Department of Dermatology, University of Michigan, Ann Arbor, Michigan, USA.
Department of Pathology, University of Michigan, Ann Arbor, Michigan, USA.
J Cutan Pathol. 2021 Aug;48(8):1088-1093. doi: 10.1111/cup.14034. Epub 2021 May 10.
Perivascular epithelioid cell tumors (PEComas) are mesenchymal neoplasms with characteristic epithelioid or spindled cytomorphology that typically grow around blood vessels. These tumors are phenotypically and immunohistochemically distinct, expressing markers of both melanocytic and smooth muscle differentiation. Herein, we describe a case of histopathologically malignant cutaneous PEComa without metastatic spread, with review of the pertinent literature. Telescoping punch biopsy demonstrated an epithelioid neoplasm with marked atypia, hypercellularity, and increased mitotic activity. Immunohistochemical stains for HMB-45, NK1-C3, PGP9.5, MiTF, CD10, and CD68 were positive within tumor cells. In addition, there was diffuse expression of caldesmon and focal cytoplasmic staining for smooth muscle actin on the excision specimen. The patient underwent treatment with surgical excision with adjuvant radiation and surveillance computed tomography (CT). The patient remains free of recurrence or metastatic disease after 10 months of follow-up. To our knowledge, this is only the third reported case of a malignant cutaneous PEComa reported in the literature to date.
血管周上皮样细胞瘤（PEComas）是一种间叶性肿瘤，具有特征性的上皮样或梭形细胞形态学，通常围绕血管生长。这些肿瘤在表型和免疫组织化学上是不同的，表达黑色素细胞和平滑肌分化的标志物。在此，我们描述了一例无转移的组织病理学恶性皮肤 PEComa，并复习了相关文献。套切活检显示为具有显著异型性、细胞丰富性和增加的有丝分裂活性的上皮样肿瘤。肿瘤细胞内 HMB-45、NK1-C3、PGP9.5、MiTF、CD10 和 CD68 的免疫组化染色呈阳性。此外，在切除标本上弥漫表达钙调蛋白，平滑肌肌动蛋白呈局灶性细胞质染色。患者接受了手术切除联合辅助放疗和监测计算机断层扫描（CT）治疗。患者在随访 10 个月后仍无复发或转移。据我们所知，这是迄今为止文献中仅有的第三例报道的恶性皮肤 PEComa 病例。
