Ghosh Indranil, Arun Indu, Sen Saugata, Mishra Lopamudra
Department of Medical Oncology, Tata Medical Center, Kolkata, West Bengal, India.
Department of Pathology, Tata Medical Center, Kolkata, West Bengal, India.
Indian J Med Paediatr Oncol. 2014 Jan;35(1):99-102. doi: 10.4103/0971-5851.133733.
Perivascular epithelioid cell tumors (PEComa) are a family of rare mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. Female genital tract and retroperitoneum are common sites of origin of PEComa-not otherwise specified. Diagnosis depends upon characteristic morphology and immunohistochemistry findings. Prognosis of unresectable or metastatic disease is poor. Responses to mammalian target of rapamycin (mTOR) inhibition are encouraging but mostly short-lived. We report a case of metastatic PEComa who responded to mTOR inhibition, albeit for a short duration. We also review the existing literature on mTOR inhibitors in PEComa.
血管周上皮样细胞肿瘤（PEComa）是一类罕见的间叶性肿瘤，由组织学和免疫组化特征性的血管周上皮样细胞组成。女性生殖道和腹膜后是未另行指定的PEComa常见起源部位。诊断依赖于特征性的形态学和免疫组化结果。不可切除或转移性疾病的预后较差。对雷帕霉素哺乳动物靶点（mTOR）抑制的反应令人鼓舞，但大多持续时间较短。我们报告1例转移性PEComa患者，其对mTOR抑制有反应，尽管持续时间较短。我们还回顾了PEComa中mTOR抑制剂的现有文献。
