Machado Isidro, Cruz Julia, Lavernia Javier, Rayon José M, Poveda Andrés, Llombart-Bosch Antonio
1 Instituto Valenciano de Oncología, Valencia, Spain.
2 Hospital NISA Nueve de Octubre, Valencia, Spain.
Int J Surg Pathol. 2017 Sep;25(6):543-549. doi: 10.1177/1066896917701245. Epub 2017 May 1.
Perivascular epithelioid cell tumors (PEComas) are infrequent neoplasms with peculiar myomelanocytic differentiation. The aggressive abdominopelvic variant is rare, with only a small number of published cases. We present an additional case of this unusual variant, which showed an aggressive histologic and clinical behavior with multiple liver metastases and resistance to several therapies. We also discuss the histological and immunohistochemical profiles as well as the differential diagnosis.
血管周上皮样细胞瘤（PEComas）是一种罕见的肿瘤，具有独特的肌黑色素细胞分化。侵袭性腹盆腔型罕见，仅有少数病例报道。我们报告了这一罕见变异型的另一例病例，其具有侵袭性的组织学和临床行为，伴有多发肝转移且对多种治疗耐药。我们还讨论了其组织学和免疫组化特征以及鉴别诊断。
