Liu Jie, Zhang Cheng-Wu, Hong De-Fei, Tao Ran, Chen Yuan, Shang Min-Jie, Zhang Yu-Hua
Jie Liu, Cheng-Wu Zhang, De-Fei Hong, Ran Tao, Min-Jie Shang, Yu-Hua Zhang, Department of Hepatopancreatobiliary Surgery and Minimally Invasive Surgery, Zhejiang Provincial People's Hospital, Hangzhou 310014, Zhejiang Province, China.
World J Gastroenterol. 2016 May 28;22(20):4908-17. doi: 10.3748/wjg.v22.i20.4908.
To improve the clinical diagnosis and recognition of hepatic epithelioid angiomyolipoma (HEAML).
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 PubMed and MEDLINE to summarize the clinical symptoms, imaging characteristics, pathological features and management of HEAML.
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 mean tumor 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 PubMed 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 metastasis after surgery, which was a very high and alarming rate.
HEAML is a very rare primary hepatic tumor that is often misdiagnosed before surgery. Patients should be followed closely after surgery because of its malignant potential.
提高肝上皮样血管平滑肌脂肪瘤（HEAML）的临床诊断与识别能力。
基于我院病理存档系统，确认2009年1月至2015年11月期间的4例原发性HEAML病例。对这些患者的一般状况、临床症状、影像学表现、组织学结果及免疫组化结果进行回顾性分析。从PubMed和MEDLINE收集过去15年发表的关于HEAML的研究，以总结HEAML的临床症状、影像学特征、病理特征及治疗方法。
从我院存档中检索到4例原发性HEAML病例。其中包括3例女性患者和1例男性患者，平均年龄为41.8±11.5岁（范围为31至56岁）。肿瘤平均大小为7.3±5.5cm（范围为3.0至15cm）。在增强影像学检查中，肿瘤在动脉期明显强化，但在静脉期和延迟期持续强化或呈低密度肿块。组织学上，肿瘤主要由上皮样细胞组成，约占肿瘤总体积的95%。虽然我们的患者未发生转移，但病理研究显示2例患者存在坏死、核分裂象及肝脏侵犯，提示具有侵袭性行为。免疫组化染色显示，4例患者的人黑色素瘤黑色45（HMB-45）和Melan-A均为阳性。从PubMed和MEDLINE上可获取的26篇文章中，包括我们目前的患者，仅识别出81例原发性HEAML病例。大多数论文为病例报告。仅5例（5/75，6%）与结节性硬化症（TSC）相关。超过一半（35/66）是在体检时偶然发现的。约65%（22/34）的患者在手术前被误诊为肝癌或其他肿瘤。约10%（8/81）的HEAML患者术后出现复发或转移，这一比例非常高且令人担忧。
HEAML是一种非常罕见的原发性肝脏肿瘤，术前常被误诊。由于其具有恶性潜能，术后应密切随访患者。
