Phillips Catherine H, Keraliya Abhishek R, Shinagare Atul B, Ramaiya Nikhil H, Tirumani Sree Harsha
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.
Abdom Radiol (NY). 2016 Feb;41(2):368-76. doi: 10.1007/s00261-015-0568-8.
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.
恶性血管周上皮样细胞肿瘤（PEComas）是一组间质性肿瘤，具有独特的组织学表型——血管周上皮样细胞。这些肿瘤以其血管周分布而闻名。恶性PEComas以女性居多，与侵袭性疾病和不良预后相关，因此及时诊断对治疗至关重要。恶性PEComas的影像学特征不具有特异性，可模仿其他良性和恶性肿瘤。手术是恶性PEComas治疗的主要手段。有前景的新型分子靶向疗法，如m-TOR抑制剂，已被证明在转移性情况下有效。本综述的目的是使放射科医生熟悉原发性和转移性恶性PEComa的影像学表现及潜在治疗方法。
