title: [A case of malignant perivascular epithelioid cell tumor of the retroperitoneum with multiple metastases].
abstract: Perivascular epithelioid cell tumors (PEComas) are unusual mesenchymal neoplasms composed of histologically and immunohistochemically distinct perivascular epithelioid cells (PECs). Although PEComas have the potential to behave in a malignant fashion, malignant PEComas arising from the retroperitoneum are extremely rare. A 68-year-old woman presented with a painful palpable mass in her left upper abdomen. Computed tomography of the abdomen showed a 9 cm sized heterogeneous mass in left para-aortic space and multiple hypervascular nodules in the liver. (18)F-fludeoxyglucose-PET/CT showed multifocal hyper-metabolic lesions in retroperitoneum, liver, and skeletal bones. Percutaneous needle biopsies were done on the retroperitoneal and hepatic mass. Both specimens were positive for human melanoma black-45 (HMB-45) on histological and immunohistochemical staining which was compatible with PEComas. Herein, we report a rare case of retroperitoneal PEComa with multiple metastases involving liver and bone at initial diagnosis that exhibited aggressive behavior and resulted in a devastating prognosis.
authors.forename: Hye Won
authors.initials: HW
authors.lastname: Oh
authors.forename: Tae Hyo
authors.initials: TH
authors.lastname: Kim
authors.forename: Ra Ri
authors.initials: RR
authors.lastname: Cha
authors.forename: Na Young
authors.initials: NY
authors.lastname: Kim
authors.forename: Hyun Jin
authors.initials: HJ
authors.lastname: Kim
authors.forename: Woon Tae
authors.initials: WT
authors.lastname: Jung
authors.forename: Ok Jae
authors.initials: OJ
authors.lastname: Lee
authors.forename: Jeong Hee
authors.initials: JH
authors.lastname: Lee
mesh_terms: D000368:Aged; D001859:Bone Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology / Q000556:secondary; D005260:Female; D019788:Fluorodeoxyglucose F18; D006801:Humans; D008113:Liver Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology / Q000556:secondary; D054973:Perivascular Epithelioid Cell Neoplasms / Q000175:diagnosis* / Q000473:pathology; D049268:Positron-Emission Tomography; D014057:Tomography, X-Ray Computed
chemical_list: D019788:Fluorodeoxyglucose F18