Proximal-Type Epithelioid Sarcoma-A Case Report and Review of the Literature

近端上皮樣肉瘤－病例報告及文獻回顧

黃鴻怡(Hung-Yi Huang) ； 王伯涵(Po-Han Wang) ； 邱琬婷(Wan-Ting Chiu) ； 吳毅穎(Yih-Yiing Wu) ； 胡淑玲(Shu-Ling Hu)

《中華皮膚科醫學雜誌》 25卷4期 (2007/12) Pp. 274-278

https://doi.org/10.29784/DS.200712.0005

摘要

近端上皮樣肉瘤是一種少見的軟組織惡性腫瘤，主要發生於年輕至中年人的身體近端處。組織學上是以大的多角形細胞，伴隨空泡狀細胞核及顯著的核仁和常見的類橫紋形態。我們在此報告一位27歲女性於會陰部罹患近端上皮樣肉瘤，病患於切片診斷後即接受腫瘤廣泛性切除、左側腹股溝淋巴結切除及裂層皮膚移植。病理報告顯示在真皮及皮下有一界限不明之腫瘤，此腫瘤主要是由大型卵圓狀類上皮細胞所構成，淋巴結並沒有發現腫瘤細胞的存在。此外我們也回顧文獻上關於近端上皮樣肉瘤的臨床、病理、及免疫組織特性。重要的是近端上皮樣肉瘤比起傳統末端上皮樣肉瘤臨床的預後更差。

並列摘要

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 results 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.

並列關鍵字

Epithelioid sarcoma ； Rhabdoid feature