title: A huge malignant perivascular epithelioid cell tumor (PEComa) of the uterine cervix and vagina.
abstract: Perivascular epithelioid cell tumors (PEComas) are a family of rare mesenchymal neoplasms, including angiomyolipoma, clear-cell "sugar" tumor of the lung and extrapulmonary sites, lymphangioleiomyomatosis, clear-cell myomelanocytic tumor of the falciform ligament/ligamentum teres, and clear-cell tumors at various other anatomic sites. These tumors are characterized by a proliferation of epithelioid cells with clear to eosinophilic cytoplasm, perivascular distribution, and coexpression of myogenic and melanocytic markers. PEComas show a female predominance, occur with some frequency in the gynecological tract, and have an unpredictable biological behavior. We report a case of a huge malignant PEComa arising from the uterine cervix and vagina. To the best of our knowledge, only 6 cases of PEComa in the cervix and 2 cases in the vagina have been reported in the literature.
authors: Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy.
authors: Valentina
authors: V
authors: Natella
authors: Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy.
authors: Francesco
authors: F
authors: Merolla
authors: Department of Obstetrics and Gynecology, University of Naples Federico II, 80131 Naples, Italy.
authors: Pierluigi
authors: P
authors: Giampaolino
authors: Department of Obstetrics and Gynecology, University of Naples Federico II, 80131 Naples, Italy.
authors: Giuseppe
authors: G
authors: Bifulco
authors: IBB-CNR, 80131 Naples, Italy.
authors: Pier Paolo
authors: PP
authors: Mainenti
authors: Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy. Electronic address: insabato@unina.it.
authors: Luigi
authors: L
authors: Insabato
mesh_terms: D014408:Biomarkers, Tumor / Q000032:analysis; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008875:Middle Aged; D064847:Multimodal Imaging / Q000379:methods; D054973:Perivascular Epithelioid Cell Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D049268:Positron-Emission Tomography; D014057:Tomography, X-Ray Computed; D047368:Tumor Burden; D002583:Uterine Cervical Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D014625:Vaginal Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery
chemical_list: D014408:Biomarkers, Tumor
keywords: Malignant behavior; Myomelanocytic tumor; PEComa; Uterine tumor