title: Neoplasms of the perivascular epithelioid cell involving the abdomen and the pelvis: cross-sectional imaging findings.
abstract: Neoplasms of the perivascular epithelioid cell (PEComas) represent a recently described heterogeneous group of mesenchymal tumors characterized by the presence of specific histological, immunohistochemical, and ultrastructural findings. The PEComas encompass a family of neoplasms that include angiomyolipomas, clear cell sugar tumors, and lymphangioleiomyomatosis. The PEComas demonstrate a wide spectrum of clinicobiological behavior and imaging findings. Perivascular epithelioid cell, as the name implies, is a unique cell that is characterized by perivascular distribution and epithelioid morphology. Perivascular epithelioid cell consistently shows immunoreactivity to melanocytic and smooth muscle markers including HMB-45 and actin. Abdominopelvic PEComas are found at a variety of somatic and visceral locations including kidney, liver, pancreas, gastrointestinal tract, genitourinary tract, peritoneum, and retroperitoneum. A subset of patients with abdominopelvic PEComas manifests tuberous sclerosis complex. In this paper, we review the histological spectrum and discuss the imaging findings of the PEComas that involve the abdomen and pelvis.
authors: Department of Radiology, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229, USA. prasads@uthscsa.edu
authors: Srinivasa R
authors: SR
authors: Prasad
authors: Dushyant V
authors: DV
authors: Sahani
authors: Mari
authors: M
authors: Mino-Kenudson
authors: Vamsi R
authors: VR
authors: Narra
authors: Peter A
authors: PA
authors: Humphrey
authors: Christine O
authors: CO
authors: Menias
authors: Kedar N
authors: KN
authors: Chintapalli
mesh_terms: D000005:Abdomen; D003287:Contrast Media; D004067:Digestive System Neoplasms / Q000175:diagnosis*; D004701:Endocrine Gland Neoplasms / Q000175:diagnosis*; D015622:Epithelioid Cells / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D010388:Pelvis; D014057:Tomography, X-Ray Computed; D014565:Urogenital Neoplasms / Q000175:diagnosis*
chemical_list: D003287:Contrast Media