Calder Kenneth B, Schlauder Scott, Morgan Michael B
Department of Pathology University of South Florida College of Medicine, Tampa, FL, USA.
J Cutan Pathol. 2008 May;35(5):499-503. doi: 10.1111/j.1600-0560.2007.00842.x. Epub 2007 Nov 12.
Perivascular epithelioid cell (PEC) tumors, also called 'PEComas,' are distinct tumors showing PEC differentiation with characteristic histologic and immunophenotypic features. PEComas are rare tumors documented in the literature presenting in numerous anatomic sites including the thorax, abdomen, pelvis, soft tissue and skin. Criteria for malignancy does not exist for the subset of PEComas that pursue an aggressive clinical course. Herein, we present an unusual case of a malignant PEC tumor presenting as a scalp nodule in a patient with a prior diagnosis of 'melanoma' based upon the immunophenotypic profile of an excised enlarged cervical lymph node. The purpose of this case presentation is to further describe the rare clinical manifestations of a subcutaneous PEC tumor, emphasize the malignant potential of this entity, and review the literature focusing upon clinicopathologic features of cutaneous/subcutaneous PEComas.
血管周上皮样细胞（PEC）肿瘤，也称为“PEComas”，是一种独特的肿瘤，表现出PEC分化，并具有特征性的组织学和免疫表型特征。PEComas是文献中记载的罕见肿瘤，出现在包括胸部、腹部、骨盆、软组织和皮肤在内的众多解剖部位。对于表现出侵袭性临床病程的PEComas亚组，不存在恶性标准。在此，我们报告一例不寻常的恶性PEC肿瘤病例，该肿瘤表现为头皮结节，患者先前基于切除的肿大颈部淋巴结的免疫表型特征被诊断为“黑色素瘤”。本病例报告的目的是进一步描述皮下PEC肿瘤罕见的临床表现，强调该实体的恶性潜能，并回顾聚焦于皮肤/皮下PEComas临床病理特征的文献。
