Sung K F, Chen T C, Hung C F, Jeng L B, Lien J M
Department of Hepato-Gastroenterology, Chang Gung Memorial Hospital, Taipei, Taiwan, R.O.C.
Chang Gung Med J. 2001 May;24(5):318-23.
Hepatic angiomyolipoma is a rare benign mesenchymal tumor of the liver. Most multiple hepatic angiomyolipomas have appeared in patients with renal angiomyolipoma and tuberous sclerosis. A 38-year-old female patient without chronic hepatitis B or C was hospitalized because of epigastric fullness for 2 months. Radiologic studies showed a large solid tumor with a small daughter nodule in the right hepatic lobe. Upon intravenous bolus injection of contrast medium, both tumors showed weak heterogeneous enhancement in the delayed phase. Although hepatocellular carcinoma was suspected by the findings of computed tomography, percutaneous transhepatic ultrasound-guided biopsy was performed for the large tumor. The histopathology showed many mature fat cells intermingled with thick-walled blood vessels, and epithelioid cells with eosinophilic cytoplasm; the epithelioid cells stained positively for HMB-45 and smooth muscle actin. Angiomyolipoma of the liver was confirmed. The main tumor enlarged considerably during a follow-up period of 3 years. Surgical resection was performed due to persistent symptoms. She had an uneventful postoperative recovery and was well when followed up 10 months after surgery. We should be aware that a hepatic angiomyolipoma can change in size during its natural course, and this finding does not necessarily indicate malignancy.
肝血管平滑肌脂肪瘤是一种罕见的肝脏良性间叶性肿瘤。多数多发性肝血管平滑肌脂肪瘤见于患有肾血管平滑肌脂肪瘤和结节性硬化症的患者。一名38岁无慢性乙型或丙型肝炎的女性患者因上腹部饱胀2个月入院。影像学检查显示右肝叶有一个大的实性肿瘤及一个小的子结节。静脉推注造影剂后,两个肿瘤在延迟期均表现为不均匀轻度强化。尽管根据计算机断层扫描结果怀疑为肝细胞癌,但仍对较大的肿瘤进行了经皮肝穿刺超声引导下活检。组织病理学显示有许多成熟脂肪细胞与厚壁血管以及胞质嗜酸性的上皮样细胞混合存在;上皮样细胞HMB-45和平滑肌肌动蛋白染色呈阳性。确诊为肝血管平滑肌脂肪瘤。在3年的随访期内,主要肿瘤显著增大。因症状持续存在而行手术切除。术后恢复顺利,术后10个月随访时情况良好。我们应该认识到,肝血管平滑肌脂肪瘤在其自然病程中大小可能会改变,而这一发现不一定提示恶性。