Clear cell 'sugar' tumor (PEComa) of the skin: a case report
Clear cell 'sugar' tumor (PEComa) of the skin: a case report
复制标题
DOI：
10.1111/j.0303-6987.2005.00347.x
复制
发表时间：
2005-07-01
影响因子：
1.7
通讯作者：
Myant, N
中科院分区：
文献类型：
--
作者：
Somerhausen, NDA;Galdon, MG;Myant, N
所谓血管周围上皮样细胞肿瘤(PEComa)家族包括血管肌脂肪瘤、透明细胞糖瘤(CCST)、淋巴管肌瘤病和透明细胞肌黑色素细胞瘤(CCMMT)。这些罕见的肿瘤的特征是黑素细胞和肌肉标记物的共同表达。它们已经在越来越多的部位被发现，但目前只有一例CCMMT亚型的PEComa在皮肤中被报道为抽象形式。我们提供了一例位于真皮和大腿浅皮下的肺外CCST的临床病理描述。病变由上皮样细胞和梭形细胞巢组成，胞质丰富，透明至颗粒状，细胞边界清晰。肿瘤对HMB-45呈强的弥漫性免疫反应。散在细胞表达结蛋白和h-钙结蛋白。S-100蛋白、Melan-A、细胞角蛋白和平滑肌肌动蛋白抗体均为阴性。首例CCST型皮肤PEComa扩大了皮肤透明细胞/颗粒细胞肿瘤的鉴别诊断。De Saint Aubain Somerhausen N，Galdon MG，Bouffioux B，Courtin C，Theunis A，Vogeleer MN，Myant N.皮肤透明细胞糖瘤(PEComa)：病例报告。
The so-called perivascular epithelioid cell neoplasm (PEComa) family includes angiomyolipoma, clear cell 'sugar' tumor (CCST), lymphangioleiomyomatosis, and clear cell myomelanocytic tumor (CCMMT). These rare tumors are characterized by the co-expression of melanocytic and muscle markers. They have been recognized in an increasing number of sites but currently only one case of PEComa, of the CCMMT subtype, has been reported in the skin in abstract form.We provide the clinicopathologic description of a case of extrapulmonary CCST located in the dermis and superficial subcutis of the thigh of a 60-year-old female. The lesion was composed of nests of epithelioid and spindle cells with abundant clear to granular cytoplasm and distinct cell borders. The tumor showed strong and diffuse immunoreactivity with HMB-45. Scattered cells expressed desmin and h-caldesmon. Antibodies to S-100 protein, melan-A, cytokeratins, and smooth muscle actin were negative. This first case of cutaneous PEComa of the CCST type expands the differential diagnosis of clear cell/granular cell tumors of the skin.de Saint Aubain Somerhausen N, Galdon MG, Bouffioux B, Courtin C, Theunis A, Vogeleer MN, Myant N. Clear cell 'sugar' tumor (PEComa) of the skin: a case report.