Nishibaba Rie, Higashi Yuko, Goto Yuko, Hisaoka Masanori, Kanekura Takuro
Department of Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan.
Department of Pathology, Field of Oncology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.
J Med Case Rep. 2016 Oct 24;10(1):295. doi: 10.1186/s13256-016-1088-z.
Epithelioid sarcoma is a rare, high-grade malignant tumor of the soft tissue. The incidence of local recurrence, regional lymph node involvement, and distant metastases is high. Epithelioid sarcoma is most often seen in adolescents and young adults. In the early stage before the development of full clinical features, epithelioid sarcoma is often misdiagnosed as a benign disease such as granuloma.
We report a case of a 74-year-old Japanese woman whose epithelioid sarcoma was initially misdiagnosed as fungal infection. Rebiopsy revealed the proliferation of atypical polygonal or oval epithelioid cells in the dermis and lymphocyte infiltration through the dermis. Immunohistochemically, the tumor cells were positive for vimentin, cell adhesion molecule 5.2, epithelial membrane antigen, and E26-related gene. The nuclear expression of integrase interactor 1 was lost in the tumor cells.
We encountered a rare case of epithelioid sarcoma and had difficulty in making the correct diagnosis. We suggest that in patients whose lesions are resistant to conventional treatments, repeat biopsy and immunohistochemical studies should be considered to rule out rare epithelioid sarcoma.
上皮样肉瘤是一种罕见的软组织高级别恶性肿瘤。局部复发、区域淋巴结受累及远处转移的发生率较高。上皮样肉瘤最常见于青少年和青年成人。在完整临床特征出现之前的早期阶段,上皮样肉瘤常被误诊为肉芽肿等良性疾病。
我们报告一例74岁日本女性,其上皮样肉瘤最初被误诊为真菌感染。再次活检显示真皮层有非典型多边形或椭圆形上皮样细胞增殖,淋巴细胞浸润穿过真皮层。免疫组化显示,肿瘤细胞波形蛋白、细胞黏附分子5.2、上皮膜抗原及E26相关基因呈阳性。肿瘤细胞中整合酶相互作用分子1的核表达缺失。
我们遇到一例罕见的上皮样肉瘤病例,诊断困难。我们建议,对于病变对常规治疗耐药的患者,应考虑重复活检及免疫组化研究以排除罕见的上皮样肉瘤。