成人肝脏未分化胚胎性肉瘤的临床表现和CT诊断

Clinical Manifestations and Spiral CT Diagnosis of Undifferentiated Embryonal Sarcoma of Liver in Adults

目的:探讨成人肝脏未分化胚胎性肉瘤的临床和CT表现,以提高该病的术前诊断水平。方法:回顾性分析6例经手术切除并经病理证实的成人肝脏未分化胚胎性肉瘤的临床和CT表现,并结合文献分析。结果:成人肝脏未分化胚胎性肉瘤多见于女性,临床表现为与肿瘤相关的症状,但发热多见(5/6例),未见明显黄疸及腹水报道;患者肝功能正常(3例)或轻度异常(3例),血清肿瘤标记物检查阴性。CT表现为囊实性肿块,边界清楚,出血多见。本组病例肿块最大径约6～13cm,单发团块状,仅1例肝左叶并存肝细胞癌患者合并肝硬化。4例CT平扫表现为团块状不均质低密度影,边界较清楚,其内见散在分布的条带状高密度出血影,实性成分动脉期轻中度强化,静脉期强化稍明显;2例CT平扫呈囊实性低密度影,实性成分位于病灶后缘,于动脉期较明显强化,静脉期强化程度减退。结论:成人肝脏未分化胚胎性肉瘤的CT征象有一定的特征性,同时结合临床表现,有利于提高该病的术前诊断水平。

Objective：To summarize the clinical characteristics and spiral CT appearances of undifferentiated embryonal sarcoma of liver in adults and improve the diagnostic accuracy. Methods.. CT findings of 6 adults with undifferentiated embryonal sarcoma of liver proved pathologically and surgically were analyzed retrospectively and compared with literature. All cases received plain and enhanced spiral CT scanning. Results：Undifferentiated embryonal sarcoma of liver in adults was mostly seen in female. The most common presenting complaint was abdominal mass or related pain on the right upper quadrant. Intermittent fever was noted in 5 patients. None had jaundice. AFP levels were normal in all,and the results of liver function test were mildly abnormal in 3. HBsAg were all negative. Only one case with hepatic carcinoma in the left lobe had liver cirrhosis. On CT,the cystic-solid mass tumor was well circumscribed,measuring 6- 13cm in the largest diameter. The solid portions were peripheral and had a small size,which showed different degrees of enhancement. Among them,4 masses had large amount of hemorrhage which showed irregular high-density. One mass compressed inferior vena cava. Conclusion： The CT appearances of undifferentiated embryonal sarcoma of liver in adults have certain characteristics,and the combination with clinical manifestations is helpful to the diagnosis.