肝移植治疗肝脏上皮样血管内皮瘤2例报告并文献复习

Liver transplantation for hepatic epithelioid hemangioendothelioma;report of two cases and review of the literature

目的探讨肝移植治疗肝脏上皮样血管内皮瘤的临床经验和疗效。方法回顾性分析2例肝脏上皮样血管内皮瘤患者肝移植诊疗经过和相关临床资料,并进行文献复习。结果 2例均为女性。术前腹部B超、CT均提示肝实质多发占位性病变:肝穿刺组织病理学检查均提示肝上皮样血管内皮瘤;免疫组化染色均显示肿瘤标志物CD31(+)、CD34(+)和FactorⅧ(+)。肝移植术后采用他克莫司(FK506)+吗替麦考酚酯(骁悉)二联免疫抑制方案,早期维持FK506血药浓度为8～12μg/L。分别于术后13 d及8 d肝功能恢复正常。术后早期1例并发急性阑尾炎,行阑尾切除术后治愈,2例均未发生急性排斥反应,康复出院。随访2年余移植物、受体正常存活,无肿瘤复发。结论肝移植是治疗不能手术切除的肝脏上皮样血管内皮瘤的最有效的治疗措施,术后效果满意。

Objective To investigate experience of liver transplantation for hepatic epithelioid hemangioendothelioma（EH） in diagnosis and treatment.Methods Clinical data and experiences of orthotopic liver transplantation（OLT） in two recipients,with primary disease of hepatic EH,were retrospectively reviewed and the relative literature was revised.Results Two cases were female.Both abdominal ultrasound examination and computed tomography showed preoperatively numerous small low echo-level and low-density spots in the liver in two cases.The histopathological diagnosis from a needle liver biopsy was epithelioid hemangioendothelioma in two cases.Immunohistochemistry revealed that epithelioid tumor cells were positive for CD31,CD34 and factorⅧ-related antigens,which have been previously described as hepatic EH markers.The protocol of immunosuppression was postoperatively tacrolimus（FK506） and mycophenlate mofetil,and the blood level of FK506 was maintained at about 8-12μg/L in early stage.Liver function recovered normal at postoperative 13 days in Case One and 8 days in Case Two,respectively.Case One suffered from acute appendicitis after OLT and recovered via appendectomy.No rejection occurred.Two cases smoothly discharged and within more than two years follow-up the two recipients presented with good liver function and no signs of tumor recurrence. Conclusion OLT is the most effective therapeutic measure for unresectable hepatic EH and presented postoperativly a satisfactory prognosis.