Department of Pathology, City of Hope National Medical Center, 1500 East Duarte Road, Duarte, CA 91010, United States.
Pathol Res Pract. 2012 Feb 15;208(2):113-7. doi: 10.1016/j.prp.2011.11.002. Epub 2011 Dec 9.
Perivascular epithelioid cell neoplasms, also known as PEComas, are unique mesenchymal tumors exhibiting perivascular epithelioid cell differentiation, characterized by a mixed myogenic and melanocytic phenotype. PEComas arising in visceral organs outside of the kidney, liver, and lung are rare, and often pose problems in diagnosis. Examples of this neoplasm originating in the adrenal gland are limited. The present report details the clinical and pathologic features of an unusual case of a pure epithelioid PEComa (epithelioid angiomyolipoma) of the adrenal gland exhibiting clinically malignant behavior in the form of pulmonary metastases, a feature not previously described in tumors of this site. The diagnosis was supported by immunohistochemical studies demonstrating expression of myoid and melanocytic antigens. The present case serves to emphasize the potential of PEComa for clinically aggressive behavior and the importance of distinguishing this tumor from other epithelioid neoplasms that are more commonly encountered in the adrenal gland.
血管周上皮样细胞肿瘤，也称为 PEComa，是一种独特的间叶性肿瘤，表现出血管周上皮样细胞分化，其特征为肌源性和黑色素细胞表型的混合。发生在肾脏、肝脏和肺以外的内脏器官的 PEComa 很少见，常常在诊断上带来问题。起源于肾上腺的这种肿瘤的例子是有限的。本报告详细介绍了一例罕见的肾上腺纯上皮样 PEComa（上皮样血管平滑肌脂肪瘤）的临床和病理特征，该肿瘤表现出临床上恶性的行为，即肺转移，这是以前在该部位的肿瘤中未描述过的特征。免疫组织化学研究支持该诊断，显示肌源性和黑色素细胞抗原的表达。本病例强调了 PEComa 具有临床上侵袭性行为的潜力，以及区分这种肿瘤与在肾上腺更常见的其他上皮样肿瘤的重要性。
