title: Perivascular epithelioid cell tumors of the uterine cervix.
abstract: The World Health Organization (WHO) defines PEComas as mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular cells. Uterus is the most common site of a subgroup of PEComas not otherwise specified(NOS). PEComas of the uterine cervix are extremely rare, and only thirteen cases have been described in the English literature to date. In this review, we summarize the available data concerning diagnostics, immunohistochemical analysis, genetics and treatment of cervical PEComas. Radical hysterectomy with bilateral salpingooophorectomy is the best surgical approach available. Adjuvant therapy in its present form is not efficient. Therefore, further studies are needed to evaluate the newest treatment strategies.
authors.affiliation: Department of Gynecology and Obstetrics, Jessenius Faculty of Medicine in Martin, Comenius University in Bratislava, Slovakia. Electronic address: kudela.erik@gmail.com.
authors.forename: E
authors.initials: E
authors.lastname: Kudela
authors.affiliation: Department of Gynecology and Obstetrics, Jessenius Faculty of Medicine in Martin, Comenius University in Bratislava, Slovakia.
authors.forename: K
authors.initials: K
authors.lastname: Biringer
authors.affiliation: Department of Gynecology and Obstetrics, Jessenius Faculty of Medicine in Martin, Comenius University in Bratislava, Slovakia.
authors.forename: P
authors.initials: P
authors.lastname: Kasajova
authors.affiliation: Department of Gynecology and Obstetrics, Jessenius Faculty of Medicine in Martin, Comenius University in Bratislava, Slovakia.
authors.forename: M
authors.initials: M
authors.lastname: Nachajova
authors.affiliation: Institute of Histology and Embryology, Jessenius Faculty of Medicine in Martin, Comenius University in Bratislava, Slovakia.
authors.forename: M
authors.initials: M
authors.lastname: Adamkov
mesh_terms: D014408:Biomarkers, Tumor / Q000378:metabolism; D002584:Cervix Uteri / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D007044:Hysterectomy; D010052:Ovariectomy; D054973:Perivascular Epithelioid Cell Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D011379:Prognosis; D002583:Uterine Cervical Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery
chemical_list: D014408:Biomarkers, Tumor
keywords: Cervix; PEComa; Tuberous sclerosis