成人婴儿型肝脏血管内皮细胞瘤的诊治:附7例报告

Diagnosis and treatment of infantile hepatic hemangioendothelioma in adults: a report of 7 cases

目的：总结成人婴儿型肝脏血管内皮细胞瘤（IHHE）的临床病理特征,提高对该病的诊治水平。方法：回顾性分析2003年1月—2013年12月东方肝胆外科医院手术治疗并病理证实的7例成年IHHE患者（32~63岁）临床病理资料。结果：7例患者中,5例患者术前无明显临床症状,另2例分别表现为右上腹间隙性疼痛和食欲减退;血常规、肝功能指标均无明显异常,肿瘤指标、肝炎病毒指标均为阴性,CD34免疫组化均为阳性;病灶均为单个,且术前影像学检查术前均未确诊。所有患者术后均定期随访,至2014年8月,未出现肿瘤复发。结论：IHHE在成人肝脏肿瘤中极为罕见,临床上患者无肝炎背景,肿瘤指标均为阴性,结合CT及MRI表现,排除海绵状血管瘤,可以考虑该病的可能。

Objective： To review the clinicopathologic features of the infantile hepatic hemangioendothelioma （IHHE） in adults, so as to improve the diagnosis and treatment for this disease. Methods： The clinical data of 7 adult patients （age ranged from 32 to 63 years） with pathological confirmation of IHHE, who underwent surgical treatment from January 2003 to December 2013 in Eastern Hepatobiliary Surgery Hospital, were retrospectively analyzed. Results： Of the 7 patients, 5 cases had no obvious clinical symptoms before operation, and the other cases had intermittent pain in the right upper abdomen and anorexia, respectively, The routine blood tests and liver function parameters showed no obvious abnormality, tumor markers and hepatitis virus indexes were all negative, and results of the CD34 immunohistochemical staining were all positive. All cases had a solitary lesion, and diagnosis was not confirmed by preoperative imaging examination. All patients were followed up regularly after operation, and no tumor recurrence was noted, as of August 2014. Conclusion： IHHE in adults is an extremely rare condition, and in cases without a history of hepatitis or positive result of tumor markers, and after exclusion of cavernous hemangioma by CT and MRI findings, this condition should be considered.