Back issues No.4 - 2007 / Case Report  

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Proximal-Type Epithelioid Sarcoma-A Case Report and Review of the Literature
Hung-Yi Huang , Po-Han Wang , Wan-Ting Chiu , Yih-Yiing Wu , Shu-Ling Hu
Dermatol Sinica 25: 274-278, 2007

Proximal-type epithelioid sarcoma is a rare, malignant soft-tissue tumor occurring in the proximal body sites of young to middle-aged adults and microscopically shows sheets of large polygonal cells with vesicular nuclei, prominent nucleoli and frequent rhabdoid features. Herein, we report a 27-year-old woman with proximal-type epithelioid sarcoma over the pubic area. The patient underwent wide excision, left inguinal lymph node dissection and split-thickness skin graft after biopsy proof. Pathological result showed an ill-defined nodule composed of large oval epithelioid cells in the dermis and subcutis. The dissected lymph nodes were free of tumor cells. We also review the literature regarding the clinical, pathologic and immunohistochemical features of proximal-type epithelioid sarcoma. Importantly, proximal-type epithelioid sarcoma has a much worse prognosis than the classical, distally located ones.

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