Epithelioid sarcoma of Enzinger

Abstract

Epithelioid sarcoma was named in 1970 in a classic paper by Enzinger, who expanded the observations in a larger series in 1985. He defined a sarcoma with a peak incidence in young adult males and a predilection for extremities, involving subcutis or deeper tissue and extending along tendon sheaths or aponeuroses. The tumor forms nodules with central necrosis surrounded by bland polygonal cells with eosinophilic cytoplasm and peripheral spindling. Fibromalike, angiomatoid, and proximal aggressive variants (with larger cells, prominent nuceloi, and rhabdoid change) have since been described. Epithelioid sarcomas regularly express vimentin, cytokeratins, and epithelial membrane antigen, and about half are positive for CD34, but a wide range of other antigens can be expressed. S100 protein, desmin, and FLI-1 are usually negative. The ultrastructure displays epithelial and mesenchymal features including myofibroblastic differentiation. There are no specific genetic findings but several cases display chromosomal abnormalities in the 22q region. The tumor has no normal cellular counterpart and differs from both synovial sarcoma and carcinoma. There is a wide differential diagnosis from numerous benign and malignant conditions, including granuloma annulare, melanoma, and epithelioid vascular neoplasms. Epithelioid sarcoma has a high recurrence rate, which can be reduced by adequate surgery, and up to 40% metastasize, to regional lymph nodes, to lung, and other locations including scalp. Adverse prognostic factors include large size, male sex, older age, necrosis, vascular invasion, rhabdoid cytomorphology, and inadequate excision. Thirty-six years after Enzinger's original account, epithelioid sarcoma remains a clinically and pathologically distinct, indolent but aggressive sarcoma of indeterminate lineage.