Charlotte Radiology, 8514 McAlpine Park Dr., Suite 100, Charlotte, NC 28211, USA.
University of California, Los Angeles, David Geffen School of Medicine at UCLA, Department of Radiological Sciences, 757 Westwood Plaza, Suite 1621, Los Angeles, CA 90095-7532, USA.
Clin Imaging. 2024 Dec;116:110332. doi: 10.1016/j.clinimag.2024.110332. Epub 2024 Oct 16.
Perivascular epithelioid cell tumors (PEComas) are a group of rare mesenchymal tumors, which demonstrate varied imaging appearances and treatment options. These tumors may arise de novo or in the setting of systemic disorders, such as tuberous sclerosis. Some PEComas are benign and easily resected while others may represent systemic or metastatic disease with limited therapeutic options. The purpose of this review is to introduce the topic of perivascular epithelioid cell tumors and the most common tumors within the PEComa family as well as discuss the epidemiology, morphology, radiographic appearance, and treatment options of these rare tumors.
血管周上皮样细胞瘤（PEComas）是一组罕见的间叶性肿瘤，具有不同的影像学表现和治疗选择。这些肿瘤可能是新发的，也可能是在结节性硬化症等全身性疾病的背景下发生的。一些 PEComas 是良性的，易于切除，而另一些则可能是全身性或转移性疾病，治疗选择有限。本综述的目的是介绍血管周上皮样细胞瘤的主题以及 PEComa 家族中最常见的肿瘤，并讨论这些罕见肿瘤的流行病学、形态学、影像学表现和治疗选择。
