Wang Chun-Ping, Li Hong-Yan, Wang Hong, Guo Xiao-Dong, Liu Chang-Chun, Liu Shu-Hong, Gao Xu-Dong, Qu Jian-Hui, Liu Ze, Chang Xiu-Juan, Lu Yin-Ying, Zeng Zhen, Lou Min, Yang Yong-Ping
From the Center of Therapeutic Research for Liver Cancer (C-PW, H-YL, HW, X-DG, J-HQ, ZL, X-JC, Y-YL, ZZ, ML, Y-PY); Center of Pathology (X-DG, S-HL); and Center of Radiology (C-CL), 302 Military Hospital, Beijing, China.
Medicine (Baltimore). 2014 Dec;93(28):e194. doi: 10.1097/MD.0000000000000194.
Hepatic angiomyolipoma (HAML) is a rare mesenchymal tumor of the liver with marked histological diversity. The present study was to review the magnetic resonance imaging (MRI) and clinical pathological features of HAML resembling hepatocellular carcinoma (HCC). Nine patients who underwent surgical resection and had pathological diagnosis of HAML were retrospectively analyzed. All of 9 patients (5 males and 4 females) had a solitary hepatic mass with a median size of 4 cm (from 1.4 cm to 15.3 cm). Seven cases were identified as incidental liver tumors during health screening and 2 patients were diagnosed for hepatic mass when visited hospitals with unspecific abdominal discomfort. Before resection, 6 cases were diagnosed as HCC on MRI. MRI on chemical shift imagings showed a large amount of lipids in 5 cases. The enhancement pattern of MRI was classified into 2 types: in 2 cases, lesions with small or no vessels that demonstrated prolonged enhancement (1 mixed subtype and 1 myomatous subtype) and in 7 cases, lesions with abundant central vessels that show rapid washout (3 mixed subtypes and 4 myomatous subtypes) in the portal venous/delayed phase. All patients underwent resection of hepatic tumor and no recurrence was observed during follow-up (range: 2-24 months) of median 10 months. By immunohistochemistry, the tumor cells demonstrated positive immunostaining for human melanoma black-45, smooth muscle actin, and CD34. In conclusion, all of 9 patients with HAML presented with none or nonspecific clinical manifestations. The diagnosis of HAML relies on disease and immunohistochemistry, but not MRI due to its resemblance to HCC.
肝血管平滑肌脂肪瘤（HAML）是一种罕见的肝脏间叶性肿瘤，组织学表现具有显著多样性。本研究旨在回顾与肝细胞癌（HCC）相似的HAML的磁共振成像（MRI）及临床病理特征。对9例行手术切除且病理诊断为HAML的患者进行回顾性分析。9例患者（5例男性，4例女性）均有单个肝脏肿块，中位大小为4厘米（范围1.4厘米至15.3厘米）。7例在健康筛查时被发现为肝脏 incidental 肿瘤，2例因非特异性腹部不适就诊时被诊断为肝脏肿块。切除术前，6例在MRI上被诊断为HCC。化学位移成像的MRI显示5例有大量脂质。MRI增强模式分为2种类型：2例病变血管少或无血管，表现为延迟强化（1例混合型和1例肌瘤型）；7例病变中央血管丰富，在门静脉期/延迟期表现为快速廓清（3例混合型和4例肌瘤型）。所有患者均接受了肝肿瘤切除术，随访（范围：2至24个月，中位10个月）期间未观察到复发。免疫组化显示，肿瘤细胞对人黑素瘤黑色45、平滑肌肌动蛋白和CD34呈阳性免疫染色。总之，9例HAML患者均无或仅有非特异性临床表现。HAML的诊断依赖于病理及免疫组化，而非MRI，因其与HCC相似。
