Zhou Yan-Ming, Li Bin, Xu Feng, Wang Bin, Li Dian-Qi, Zhang Xiao-Feng, Liu Peng, Yang Jia-Mei
Department of Hepato-Biliary-Pancreato-Vascular Surgery, First Xiamen Hospital, Fujian Medical University, Xiamen 361003, China.
Hepatobiliary Pancreat Dis Int. 2008 Jun;7(3):284-7.
Hepatic angiomyolipoma (HAML) is a rare hepatic mesenchymal tumor. This study was designed to explore its clinical features.
Clinical data from 26 patients who had been pathologically confirmed with HAML and had received surgical resection at our hospital were analyzed retrospectively.
HAML was seen more frequently in females (18/26) in this series, and most of the patients presented no significant symptoms except for one who had a spontaneous rupture hemorrhage. Serum alpha-fetoprotein (AFP), carbohydrate antigen 19-9 (CA19-9) and carcinoembryonic antigen (CEA) were negative in all patients. Imaging presentations were diverse. Pre-operative diagnosis was made in only 3 patients. Pathological study showed that the tumor was composed of adipose tissue, smooth muscle and blood vessels in different proportions. One patient showed hepatic vessel invasion. HMB-45 immunohistochemical staining was positive in all tumors. All patients underwent surgical resection without significant complications. Except for one patient who died 14 months after operation because of recurrent disease, no tumor recurrence was observed in the remaining 25 patients during a 2-3 years follow-up.
Pre-operative diagnosis of HAML is difficult. There are potential risks of spontaneous rupture and malignant transformation. Surgical resection is the treatment of choice for HAML.
肝血管平滑肌脂肪瘤（HAML）是一种罕见的肝脏间叶性肿瘤。本研究旨在探讨其临床特征。
回顾性分析我院26例经病理确诊为HAML并接受手术切除的患者的临床资料。
本系列中HAML在女性中更为常见（18/26），除1例发生自发性破裂出血外，大多数患者无明显症状。所有患者血清甲胎蛋白（AFP）、糖类抗原19-9（CA19-9）和癌胚抗原（CEA）均为阴性。影像学表现多样。仅3例患者术前得到诊断。病理研究显示肿瘤由不同比例的脂肪组织、平滑肌和血管组成。1例患者出现肝血管侵犯。所有肿瘤HMB-45免疫组化染色均为阳性。所有患者均接受了手术切除，无明显并发症。除1例患者术后14个月因疾病复发死亡外，其余25例患者在2至3年的随访期间未观察到肿瘤复发。
HAML术前诊断困难。存在自发性破裂和恶变的潜在风险。手术切除是HAML的首选治疗方法。
