti I, Watson JD, Dorrance H. Skin tumours of the hand. A 10-year review. J Hand Surg Br. 1993; 18:484-486.
- Forsythe RL, Bajaj P, Engeron O, Shadid EA. The treatment of squamous cell carcinoma of the hand. Hand. 1978; 10:104-108.
- Schiavon M, Mazzoleni F, Chiarelli A, Matano P. Squamous cell carcinoma of the hand: Fifty-five case reports. J Hand Surg Am. 1988; 13:401-404.
- Kendall TE, Robinson DW, Masters FW. Primary malignant tumors of the hand. Plast Reconstr Surg. 1969; 44:37-40.
- Leung PC. Tumors of the hand. Hand. 1981; 13:169-176.
- Vitaliano PP, Urbach F. The relative importance of risk factors in nonmelanoma carcinoma. Arch Dermatol. 1980; 116:454-456.
- Novick M, Gard DA, Hardy SB, Spira M. Burn scar carcinoma: A review and analysis of 46 cases. J Trauma. 1977; 17:809-817.
- Hochwald NL, Green SM. Tumors. In: Spivak JM et al, eds. Orthopaedics: A Study Guide. New York, NY: McGraw-Hill Companies, Inc.; 1999:709-716.
- Cottel WI. Skin tumors I: Basal cell and squamous cell carcinoma. [selected readings] Plastic Surgery. 1992; 7:1-34.
- Koplin L, Zarem HA. Recurrent basal cell carcinoma. A review concerning the incidence, behavior, and management of recurrent basal cell carcinoma, with emphasis on the incompletely excised lesion. Plast Reconstr Surg. 1980; 65:656-664.
- Shanoff LB, Spira M, Hardy SB. Basal cell carcinoma: A statistical approach to rational management. Plast Reconstr Surg. 1967; 39:619-624.
- McPeak CJ, Cruz T, Nicastri AD. Dermatofibrosarcoma protuberans. An analysis of 86 cases - five with metastasis. Ann Surg. 1967; 166:803-816.
- Brien EW, Terek RM, Geer RJ, Caldwell G, Brennan MF, Healey JH. Management of soft-tissue sarcomas of the hand. J Bone Joint Surg Am. 1995; 77:564-571.
- Campanacci M, Bertoni F, Laus M. Soft tissue sarcoma of the hand. J Orthop Ttraumatol Ital. 1981; 7:313-327.
- Dreyfuss UY, Boome RS, Kranold DH. Synovial sarcoma of the hand - A literature study. J Hand Surg Br. 1986; 11:471-474.
- Owens JC, Shiu MH, Smith R, Hajdu SI. Soft tissue sarcomas of the hand and foot. Cancer. 1985; 55:2010-2018.
- Terek RM, Brien EW. Soft-tissue sarcomas of the hand and wrist. Hand Clin. 1995; 11:287-305.
- Brennan MF, Casper ES, Harrison LB, Shiu MH, Gaynor J, Hajdu SI. The role of multimodality therapy in soft-tissue sarcoma. Ann Surg. 1991; 214:328-336.
- Enneking WF. A system of staging musculoskeletal neoplasms. Clin Orthop. 1986; 204:9-24.
- Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcomas. Clin Orthop. 1980; 153:106-120.
- Bryan RS, Soule EH, Dobyns JH, Pritchard DJ, Linscheid RL. Primary epithelioid sarcoma of the hand and forearm. J Bone Joint Surg Am. 1974; 56:458-465.
- Ahmed MN, Feldman M, Seemayer TA. Cytology of epithelid sarcoma [letter]. Acta Cytol. 1974; 18:459-461.
- Ariel M, Pack GT. Synovial sarcoma: Review of 25 cases. N Engl J Med. 1963; 268:1272.
- Maurer HM, Beltangady M, Gehan EA, et al. The Intergroup Rhabdomyosarcoma Study-I. Cancer. 1988; 61:209-220.
- Agamanolis DP, Dasu S, Krill CE Jr. Tumors of skeletal muscle. Hum Pathol. 1986; 17:778-798.
- Rosenberg AE, Schiller AL. Soft tissue sarcomas of the hand. Hand Clin. 1987; 3:247-261.
- Dick HM, Lee HD. Synovial sarcoma and rhabdomyosarcoma. In: Bogumill GP, Fleeger EJ, eds. Tumor of the Hand and Upper Limb. New York, NY: Churchill Livingstone; 1993.
- Enneking WF. Clinical Musculoskeletal Pathology. 3rd ed. Gainesville, Fla: University of Florida, Press/J. Hillis Miller Health Science Center; 1990.
- Enneking WF. Musculoskeletal Tumor Surgery. New York, NY: Churchill Livingstone; 1983.
- Weiss SW, Enzinger FM. Malignant fibrous histiocytoma: An analysis of 200 cases. Cancer. 1978; 41:2250-2266.