Ieremia Eleni, Robson Alistair
Department of Dermatopathology, St John's Institute of Dermatology, St Thomas' Hospital, London, United Kingdom.
Am J Dermatopathol. 2014 Dec;36(12):e198-201. doi: 10.1097/DAD.0000000000000041.
PEComa is a mesenchymal neoplasm thought to derive from the perivascular epithelioid cell, a hypothetical cell type not yet identified. PEComa is a broad term that was recently expanded to include visceral, retroperitoneal, and somatic soft tissue, in addition to cutaneous tumors, which share morphological and immunohistochemical features in common with angiomyolipoma, lymphangiomyomatosis, and clear cell "sugar" tumor. The latter have distinct, site-related, clinical, morphological, and biological features. PEComas share features of concurrent melanocytic and myocytic differentiation. Most cases are sporadic, but renal and pulmonary PEComas can be part of the tuberous sclerosis syndrome more often than PEComas from other sites. We report a case of cutaneous PEComa to raise awareness of the entity occurring at this site and caution against misinterpretation as a primary or metastatic malignancy, for example melanoma or renal cell carcinoma, clear cell type.
PEComa是一种间叶性肿瘤，被认为起源于血管周上皮样细胞，这是一种尚未被识别的假设细胞类型。PEComa是一个广义术语，最近其范围有所扩大，除皮肤肿瘤外，还包括内脏、腹膜后和躯体软组织肿瘤，这些肿瘤与血管平滑肌脂肪瘤、淋巴管平滑肌瘤病及透明细胞“糖”瘤具有共同的形态学和免疫组化特征。后者具有独特的、与部位相关的临床、形态学和生物学特征。PEComas具有同时存在黑素细胞和肌细胞分化的特征。大多数病例为散发性，但肾和肺PEComas比其他部位的PEComas更常成为结节性硬化综合征的一部分。我们报告一例皮肤PEComa病例，以提高对该部位出现此病变的认识，并提醒注意避免误诊为原发性或转移性恶性肿瘤，如黑色素瘤或透明细胞型肾细胞癌。
