Figure A shows an erythematous, ill defined, multi-nodular firm mass with areas of ulceration. Figures B and C shows the characteristic appearance of epitheliod sarcoma with a multi-nodular granulomatous pattern and areas of central necrosis.
Epitheliod sarcomas are the most common soft tissue sarcoma in the hand. They typically occur on the upper extremities of young adults. They have a tendency to spread locally by way of lymphatics or along fascial planes. Diagnosis is typically confirmed with biopsy that shows distinct nodular aggregates of epithelioid and spindle cells with zonal necrosis. It exhibits immunohistochemical reactivity for epithelial markers including keratin, vimentin and CD34. Treatment is wide surgical excision with adjuvant radiotherapy, and inadequate excision typically results in recurrence.
In this case, the clinical photo is quite concerning. It should be noted that initial appearance may often be benign and is frequently misdiagnosed. At time of presentation 30% of cases have metastasized, further reinforcing the importance of correct diagnosis upon initial presentation.
Halling et al. describe clinical, pathologic, treatment and outcome features of 55 cases of epitheliod sarcoma. 27% of patients died of the disease despite surgical management. They also found a decreasing rate of recurrence when more aggressive surgical margins were taken.
Illustration A shows a more benign appearing example of epithelioid sarcoma.
Answer 1: While the histopathology may also be consistent with a rheumatoid nodule, his age and the location of the mass make epithelioid sarcoma the more correct answer.
Answer 2: Histopathology is not consistent with squamous cell carcinoma, as evidenced by lack of keratin "pearls."
Answer 4: Angiosarcoma biopsy would show well formed vascular channels.
Answer 5: Epidermal inclusion cysts typically have a more benign clinical appearance and a low risk of recurrence after excision.
Enzinger FM, Weiss SW: Soft Tissue Tumors, ed 3. St Louis, MO, Mosby, 1995, p 1074.
Halling AC, Wollan PC, Pritchard DJ, et al: Epithelioid sarcoma: A clinicopathologic review of 55 cases. Mayo Clin Proc 1996;71:636-642.
PMID:8656704 (Link to Abstract)