肝未分化(胚胎性)肉瘤的CT表现和病理特点

摘要

目的 分析肝未分化(胚胎性)肉瘤的CT影像和病理特点,以提高对这种罕见肿瘤的认识.方法 2000-2006年间,10例病理证实的肝未分化(胚胎性)肉瘤病人(5～56岁),对其临床、CT影像及病理特点进行回顾性分析.结果 肿瘤直径平均为11.2 cm(范围9～25 cm);CT影像表现为多房、囊实性,囊性为主(略高于水密度),增强后囊壁下软组织及分隔可见强化.肿瘤切面囊实性,主要包括出血坏死、凝胶样内容物和肿瘤组织,肿瘤组织由多种间叶性肉瘤成分组成,异型明显,另可见较多多形性细胞和瘤巨细胞,在细胞内和间质中有特征性小圆形嗜酸性小体.免疫组化肿瘤呈多种间叶性成分分化.结论 肝未分化(胚胎性)肉瘤恶性程度高、预后差.在青少年或儿童病人中发现肝脏囊实性占位,应考虑肝UES可能,尽早手术及综合治疗能改善预后.%Objective To analyze the CT findings and pathological features of the undifferenti-ated embryonic sarcoma (UES) of the liver to improve the diagnostic accuracy of this rare liver malig-nancy. Methods The clinical, pathological and CT findings in 10 patients (aged 5-56 years) with pathologically proved UES were retrospectively analyzed. Results The mean transverse diameter of the tumors was 11.2 cm (9-25 cm). CT scan showed muhiloculated cystic mass in the different lobe of the liver. All tumors demonstrated predominantly water attenuation. But they contained irregular areas of higher density under the capsule and multiple internal septations of varying thickness. On dy-namic contrast CT, these parts of the tumors may have a marked enhancement. The cut surface of tumors was variegated, with solid and cystic gelatinous areas, hemorrhage and necrosis, or both.Tumor cells showed pluripotential differentiation, greater cellularity, anaplasia and muhinucleated cells. Intracellular and extracellular distinctive hyaline globules were commonly present. Immunohis-tochemistry showed evidence of widely divergent differentiation into mesenchymal and epithelial pheno-types. Conclusions UES is an unusual malignancy of the liver and patients with these tumors report-edly have a poor prognosis. In a child or young adult with a liver tumor, these findings are useful in making a prospective diagnosis of UES.