肝上皮样血管内皮瘤5例分析并文献复习

Analysis of 5 Cases with Hepatic Epithelioid Hemangioendothelioma and Literature Review

背景:肝上皮样血管内皮瘤是一种罕见的血管生成肿瘤,目前尚无公认的有效治疗方法。世界范围内使用的治疗方法包括肝移植、肝切除术、射频消融、化疗和观察。本研究的目的是分析我们医院用于肝上皮样血管内皮瘤的不同治疗方案的疗效。方法:回顾性分析2013年1月至2019年12月在青岛大学附属医院诊治的5例肝上皮样血管内皮瘤患者的病历资料。结果:患者均经术后病理或细针穿刺活检确诊。其中1例肝脏弥漫性病变的患者接受了肝移植,并在最后一次随访中无复发存活;1例患者接受肝切除术作为初始治疗,在随访期间出现复发;1例患者接受了射频消融术,并保持无病;其余2例患者选择了随诊观察而不治疗。1例接受肝切除术的患者术后32个月内因肿瘤复发去世,1例观察未治疗患者随访21个月后拒绝治疗而去世,其他患者在治疗复发病灶后未出现明显的疾病活动。随访截至2019年12月,平均随访时间为42.6 ±28.8个月(15~82个月)。结论:肝上皮样血管内皮瘤治疗有多种方法。考虑到其惰性临床特点,评估病变行为的初步观察可能有助于选择适当的治疗方法。对于观察期内病情进展的患者可以考虑手术或肝移植。然而,由于我们的样本量很小,需要进一步的研究来分析最佳治疗方案的选择。

Background: Hepatic epithelioid hemangioendothelioma is a rare angiogenic tumor and there is currently no recognized effective treatment. Treatments are used worldwide including liver transplantation, liver resection, radiofrequency ablation, chemotherapy and observation. The purpose of this study was to analyze the efficacy of different treatment regimens for hepatic epithelioid hemangioendothelioma in our hospital. Methods: Medical records of 5 patients with hepatic epithelioid hemangioendothelioma treated in the Affiliated Hospital of Qingdao University from January 2013 to December 2019 were retrospectively analyzed. Results: All patients were confirmed by postoperative pathology or fine needle aspiration biopsy. One of the patients with diffuse liver disease received liver transplantation and was recurrence-free at the last follow-up. One patient received hepatectomy as initial treatment and relapsed during follow-up. One patient received radiofrequency ablation and remained disease free, while the remaining two patients opted for follow-up observation without treatment. One patient who received hepatectomy died of tumor recurrence within 32 months after surgery and one patient who was not treated under observation died after being followed up for 21 months and refused treatment. Other patients did not show significant disease activity after treatment of their recurrent lesions. As of December 2019, the mean follow-up time was 42.6 ±28.8 months (15~82 months). Conclusion: There are many treatments of hepatic epithelioid hemangioendothelioma. Given its indolent course, an initial observation on the assessment of lesion behavior may help select an appropriate treatment. Surgery or liver transplantation may be considered for patients whose disease progresses during the observation period. However, due to our small sample size, further studies are required to gather more information that can aid in optimal treatment selection.