儿童胰母细胞瘤影像特征及相关临床病理研究

Study of Imaging Features and Clinicopathological Correlation of Pancreatoblastoma in Children

目的:探讨儿童胰母细胞瘤(PBL)的影像特征及相关临床病理。方法:回顾性收集2013年6月—2020年7月上海交通大学医学院附属新华医院经穿刺活检或手术病理确诊为胰母细胞瘤的9例患儿资料,分析所有患儿的CT及MRI影像表现,对肿瘤的部位、形态、大小、边缘及包膜、密度/信号特点、肿块强化方式、肿瘤的血供、肝内外胆管及胰管扩张、对周围组织的侵犯及远处转移情况与术中及术后病理进行符合性比较。结果:9例患儿中肿瘤位于胰头部5例,胰体1例,胰尾3例。最大径2.8~10.9 cm,平均6.9 cm。7例见囊变或坏死,2例均匀实性。出现钙化5例,均未见出血。增强呈不均匀强化7例,其中5例实性成分门脉期强化接近胰腺实质,2例稍弱于胰腺实质。增强后肿瘤动脉期边缘及内部见细小血管穿行者8例。6例甲胎蛋白(AFP)及神经元特异性烯醇化酶(NSE)升高。结论:胰母细胞瘤的CT和MRI影像表现具有一定特征,对术前准确诊断及评估肿瘤恶性风险具有重要价值。

Purpose: To investigate the imaging characteristics and clinicopathological correlation of children with pancreatoblastoma(PBL).Methods: A retrospective study was performed on 9 children who were diagnosed as pancreatoblastoma by puncture biopsy or surgical pathology in Xinhua Hospital affiliated to Shanghai Jiao Tong University School of Medicine from June 2013 to July 2020. CT and MRI images of all the children were analyzed.The site, shape, size, edge and capsule of the tumor, density/signal characteristics, tumor enhancement, blood supply,expansion of intrahepatic and extrahepatic bile ducts and pancreatic ducts, invasion of surrounding tissues and distant metastasis were compared with intraoperative and postoperative pathology results.Results: The tumors were located in the head(5 cases), body(1 case) and tail(3 cases) of pancreas. Maximum tumor diameter was 2.8-10.9 cm, with an average of 6.9 cm. Cystic lesions or necroses were observed in 7 cases and homogenous consolidation was observed in 2 cases. Calcification occurred in 5 cases without bleeding. The heterogeneous enhancement was seen in 7 cases,and the degree of enhancement was close to pancreatic parenchyma in 5 cases and slightly weaker in 2 cases on the venous phase image. After the intravenous administration of contrast material, small vessels were seen in 8 cases at the margin and inside of the tumor on the arterial phase image. Alpha fetoprotein(AFP) and neuron specific enolase(NSE) were elevated in 6 cases. Conclusion: CT and MRI imaging features of pancreatoblastoma have certain characteristics, which are of great value for accurate preoperative diagnosis and assessment of malignant risk of tumor.