Rial Xiana, López-Monclús Javier, Lucena José Luis, Chaparro María Dolores, Pueyo Alberto, Giménez Alvira Luis, Jiménez Garrido Manuel, Martínez Arrieta Félix, Remírez Xabier, Sánchez Turrión Víctor
General and Digestive Surgery Department, Puerta de Hierro-Majadahonda University Hospital, Madrid, Spain.
General and Digestive Surgery Department, Puerta de Hierro-Majadahonda University Hospital, Madrid, Spain.
Transplant Proc. 2020 Jun;52(5):1453-1454. doi: 10.1016/j.transproceed.2020.01.090. Epub 2020 Mar 31.
Hepatic epithelioid hemangioendothelioma (HEHE) is a rare neoplasm of vascular origin, with nonspecific presentation and unpredictable clinical course. Several therapeutic options are available according to the literature, including chemotherapy and radiotherapy, liver resection (LR), and liver transplantation (LT).
We present 2 cases of patients with HEHE treated with liver transplantation in our center, a 30-year-old man and a 42-year-old woman, diagnosed with several lesions involving both liver lobes.
Levels of tumor markers (carcinoembryonic antigen [CEA], cancer antigen 19.9 [Ca19.9], and alpha-fetoprotein [AFP]) were negative. Percutaneous biopsy revealed HEHE in both cases. Due to the bilobar location, liver transplantation was performed. Postoperative course was unremarkable, and no significant complications developed. At this time, 10 and 5 years' follow-up has concluded, respectively. Both patients remain alive, asymptomatic, with normal liver function and no sign of recurrence.
HEHE is an uncommon malignant hepatic disease. Most cases present with bilobar involvement, and LT is probably the most useful treatment with favorable outcomes according to the literature and our experience. Further studies are needed to establish the optimal management of this rare entity.
肝上皮样血管内皮瘤(HEHE)是一种罕见的血管源性肿瘤,临床表现不具特异性,临床病程难以预测。根据文献,有多种治疗选择,包括化疗、放疗、肝切除术(LR)和肝移植(LT)。
我们报告了在我们中心接受肝移植治疗的2例HEHE患者,1例为30岁男性,1例为42岁女性,均被诊断为累及两肝叶的多处病变。
肿瘤标志物(癌胚抗原[CEA]、癌抗原19.9[Ca19.9]和甲胎蛋白[AFP])水平均为阴性。经皮活检在两例中均显示为HEHE。由于病变累及两叶,故实施了肝移植。术后过程平稳,未出现明显并发症。此时,分别已完成10年和5年的随访。两名患者均存活,无症状,肝功能正常,无复发迹象。
HEHE是一种罕见的肝脏恶性疾病。大多数病例累及两叶,根据文献及我们的经验,肝移植可能是最有效的治疗方法,预后良好。需要进一步研究以确立对这种罕见疾病的最佳管理方案。