Selection of treatment for hepatic epithelioid hemangioendothelioma: a single-center experience

Selection of treatment for hepatic epithelioid hemangioendothelioma: a single-center experience
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肝上皮样血管内皮瘤的治疗选择:单中心经验

DOI:
10.1186/s12957-019-1729-y
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发表时间:
2019-11-07
影响因子:
3.2
通讯作者:
Zheng, Shusen
Zheng, Shusen
中科院分区:
医学3区
文献类型:
--
作者:
Cao, Linping;Hong, Jiawei;Zheng, Shusen

文献摘要

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背景肝上皮样血管内皮瘤(HEHE)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。全球使用的治疗方案包括肝移植 (LT)、肝切除 (LR)、射频消融 (RFA)、化疗和观察。本研究的目的是描述我中心针对HEHE不同治疗方案的疗效。方法回顾性分析2011年1月至2017年12月在浙江大学第一附属医院诊断和治疗的12例HEHE患者(9女3男)的病历。结果患者均经术后组织病理学或细针穿刺活检确诊。两名弥漫性病灶患者接受了 LT,末次随访时仍存活,未出现复发。 3名患者接受LR作为初始治疗,随访期间均出现复发。一名患者接受 RFA 后仍无疾病,而其余 6 名患者选择简单观察而不是治疗。其中一名接受LR的患者在术后32个月内因肿瘤复发去世;其他患者在治疗复发性病变后没有表现出明显的疾病活动性。截至2018年4月,平均随访时间为39.6±20.1个月(15-82个月)。 结论 HEHE有多种治疗策略。考虑到其惰性病程,对病变行为评估的初步观察可能有助于选择适当的治疗方法。手术或 LT 适合观察期内疾病进展的患者。然而,我们的样本量很小,需要进一步的研究来收集更多有助于最佳治疗选择的信息。
BackgroundHepatic epithelioid hemangioendothelioma (HEHE) is a rare angiogenic tumor with no recognized effective treatment. Treatment options used worldwide include liver transplantation (LT), liver resection (LR), radiofrequency ablation (RFA), chemotherapy, and observation. The aim of this study was to describe the efficacy of different treatment options used for HEHE at our center.MethodsThe medical charts of 12 patients with HEHE (9 women and 3 men) who were diagnosed and treated at the First Affiliated Hospital of Zhejiang University, China, between January 2011 and December 2017 were retrospectively reviewed.ResultsThe patients were diagnosed by postoperative histopathology or fine needle aspiration biopsy. Two patients with diffuse lesions received LT and were alive without recurrence at the last follow-up. Three patients received LR as the initial treatment, and all of them developed recurrence during the follow-up period. One patient received RFA and remained free of disease, while the remaining six patients opted for simple observation rather than treatment. One of the patients who received LR passed away because of tumor recurrence within 32 months after surgery; the other patients showed no significant disease activity after treatments for their recurrent lesions. As of April 2018, the mean follow-up duration was 39.6 ± 20.1 months (15–82 months).ConclusionsThere are multiple strategies for HEHE. Considering its indolent course, initial observation for assessment of the lesion behavior may aid in the selection of appropriate treatment. Surgery or LT is suitable for patients with disease progression during the observation period. However, our sample size was small, and further studies are required to gather more information that can aid in optimal treatment selection.