女性生殖道惡性血管旁類上皮細胞瘤：北榮經驗以及系統性文獻回顧

Malignant perivascular epithelioid cell tumor in the female genital tract: Taipei VGH experience and systematic reviews

惡性血管旁類上皮細胞瘤（perivascular epithelioid cell tumor, PEComa）為一種非常罕見之間質細胞腫瘤，可能從各種解剖學位置生長出來。文獻上婦女生殖道惡性血管旁類上皮細胞瘤一共有114例，其中最常見的好發位置是子宮（71.9％），其次是子宮頸（10.5％）。免疫學染色研究上，幾乎所有的女性生殖道惡性血管旁類上皮細胞瘤皆為HMB-45陽性（99.1％），超過半數為Desmin陽性（58.8％）。治療方面以完善減積手術（optimal debulking surgery）為主以及針對侵襲性的腫瘤給予口服mammalian target of rapamycin（mTOR）抑制劑，能夠延長disease-free survival（治癒率可達百分之五十至一百）。從2008年到2018年間，台北榮民總醫院婦女醫學部診斷治療了三例女性生殖道惡性血管旁類上皮細胞瘤個案，我們針對這3例北榮個案以及114個過去發表的個案做了詳細的分析以及文獻回顧。

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor, can arise from various anatomic sites. This study aimed to evaluate the clinic-pathological characteristics of patients with malignant PEComa arising from the female genital tract. A retrospective study was conducted in Taipei Veterans General Hospital (Taipei VGH) between 2008 and 2018. All published English cases based on the Preferred Reporting Items for Systematic Reviews and Meta- Analyses (PRISMA) statement were also included in the current review. A total of 114 women from PRISMA and 3 women from Taipei VGH were identified. The uterus was the most commonly involved site (82/114, 71.9%), followed by the cervix (12/114, 10.5%). Immunohistochemical staining showed that nearly all gynecological PEComas were positive for human melanoma black 45 (113/114, 99.1%). More than half of the gynecological PEComas were immune-reactive for desmin (50/85, 58.8%). Our results showed an optimal cytoreductive surgery, and mammalian target of rapamycin (mTOR) inhibitors for aggressive tumor behavior as targeted therapy, provided long-term disease-free survival (cure rate ranging from 50% to 100%, based on the different anatomic sites of the female genital tract). In conclusion, multi-modality treatment, including cytoreductive surgery and mTOR inhibitors with/without chemotherapy and/ or radiation, should be considered for the management of women with PEComas in the female genital tract with aggressive behavior.