A Rare Case of Epithelioid Angiosarcoma Exhibiting Diagnostic Ambiguity

Abstract

Epithelioid angiosarcoma is a rare highly malignant tumor characterized by large cells with epithelioid features mimicking poorly differentiated carcinoma by its epithelial appearance. It arises from deep soft tissues of extremities or a variety of primary sites including thyroid, skin, and adrenals. Here is a case of epithelioid angiosarcoma of a 48-year-old female who presented with right supraclavicular swelling since 2 months of size 2 × 3 cm which was fixed, firm in consistency with no significant past, family, and personal history. Fine needle aspiration cytology (FNAC) of the same showed features of metastatic poorly differentiated carcinoma. Evaluation for the primary yielded no significant finding. A trucut biopsy showed tumor consistent with features of epithelioid hemangioendothelioma. A wide excision of the same showed features of epithelioid angiosarcoma with tumor cells having a pleomorphic vesicular nucleus with prominent nucleoli and moderate amount of cytoplasm and significant number of tumor giant cells, vascular channels lined by tumor cells. The patient received radiotherapy after surgery. After 2 years of follow-up, no evidence of recurrence or metastasis was noted. Epithelioid angiosarcoma is a rare aggressive tumor mimicking features of poorly differentiated carcinoma. Hence, this should be considered as differential diagnosis for any swelling in the unusual location (supraclavicular region) with epithelioid features.