PMID- 30633211 OWN - NLM STAT- MEDLINE DCOM- 20190121 LR - 20210109 IS - 1536-5964 (Electronic) IS - 0025-7974 (Print) IS - 0025-7974 (Linking) VI - 98 IP - 2 DP - 2019 Jan TI - Malignant perivascular epithelioid cell tumor in the female genital tract: Preferred reporting items for systematic reviews and meta-analyses. PG - e14072 LID - 10.1097/MD.0000000000014072 [doi] LID - e14072 AB - BACKGROUND: Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor, located at various anatomic sites, including the female genital tract. This study aimed to evaluate the clinicopathological characteristics of patients with PEComa arising from the female genital tract. METHODS: 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. RESULTS: 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 immunoreactive for desmin (50/85, 58.8%). Multi-modality treatment, including surgery and mammalian target of rapamycin (mTOR) inhibitors 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). 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 genital tract. FAU - Liu, Chia-Hao AU - Liu CH AD - Department of Obstetrics and Gynecology, Taipei Veterans General Hospital. AD - Institute of Clinical Medicine, National Yang-Ming University. AD - Department of Obstetrics and Gynecology, National Yang-Ming University. FAU - Chao, Wei-Ting AU - Chao WT AD - Department of Obstetrics and Gynecology, Taipei Veterans General Hospital. AD - Department of Obstetrics and Gynecology, National Yang-Ming University. AD - School of Medicine, Fu-Jen Catholic University, New Taipei City. FAU - Lin, Shih-Chieh AU - Lin SC AD - Institute of Clinical Medicine, National Yang-Ming University. AD - Department of Pathology and Laboratory Medicine, Taipei Veterans General Hospital, Taipei. AD - School of Medicine, National Defense Medical Center, Taipei. FAU - Lau, Hei-Yu AU - Lau HY AD - Department of Obstetrics and Gynecology, Taipei Veterans General Hospital. AD - Department of Obstetrics and Gynecology, National Yang-Ming University. AD - School of Medicine, China Medical University, Taichung. FAU - Wu, Hua-Hsi AU - Wu HH AD - Department of Obstetrics and Gynecology, Taipei Veterans General Hospital. AD - Department of Obstetrics and Gynecology, National Yang-Ming University. AD - School of Medicine, Chung Shan Medical University, Taichung, Taiwan. FAU - Wang, Peng-Hui AU - Wang PH AD - Department of Obstetrics and Gynecology, Taipei Veterans General Hospital. AD - Institute of Clinical Medicine, National Yang-Ming University. AD - Department of Obstetrics and Gynecology, National Yang-Ming University. AD - Department of Medical Research, China Medical University Hospital, Taichung. LA - eng PT - Case Reports PT - Journal Article PT - Systematic Review PL - United States TA - Medicine (Baltimore) JT - Medicine JID - 2985248R SB - IM MH - Aged MH - Aged, 80 and over MH - Combined Modality Therapy MH - Female MH - Genital Neoplasms, Female/*diagnosis/pathology/therapy MH - Humans MH - Middle Aged MH - Perivascular Epithelioid Cell Neoplasms/*diagnosis/pathology/therapy PMC - PMC6336598 COIS- The authors declare that they have no competing interests. EDAT- 2019/01/12 06:00 MHDA- 2019/01/22 06:00 PMCR- 2019/01/11 CRDT- 2019/01/12 06:00 PHST- 2019/01/12 06:00 [entrez] PHST- 2019/01/12 06:00 [pubmed] PHST- 2019/01/22 06:00 [medline] PHST- 2019/01/11 00:00 [pmc-release] AID - 00005792-201901110-00062 [pii] AID - MD-D-18-06863 [pii] AID - 10.1097/MD.0000000000014072 [doi] PST - ppublish SO - Medicine (Baltimore). 2019 Jan;98(2):e14072. doi: 10.1097/MD.0000000000014072.