肝脏血管平滑肌脂肪瘤的临床病理分析

目的 探讨肝脏血管平滑肌脂肪瘤(HAML)的临床病理特点及诊治方法.方法 对1998年10月至2007年10月复旦大学附属中山医院肝脏外科收治的51例HAML患者的临床表现、病理特点、诊治及预后进行回顾性分析.结果 本组男性10例,女性41例,平均年龄44岁,症状以腹部肿块(33例)和腹部疼痛不适(15例)为主,少数表现为发热(2例).肿瘤由血管、平滑肌和脂肪细胞组成,免疫组织化学染色显示HMB-45(50/51)、SMA(47/49)和S-100(39/42)的阳性率较高.51例HAML均手术切除,平均住院时间13.8 d,术中平均出血263 ml,平均随访时间为55.4个月,无一例出现复发或转移.结论 HAML为肝脏少见良性间质瘤,确诊依赖于病理学及组织化学检查,HBM-45阳性肌细胞是重要的诊断标志.手术切除是最佳治疗手段,预后好.

A clinicopathologic study of hepatic angiomyolipoma

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, HALM 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.