Jia Junjun, Luo Jia, Pan Cheng-Gen, Ge Guomei, Feng Meibao, Zou Bei, Liu Li, Zheng Shusen, Yu Jun
Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China.
Key Laboratory of Organ Transplantation, Research Center for Diagnosis and Treatment of Hepatobiliary Diseases, Hangzhou, Zhejiang, China.
J Clin Transl Hepatol. 2022 Feb 28;10(1):72-79. doi: 10.14218/JCTH.2020.00170. Epub 2021 Jun 21.
Perivascular epithelioid cell neoplasms (PEComas) are a rare type of mesenchymal neoplasm and their preoperative diagnosis is challenging. In this study, we summarized the experience from a single medical center to study the examinations, clinical presentations, and pathological and histological characteristics of PEComas in the liver in order to optimize overall understanding of the diagnosis and treatment of these neoplasms.
We conducted a retrospective analysis to investigate the clinical and pathological characteristics as well as imaging presentations of 75 patients diagnosed with hepatic PEComa in The First Affiliated Hospital of Zhejiang University between April 2010 and April 2020.
Among the 75 patients, 52 were women, and the median age was 48 years. Most patients had no specific symptoms, and two were admitted to the hospital for a second time owing to relapse. All patients underwent surgical resection. Histologically, 38 patients had classical angiomyolipoma (AML) and 37 had epithelioid AML. The PEComas were accompanied by positive immunohistochemical expression of HMB45, Melan-A, and smooth muscle actin. Follow-up data were obtained from 47 of the total 75 patients, through October 2020. Two patients had metastasis after surgery.
AML is the most common type of hepatic PEComa. There are no specific symptoms of hepatic PEComa, and serological examinations and imaging modalities for accurate preoperative diagnosis are lacking. Epithelioid AML should be considered a tumor of uncertain malignant potential; however, the prognosis of PEComa after resection is promising.
血管周上皮样细胞肿瘤（PEComas）是一种罕见的间叶性肿瘤，其术前诊断具有挑战性。在本研究中，我们总结了单一医学中心的经验，以研究肝脏PEComas的检查、临床表现以及病理和组织学特征，从而优化对这些肿瘤诊断和治疗的整体认识。
我们进行了一项回顾性分析，以调查2010年4月至2020年4月期间在浙江大学第一附属医院确诊为肝脏PEComa的75例患者的临床和病理特征以及影像学表现。
75例患者中，52例为女性，中位年龄为48岁。大多数患者无特异性症状，2例因复发再次入院。所有患者均接受了手术切除。组织学上，38例患者为经典血管平滑肌脂肪瘤（AML），37例为上皮样AML。PEComas伴有HMB45、Melan-A和平滑肌肌动蛋白免疫组化阳性表达。截至2020年10月，共获得了75例患者中47例的随访数据。2例患者术后出现转移。
AML是肝脏PEComa最常见的类型。肝脏PEComa无特异性症状，缺乏用于准确术前诊断的血清学检查和影像学方法。上皮样AML应被视为具有不确定恶性潜能的肿瘤；然而，PEComa切除术后的预后良好。
