儿童胰母细胞瘤的临床表现、CT表现与病理改变的特点

The Clinical Symptoms, CT Findings and the Pathology of Pancreatoblastoma in Children

目的探讨儿童胰母细胞瘤临床表现、CT表现与病理及免疫组化的特征,提高对该病的认识。方法回顾性分析4例经手术治疗后病理证实为胰母细胞瘤的术前的CT表现、临床表现与术后病理表现的特征,总结该病的特点。结果胰母细胞瘤临床表现为消化道症状、腹部包块、不同程度的贫血及肿瘤标准物NSE的升高。CT表现为腹腔巨大占位,多跨越中线,边界清,多伴有囊变坏死成分,增强后实性成分呈中度延迟性不均匀强化,由腹腔干分支供血,周围或腹膜后多有肿大淋巴结。上皮细胞构成的巢状小体是胰母细胞瘤典型的病理改变,EMA、CK、Ki-67和Syn是主要的阳性标志物。结论胰母细胞瘤临床表现、CT表现可提高该病诊断,但其确诊依靠病理。

Objective To describe the clinical symptoms, CT findings and the pathology, of pancreatoblastoma（PBL） in children and improve the diagnosis betbre surgery, Methods Clinical symptoms, CT findings and the pathology of 4 children proven PBL after surgery, were analyzed and summarized. Results The abdominal mass, anemia and the exaltation of NSE were the typical clinical symptoms. The huge mass crossing the middle line in abdominal with clear bonndary and cystic and necrosis inside, whose blood corned from celiac branch and substance were enhanced moderately and heterogeneously, and the lymph nodes enlarged around the mass or located in the retroperitoneal were the main CT findings. Epithelial cells arranged in nests were the typical pathology, and EMA, CK, Ki-67 and Syn were the main positive marker. Conclusions The clinical symptoms and the CT findings could improve the diagnosis, but the disease was confirmed by pathology.