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肝血管平滑肌脂肪瘤的临床病理研究

[A clinicopathologic study of hepatic angiomyolipoma].

作者信息

Li Tao, Fan Jia, Qin Lun-xiu, Zhou Jian, Sun Hui-chuan, Wang Lu, Ye Qing-hai, Qiu Shuang-jian, Tang Zhao-you

机构信息

Department of Hepatic Surgery, Zhongshan Hospital Fudan University, Shanghai 200032, China.

出版信息

Zhonghua Wai Ke Za Zhi. 2009 Oct 15;47(20):1536-9.

Abstract

OBJECTIVE

To investigate the clinicopathologic features, diagnosis and treatment of hepatic angiomyolipoma (HAML).

METHODS

The clinical, histopathological, treatment and prognosis data of 51 patients treated for HAML from October 1998 to October 2007 were retrospectively analyzed.

RESULTS

HAML had a female predilection (female/male = 41/10) and the mean age was 44 years old. The main symptoms were abdominal mass (33 cases) and abdominal pain or discomfort (15 cases), the other 2 cases presented as fever. Histopathologically, HAML was composed of a heterogeneous mixture of blood vessels, smooth muscle, and adipose cells. Immunohistochemical staining showed relatively high positive rate of HMB-45 (50/51), SMA (47/49) and S-100 (39/42). All 51 patients underwent partial hepatectomy. The mean hospital stay was 13.8 days and mean intraoperative blood loss was 263 ml. There was no recurrence or metastasis after a mean follow-up of 55.4 months.

CONCLUSIONS

HAML is a rare benign mesenchymal tumor of the liver. Definitive diagnosis of HAML depends on the pathohistological findings and HMB-45 positive myoid cell is an important diagnostic marker. Complete surgical resection is the optimal treatment for HAML with favorable prognosis.

摘要

目的

探讨肝血管平滑肌脂肪瘤(HAML)的临床病理特征、诊断及治疗方法。

方法

回顾性分析1998年10月至2007年10月间接受治疗的51例HAML患者的临床、组织病理学、治疗及预后资料。

结果

HAML以女性多见(女/男=41/10),平均年龄44岁。主要症状为腹部肿块(33例)及腹痛或不适(15例),另2例表现为发热。组织病理学上,HAML由血管、平滑肌和脂肪细胞组成的异质性混合物构成。免疫组化染色显示HMB-45(50/51)、SMA(47/49)和S-100(39/42)的阳性率相对较高。51例患者均接受了肝部分切除术。平均住院时间为13.8天,平均术中出血量为263毫升。平均随访55.4个月后无复发或转移。

结论

HAML是一种罕见的肝脏良性间叶组织肿瘤。HAML的确诊依赖于病理组织学检查结果,HMB-45阳性的肌样细胞是重要的诊断标志物。完整的手术切除是HAML的最佳治疗方法,预后良好。

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