Honsová E, Gottfriedová H, Oliverius M, Trunecka P
Department of Clinical and Transplant Pathology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic.
Prague Med Rep. 2009;110(3):214-21.
Primary hepatic epitheloid heamangioendothelioma (EHE) is a rare neoplasm of vascular origin. We present a retrospective study of 6 patients with EHE treated by liver transplantation that were monitored clinically for more than 3 years and had protocol biopsy samples taken at 1, 3, 5, 7, and 10 years posttransplant. None of the patients suffered from any form of viral or autoimmune hepatitis before or after the transplantation. Two patients had lung metastases detected by preoperative imaging. All tumours showed factor VIII, CD31, and CD34 strong positive staining. In 5 of the 6 transplant recipients the protocol graft biopsies showed chronic non-specific hepatitis with slowly progressive periportal fibrosis that appeared during the 3rd post-transplant year. The septal fibrosis was diagnosed in the 6th and 10th year after transplantation. Liver tests did not reflect either the presence or the degree of inflammation or fibrosis and have remained normal. In retrospect, we consider that our recipients most probably developed alloantigen dependent inflammatory and fibrotic damage to their liver grafts. All six recipients are still alive for a median survival time of 95.1 month (range 44 months to 132 months), with good graft function, and without recurrence of the tumor. The lung metastases in 2 of the 6 patients have remained unchanged for 10 and 12 years retrospectively.
原发性肝上皮样血管内皮瘤（EHE）是一种罕见的血管源性肿瘤。我们对6例接受肝移植治疗的EHE患者进行了一项回顾性研究，这些患者接受了超过3年的临床监测，并在移植后1年、3年、5年、7年和10年采集了方案活检样本。所有患者在移植前后均未患任何形式的病毒性或自身免疫性肝炎。2例患者术前影像学检查发现有肺转移。所有肿瘤均显示因子VIII、CD31和CD34强阳性染色。6例移植受者中有5例的方案移植肝活检显示为慢性非特异性肝炎，伴有移植后第3年出现的缓慢进展的汇管区纤维化。在移植后第6年和第10年诊断出间隔纤维化。肝功能检查既未反映炎症或纤维化的存在，也未反映其程度，且一直保持正常。回顾性分析，我们认为我们的受者很可能对其移植肝发生了同种抗原依赖性炎症和纤维化损伤。所有6例受者均存活，中位生存时间为95.1个月（范围44个月至132个月），移植肝功能良好，且无肿瘤复发。回顾性分析，6例患者中有2例的肺转移在10年和12年内保持不变。
