儿童胰母细胞瘤6例临床分析并文献回顾  

Clinical analysis of 6 children with pancreatoblastoma and literature review

作　　者：李媛[1] 王军[1] 王建文[1] 柴丽娜 冯晨[1] LI Yuan;WANG Jun;WANG Jianwen;CHAI Li’na;FENG Chen(Department of Pediatrics,the First Medical Center,Chinese PLA General Hospital,Beijing 100853,China)

机构地区：[1]解放军总医院第一医学中心儿科,北京100853

出　　处：《解放军医学院学报》2023年第1期23-27,32,共6页Academic Journal of Chinese PLA Medical School

摘　　要：背景 胰母细胞瘤(pancreatoblastoma,PBL)是一种罕见的肿瘤,但却是儿童最常见的胰腺恶性肿瘤,目前国内外报道仅几百例,大样本研究报道更少,临床上可因认识不足而误诊或治疗方式不当影响预后。目的 分析儿童PBL的临床特点,提高对其的认识和诊治水平。方法 回顾性分析2012年5月-2021年8月解放军总医院第一医学中心收治的6例PBL临床特征、辅助检查、治疗转归等。结果 6例PBL中,男5例,女1例;中位年龄4岁7个月。肿瘤位于胰头体交界处2例,胰体尾部1例,胰尾部2例,全胰1例;门静脉受累3例,脾静脉受累2例,淋巴结转移1例,肝内多发转移1例,腹膜转移1例。肿瘤分期Ⅰ期2例,Ⅳ期4例。6例均出现腹痛,2例发现腹部包块,1例早饱、消瘦,1例呕吐、食欲缺乏,1例出现盆腹腔大量积液。AFP升高3例。肿物超声检查结果:等回声4例,不均质回声1例,低回声1例,伴钙化3例。肿物CT扫描显示:高低混杂密度1例,等密度及低密度1例,低密度3例,囊实性1例,轻度强化3例,中度强化1例。免疫组化检查结果显示:1例无免疫组化报告,其余5例中,4例Syn阳性,1例CD56阳性,1例CD10阳性,1例AFP阳性,3例CK19阳性,2例AAT阳性,无CEA阳性。3例行辅助化疗+手术切除,分别随访126个月、46个月、40个月无复发;1例仅行手术切除,随访116个月无复发;2例行辅助化疗,肿瘤缩小但仍无法手术,其中1例放弃治疗死亡,1例暂停治疗5个月肿物无继续进展。结论 PBL是儿童常见的胰腺恶性肿瘤,腹痛、腹部肿块是最常见的临床表现,AFP常明显升高,超声、CT均为可靠的辅助诊断手段。治疗首选完整手术切除,辅助化疗有助于肿瘤完整切除和改善预后,但化疗方案的设计仍需多中心大样本研究。Background Pancreatoblastoma(PBL) is a rare tumor, but it is the most common pancreatic malignant tumor in children.At present, only a few hundred cases have been reported at home and abroad, and there are fewer reports from multi-center and large-sample studies. Misdiagnosis or improper treatment may affect the prognosis due to insufficient understanding of PBL.Objective To improve the understanding, diagnosis and treatment of PBL by analyzing the clinical features of children with PBL.Methods A retrospective analysis was performed on 6 children with PBL admitted to the First Medical Center of Chinese PLA General Hospital from May 2012 to August 2021, and their clinical features, auxiliary examinations, treatments and outcomes were summarized. Results Among the 6 cases, there were 5 boys and 1 girl, with a median age of 4 years and 7 months. The tumors were located at the junction of the head and body of the pancreas in 2 cases, the body-tail of the pancreas in 1 case, the tail of the pancreas in 2 cases, the whole pancreas in 1 case. There were 3 cases of portal vein involvement, 2 cases of splenic vein involvement, 1 case of lymph node metastasis, 1 case of multiple intrahepatic metastasis, and 1 case of peritoneal metastasis. Two cases were classified in stage I, 4 cases were in stage Ⅳ. Abdominal pain occurred in all cases. There were 2 cases of abdominal mass, 1 case of early satiety and weight loss, 1 case of vomiting and poor appetite, and 1 case of massive pelvic and abdominal effusion. AFP increased in 3 cases. Ultrasound(US) examination showed that lesions in 4 cases were isoechoic, 1 case inhomogeneous echo, 1 case hypoechoic, and 3 cases with calcification. Computed tomography(CT) of the tumors showed uneven density(high and low mixed)in 1 case, uneven density(equal and low mixed) in 1 case, low density in 3 cases, cystic solid in 1 case, 3 cases of, mild enhancement in 1 case. The immunohistochemical results showed that 1 case had no immunohistochemistry report;of the other 5 cases, 4 cases were S

关 键 词：胰母细胞瘤 儿童 临床表现 治疗 预后 

分 类 号：R735.9[医药卫生—肿瘤]