16例肝脏未分化性胚胎性肉瘤术前误诊分析  

Analysis of 16 cases misdiagnosed undifferentiated embryonal sarcoma of the liver

作　　者：李妍卓 蔡权宇 贾宁阳[1] 陈栋[1] 陆伦[1] 程红岩[1] 

机构地区：[1]第二军医大学附属东方肝胆外科医院放射科,上海200438

出　　处：《临床与病理杂志》2015年第4期615-621,共7页Journal of Clinical and Pathological Research

摘　　要：目的:探讨肝脏未分化性胚胎性肉瘤(undifferentiated embryonal sarcoma of the liver,UESL)的临床特点,加深对该病的认识,提高术前诊断准确性。方法:回顾性分析16例经手术病理证实、术前误诊UESL患者的临床、影像及病理资料,并对比分析。结果:16例患者中诊断为原发性肝癌9例,肝母细胞瘤3例,肝内恶性占位4例。12例以腹痛不适就诊;超声显示实性为主肿瘤,而CT及MRI呈囊性为主的肿瘤,瘤内成分混杂;增强后病灶内囊壁下软组织、分隔及边缘可见强化。术后病理见肿瘤切面呈囊实性,内见不同程度出血坏死、凝胶样区域和肿瘤组织,癌细胞间可见特征性嗜酸性小体。结论:未分化胚胎性肉瘤临床罕见,临床资料无明显特异性,超声与CT、MRI表现不一致性为重要鉴别要点,有助于提高术前诊断率。Objective： To investigate the clinical features of undifferentiated embryonal sarcoma of the liver （UESL） to improve its preoperative diagnostic accuracy. Methods： Retrospectively analysis the clinical, imaging and histopathologic findings of 16 cases preoperatively misdiagnosed UESL which were all pathologically confirmed. Results： Among these 16 cases, clinically diagnosed primary liver carcinoma 9 cases, hepatoblastoma 3 cases, malignant hepatic tumor 4 cases. Twelve patients mainly presented abdominal discomfort. Sonograms revealed predominant solid lesions; CT and MRI demonstrated predominant cystic masses within irregular soft tissue. Contrast-enhanced images showed intralesional foci, multiple internal septations andedges enhancement. qhe postoperative pathology showed the cut surface of tumors was variegated, with solid and cystic gelatinous areas, hemorrhage and necrosis. Intracytoplasmic hyaline globules were commonly present among cancer cells. Conclusion： UESL is a rare malignancy of the liver with no specific clinical manifestation, imaging finding inconsistent between ultrasound, CT and MRI which might be helpful to improve the preoperative diagnosis accuracy

关 键 词：肝肿瘤 未分化性胚胎性肉瘤 影像学表现 凝胶样区域 

分 类 号：R735.7[医药卫生—肿瘤]