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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 summarize the clinical and pathological features of adult infantile hepatic hemangioendothelioma (IHHE) and to improve the diagnosis and treatment of the disease. Methods: The clinical and pathological data of 7 adult IHHE patients (32-63 years old) who were surgically treated and pathologically confirmed from January 2003 to December 2013 in the Hepatobiliary Surgery Hospital of Guangdong Province were retrospectively analyzed. Results: Of the 7 patients, 5 patients had no obvious clinical symptoms before operation, the other 2 patients showed intermittent pain and loss of appetite in the right upper quadrant, respectively. There was no obvious abnormalities in blood and liver function indexes. The tumor index and hepatitis virus index Negative, CD34 immunohistochemistry were positive; lesions were single, and preoperative imaging examination were not diagnosed before surgery. All patients were followed up regularly until August 2014, and no tumor recurrence occurred. CONCLUSIONS: IHHE is extremely rare in adult liver tumors. Clinically, patients with no hepatitis background and tumor markers are negative. Combined with CT and MRI findings, IHHE may be able to consider the possibility of cavernous hemangiomas.