title: Update on the imaging of malignant perivascular epithelioid cell tumors (PEComas).
abstract: Malignant perivascular epithelioid cell tumors (PEComas) are a histologic group of mesenchymal neoplasms that share a distinctive histological phenotype, the perivascular epithelioid cell. These tumors are known for their perivascular distribution. Malignant PEComas have a female predominance and are associated with aggressive disease and poor prognosis, making timely diagnosis critical to management. Imaging features of malignant PEComas are nonspecific and mimic other benign and malignant neoplasms. Surgery is the mainstay in the management of malignant PEComas. Promising novel molecular targeted therapies like m-TOR inhibitors have been shown to be effective in the metastatic setting. The aim of this review is to familiarize radiologists with the imaging appearances of and potential therapies for primary and metastatic malignant PEComa.
authors: Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA, 02115, USA.
authors: Catherine H
authors: CH
authors: Phillips
authors: Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA, 02115, USA.|Department of Imaging, Dana-Farber Cancer Institute, Harvard Medical School, 450 Brookline Avenue, Boston, MA, 02215, USA.
authors: Abhishek R
authors: AR
authors: Keraliya
authors: Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA, 02115, USA.|Department of Imaging, Dana-Farber Cancer Institute, Harvard Medical School, 450 Brookline Avenue, Boston, MA, 02215, USA.
authors: Atul B
authors: AB
authors: Shinagare
authors: Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA, 02115, USA.|Department of Imaging, Dana-Farber Cancer Institute, Harvard Medical School, 450 Brookline Avenue, Boston, MA, 02215, USA.
authors: Nikhil H
authors: NH
authors: Ramaiya
authors: Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA, 02115, USA. Sreeharsha_Tirumani@DFCI.HARVARD.EDU.|Department of Imaging, Dana-Farber Cancer Institute, Harvard Medical School, 450 Brookline Avenue, Boston, MA, 02215, USA. Sreeharsha_Tirumani@DFCI.HARVARD.EDU.
authors: Sree Harsha
authors: SH
authors: Tirumani
mesh_terms: D006801:Humans; D054973:Perivascular Epithelioid Cell Neoplasms / Q000000981:diagnostic imaging* / Q000473:pathology / Q000628:therapy; D010641:Phenotype; D011379:Prognosis
keywords: CT; MRI; Malignant PEComa; PET/CT