Epithelioid sarcoma

Abstract

Epithelioid sarcomas are rare, mesenchymal tumors of unknown histogenesis and display multidirectional differentiation, which is predominantly epithelial. They have no normal cellular counterpart and differ from both synovial sarcoma and carcinoma. They account for less than 1% of all soft tissue sarcomas and are usually slow growing, with peak incidence in young adult men and occur predominantly in extremities. Histologically, they form nodules, with central necrosis surrounded by bland, polygonal cells with eosinophilic cytoplasm and peripheral spindling. They regularly express vimentin, cytokeratins, epithelial membrane antigen, and CD34, whereas staining is usually negative with S100, desmin, and FLI-1. Ultrastructurally, they display epithelial and mesenchymal features, including myofibroblastic differentiation. They manifest no specific cytogenetic findings, but several cases have displayed chromosomal abnormalities in 22q region. Clinically, they have a high recurrence rate, and up to 50% of epithelioid sarcomas metastasize. Proximal, fibroma-like, and angiomatoid variants have been described. The proximal variant (with larger cells, prominent nucleoli, and rhabdoid changes) is clinically more aggressive.