原位肝移植治疗肝脏上皮样血管内皮瘤(附2例报告及文献复习)

Orthotopic liver transplantation for treatment of hepatic epithelioid hemangioendothelioma: report of two cases and literature review

目的探讨原位肝移植治疗肝脏上皮样血管内皮瘤(EHE)的疗效及相关治疗进展。方法回顾性分析2例肝脏EHE患者的临床资料。例1为肝脏广泛多发EHE合并脾脏多发梗死灶的患者,实施了原位肝移植+脾切除术;例2为肝脏多发EHE患者,仅实施了原位肝移植。结合国内外文献,分析肝脏EHE患者的病理学特点、肝移植治疗及预后情况。结果两例患者手术过程顺利,均康复出院。病理结果证实为肝脏EHE,例1合并脾脏EHE。例1术后1个月抗排斥反应药由他克莫司换为西罗莫司,术后4个月EHE复发,给予卡培他滨口服化学药物治疗(化疗);术后6个月考虑肝脏EHE复发并多发性骨转移,予索拉非尼口服抗肿瘤治疗,术后7个月患者死于恶病质、肝衰竭。例2随访至投稿日(术后8个月),未见术后相关并发症及肿瘤复发迹象。国内外文献报道提示肝脏EHE首选手术切除,肝内广泛多发而无法手术切除者,甚至有肝外病灶者,肝移植疗效最佳。另外,化疗、经皮穿刺肝动脉化疗栓塞和抗血管生成治疗对本病也有一定疗效。结论肝脏EHE治疗首选手术切除,对于肝内广泛多发者,肝移植被认为是最佳的治疗选择。应重视肝脏EHE移植术后复发的防治,以期进一步提高该病的疗效。

Objective To investigate the clinical efficacy and therapeutic progress of orthotopic liver transplantation for the treatment of hepatic epithelioid hemangioendothelioma （ EHE ） . Methods Clinical data of 2 patients diagnosed with hepatic EHE were retrospectively analyzed. One patient （ case 1 ） was diagnosed with multiple hepatic EHE complicated with multiple infarction lesions of the spleen, and underwent orthotopic liver transplantation combined with splenectomy. The other case （ case 2 ） was diagnosed with multiple hepatic EHE and received orthotopic liver transplantation alone. Literature review was performed. Pathological characteristics, clinical efficacy of liver transplantation and clinical prognosis of hepatic EHE patients were analyzed. Results Two patients successfully underwent surgery and were discharged postoperatively. The diagnosis of hepatic EHE was confirmed by pathological examination and case 1 was complicated with EHE of the spleen. For case l, tacrolimus was replaced by sirolimus at postoperative 1 month. At postoperative 4 months, capecitabine was orally administered （ chemotherapy ） for EHE recurrence. At 6 months after surgery, the patient was diagnosed with recurrent hepatic EHE complicated with multiple bone metastases, and was orally administered with sorafenib. At postoperative 7 months, the patient died from cachexia and liver failure. Case 2 was followed up until the submission date （ 8 months after surgery ） . No postoperative complications and tumor recurrence were observed. Previous studies had demonstrated that surgical resection was the primary therapy of hepatic EHE. Liver transplantation was highly recommended for patients with multiple unresectable hepatic EHE and extra-hepatic lesions. Moreover, chemotherapy, percutaneous puncture combined with transcatheter arterial chemoembolization and anti-angiogenesis treatment exerted certain clinical efficacy. Conclusions Surgical resection remains the primary therapy of hepatic EHE. For patients with multiple intrahepatic EHE, liver transplantation is considered as the optimal treatment. Much attention should be diverted to the prevention and treatment of recurrent hepatic EHE following liver transplantation, aiming to improve the clinical efficacy.