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Primary hepatic epithelioid angiomyolipoma: A malignant potential tumor which should be recognized

Primary hepatic epithelioid angiomyolipoma: A malignant potential tumor which should be recognized

作     者:Jie Liu Cheng-Wu Zhang De-Fei Hong Ran Tao Yuan Chen Min-Jie Shang Yu-Hua Zhang 

作者机构:Department of Hepatopancreatobiliary Surgery and Minimally Invasive Surgery Zhejiang Provincial People’s Hospital Department of Pathology Zhejiang Provincial People’s Hospital 

出 版 物:《World Journal of Gastroenterology》 (世界胃肠病学杂志(英文版))

年 卷 期:2016年第22卷第20期

页      面:4908-4917页

核心收录:

学科分类:1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学] 

主  题:Epithelioid angiomyolipoma Imaging Liver Immunohistochemical staining Human melanoma black 45 

摘      要:AIM: To improve the clinical diagnosis and recognition of hepatic epithelioid angiomyolipoma(HEAML).METHODS: Four cases of primary HEAML were confirmed based on the pathology archive system in our hospital from January 2009 to November 2015. The general state, clinical symptoms, imaging manifestations, histological results and immunohistochemistry of these patients were retrospectively reviewed and analyzed. Studies of HEAML published in the last 15 years were collected from Pub Med and MEDLINE to summarize the clinical symptoms, imaging characteristics, pathological features and management of ***: Four cases of primary HEAML were retrieved from our archives. These included three female patients and one male patient, with a mean age of 41.8 ± 11.5 years(ranging from 31 to 56 years). The meantumor size was 7.3 ± 5.5 cm(ranging from 3.0 to 15 cm). In the contrast-enhanced imaging, the tumor was obviously enhanced in the arterial phase, but enhanced continuously or exhibited a slow-density masse during the venous and delayed phases. Histologically, the tumors mainly consisted of epithelioid cells that comprised approximately 95% of the total neoplastic mass. Although no metastases occurred in our patients, pathological studies revealed necrosis, mitotic figures and liver invasion in two patients, which indicates aggressive behavior. Immunohistochemical staining revealed that human melanoma black 45(HMB-45) and Melan-A were positive in 4 cases. We only identified 81 cases with primary HEAML, including our present patients, from 26 articles available from Pub Med and MEDLINE. The majority of the papers were published as case reports. Only 5(5/75, 6%) cases were associated with tuberous sclerosis complex(TSC). More than half(35/66) were discovered incidentally upon physical examination. Approximately 65%(22/34) of the patients were misdiagnosed with HCC or other tumors before surgery. Approximately 10%(8/81) of the patients with HEAML had recurrence or metastasi

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