Prasad Srinivasa R, Sahani Dushyant V, Mino-Kenudson Mari, Narra Vamsi R, Humphrey Peter A, Menias Christine O, Chintapalli Kedar N
Department of Radiology, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229, USA.
J Comput Assist Tomogr. 2007 Sep-Oct;31(5):688-96. doi: 10.1097/rct.0b013e318031912f.
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.
血管周上皮样细胞瘤（PEComas）是一组最近才被描述的间质性肿瘤，具有异质性，其特征是存在特定的组织学、免疫组织化学和超微结构表现。PEComas包括一系列肿瘤，如血管平滑肌脂肪瘤、透明细胞糖瘤和淋巴管平滑肌瘤病。PEComas表现出广泛的临床生物学行为和影像学表现。血管周上皮样细胞，顾名思义，是一种独特的细胞，其特征是血管周分布和上皮样形态。血管周上皮样细胞始终对黑素细胞和平滑肌标志物（包括HMB-45和肌动蛋白）呈免疫反应性。腹盆腔PEComas可发生于多种躯体和内脏部位，包括肾脏、肝脏、胰腺、胃肠道、泌尿生殖道、腹膜和腹膜后。一部分腹盆腔PEComas患者表现为结节性硬化症复合体。在本文中，我们回顾了PEComas的组织学谱，并讨论了累及腹部和盆腔的PEComas的影像学表现。
