肝脏血管平滑肌脂肪瘤的诊断和治疗

Diagnosis and treatment of hepatic angiomyolipoma

目的总结肝脏血管平滑肌脂肪瘤(HAML)的临床、影像学和病理学特点以及治疗方法。方法回顾性分析2003年11月至2007年5月我科收治的6例HAML患者的临床资料。所有患者行B超、CT检查之后均行肝切除术,术后经病理及免疫组化明确诊断并随访至今。结果6例患者中女5例,男1例,多无明显临床症状,且血清学检查正常。影像学显示肿瘤形态规则、边界清晰,增强后早期及延迟增强。术中发现肿瘤位于右肝3例、左肝3例,大小为4cm×4cm×3cm～17cm×10cm×10cm。病理结果提示病灶由不同比例的血管、平滑肌细胞及脂肪组成,免疫组化结果HMB-45(6/6)、SMA(6/6)表达阳性。所有病例手术切除后随访6～48个月,至今无复发。结论HAML在肝脏占位中并不多见,肿瘤以良性为主,但存在恶性生物学行为可能。外科医师术前诊断时应有HAML的概念,一旦诊断宜尽早手术,术后密切随诊。

Objective Hepatic angiomyolipoma （HAML） is a rare liver tumor. This study was to analyze and summarize the clinical,imaging,and pathological characteristics, and the treatment of HAML by combining our experiences with the literatures. Methods The clinical data of 6 patients with HAML treated in our Department from November 2003 to May 2007 were retrospectively reviewed. All the patients received liver resection after the imaging examinations. The final diagnosis was made by pathology and immunohistochemistry. The patients were followed up till now. Results There was a marked female predominance （F： M = 5：1 ）, and most of the patients had no symptom. The liver functions and AFP of all patients were normal. The lesions showed regular, well-circumscribed figures in both plain and enhanced imagings of the arterial phase and delayed phase. There were 3 tumors located in the right lobe, and 3 in the left lobe. The range of the tumor size is 4 cm ×4 cm × 3 cm- 17 cm × 10 cm × 10 cm. The nature of the tumors were mixture of thick-walled blood vessels, smooth muscle and mature fat. Immunohistochemistry showed that all lesions were positive for HMB-45 and SMA. Follow up study showed no signs of recurrence in all patients. Conclusion HAML has low incidence presenting in the liver. It is generally considered as a benign tumor,yet has the potentially of malignant. As soon as the diagnosis of HAML is confirmed,a surgical operation should be scheduled. The post-operative follow-up is necessary.