儿童胰腺实性-假乳头状瘤的临床病理分析  

The clinical and pathological analysis of solide-pseudopapillary neoplasms of the pancreas in children

作　　者：邹继珍[1] 蔡玲玲[1] 肖萍[1] 何偲[1] 白云[1] 刘丹 刘菁[2] 张晓伦[2] ZOU Jizhen;CAI Lingling;XIAO Ping;HE Si;BAI Yun;LIU Dan;LIU Jing;ZHANG Xiaolun(Department of Pathology,Capital Institute of Pediatrics,Beijing 100020,China;Department of Surgical Oncology,Capital Institute of Pediatrics,Beijing 100020,China)

机构地区：[1]首都儿科研究所病理科,北京100020 [2]首都儿科研究所肿瘤外科

出　　处：《中国小儿血液与肿瘤杂志》2018年第6期301-306,共6页Journal of China Pediatric Blood and Cancer

摘　　要：目的探讨儿童发生的胰腺实性-假乳头状肿瘤的临床、病理特点及鉴别诊断。方法对我院2005年-2016年确诊的21例临床资料及病理特点进行回顾性分析。结果男5例,女16例,发病年龄平均10.3(5～15.5)岁,临床表现:上腹痛12例(5例伴呕吐,1例伴皮肤巩膜黄染、大便色浅及尿色深;2例因急腹症入院),发现腹部包块9例。影像学(超声=21,CT=20,MRI=9)均提示胰腺囊和(或)实性占位。实验室检查:除3例淀粉酶和白细胞升高外,余未见异常。21例均行胰腺肿瘤切除术(其中3例同时行Whipple手术),1例先行胰腺囊肿内引流术后,再择期行肿瘤切除。肿瘤部位:胰头10例,胰体3例,胰体尾4例,胰尾4例。全部患者经术后病理确诊后,仅密切随访未再行化疗。随访结果:仅1例术后11个月复发,再次手术切除;所有患儿仍在随访中。结论胰腺实性-假乳头状肿瘤属较罕见的低度恶性胰腺外分泌肿瘤。临床表现及影像学检查均无特征性。病理组织学见上皮样细胞形成的实性或假乳头状结构。若瘤体较大,且有明确的包膜、血管及周围神经的浸润,异型性明显及核分裂活性增加,以及肿瘤坏死明显等,则提示恶性的可能。肿瘤的完整切除既是主要的治疗手段,也是预后相关的重要因素。Objective To analyze the clinical and pathological characteristics,diagnosis and differential diagnosis,as well as the prognosis of solide-pseudopapillary neoplasms of the pancreas(SPN)in children.Methods The clinical and pathological data of 21 cases with SPN were retrospectively analyzed and followed-up.Results Five cases males and sixteen females were included in this study.Age from 5 years old to 15 and half years old were diagnosed and average ages were 10.3 years old.12 cases were found with abdominal pain(There were 5 cases with vomiting at the same time.One with jundica,light stool and dark urine.Two had acute abdominal pain)and 9 cases were found with abdominal masses.Imaging examinations(ultrasound n=21,CT n=20,and MRI n=9)all indicated cystic or/and solide lesion in pancreas.Diastase and leukocyte were slightly elevated only in 3 cases.Surgical intact excision was completed in 21 cases.Three cases of them were completed Whipple operation.One of them had drainage from cystic in pancreas and then was completed surgical excision.Position of tumor:the head of pancreas were 10 cases,the body of pancreas were 3,the body and tail of pancreas were 4,tail of pancreas were 4.Chemotherapy wasn′t given to all patients after pathological diagnosis.All cases were followed up to present.Only one was found local recurrence of tumor after 11 months of operation and surgery excision was operated again.Conclusions SPN is a rare and low grade tumor of exocrine pancreatic tumor.Clinical and imaging features are not characterised.There is a pseudopapillary pattern and solid area of microscopic appearance.While the tumor size is larger and infiltrates into the surrounding tissue and has obvious nuclear atypical and mitotic count and necrosis,indicates that has a malignant behavior of SPN.Complete resection tumor is the main therapeutic approach and important prognostic factor.

关 键 词：胰腺实性-假乳头状肿瘤 胰母细胞瘤 胰腺假囊肿 儿童 WHIPPLE手术 

分 类 号：R735.9[医药卫生—肿瘤]