胆囊原发神经内分泌肿瘤5例临床特征及诊治分析  

Clinical features, diagnosis and treatment analysis of 5 cases of primary gallbladder neuroendocrine neoplasms

作　　者：杜倩 梁霄[1] 王观宇[1] 沈波[1] 吴峥嵘[1] 王翀韡 曹利平[1] DU Qian;LIANG Xiao;WANG Guan-yu(Department of Hepatobiliary&Pancreatic Surgery,Sir Run Run Shaw Hospital,School of Medicine,Zhejiang University,Hangzhou 310000,China;不详)

机构地区：[1]浙江大学医学院附属邵逸夫医院肝胆胰外科,浙江杭州310000 [2]浙江大学医学院附属邵逸夫医院病理科,浙江杭州310000

出　　处：《中国实用外科杂志》2022年第7期790-793,799,共5页Chinese Journal of Practical Surgery

基　　金：国家自然科学基金(No.81971479)。

摘　　要：目的 分析胆囊原发性神经内分泌肿瘤(GB-NEN)的临床病理特征和诊疗方案。方法 回顾性分析2014年1月至2021年3月浙江大学医学院附属邵逸夫医院收治的5例确诊为GB-NEN病人的临床病理资料,随访观察临床疗效。结果 5例病人中3例出现右上腹隐痛,2例无临床表现。术前体格检查和实验室检查均无异常,未发现远处转移。术后病理学检查发现,5例均为GB-NEN,其中1例大细胞神经内分泌癌(LCNEC),1例小细胞神经内分泌癌(SCNEC),1例混合性腺神经内分泌癌(MANEC),另外2例胆囊神经内分泌瘤(GB-NET)分别为G1、G3级。免疫组化检查示,4例病人CgA阳性,5例病人SyA、CD56均为阳性。1例分化良好的GB-NET G1病人行腹腔镜胆囊切除术,余4例行胆囊癌根治术,其中3例为腹腔镜手术,1例为开放手术。5例均未出现术后并发症。GB-NET G3病人和SCNEC、LCNEC病人术后行辅助化疗。LCNEC病人术后9个月出现肺多发转移,更改化疗方案联合免疫抑制剂继续治疗。MANEC病人术后5.5个月出现肝转移,行经导管肝动脉栓塞(TACE)治疗。随访14.0(4.5~78.0)个月,无死亡病例。结论 GB-NEN极为罕见,临床特征及实验室检查缺乏特异性,术前诊断困难,确诊依赖常规病理及免疫组化检查,手术为首选治疗方法,联合化疗等多种模式综合治疗有助于改善病人生存。ObjectiveTo explore the clinical features,pathology grading,diagnosis,treatment and prognosis ofgallbladder neuroendocrine neoplasm(GB-NEN).MethodsThe clinicopathological features and follow-up data of 5cases with GB-NEN admitted to Sir Run Run Shaw Hospital from January 2014 to May 2021 were analyzedretrospectively.ResultsThere were 5 patients with GB-NEN,the median age of patients was 57 years(range 23-70),with an M:F ratio of 3:2. The major complaint was right upper quadrant pain. The laboratory examination and imagingexamination had no specific features,and no distant metastasis was found in all cases. Based on the 2019 WHOclassification,five cases were pathologically confirmed as GB-NEN and respectively graded in NET G1(1 case),NET G3(1 case),NEC(2 cases,Large cell/Small cell=1/1),MANEC(1 case). Immunohistochemistry findings showed that 80%(4/5)expressed Cg A,100%(5/5)expressed Syn and 100%(5/5)expressed CD56. 1 case of well-differentiated NET G1underwent laparoscopic cholecystectomy(LC). The other 4 cases were treated with radical cholecystectomy including 4cases of laparoscopy and 1 case of laparotomy. There were no postoperative complications in all cases. 2 patients withNEC and 1 patient with NET G3 received adjuvant chemotherapy after the operation.The patient with LCNEC wasfinding lung metastasis after 9 months of surgery,then she was treated with chemotherapy combined withimmunosuppressive agents. The patient with MANEC was treated with TACE because of liver metastasis after 5.5 monthsof radical cholecystectomy. Follow-up period was 4.5 to78 months,all patients survived till the end of the follow-up.ConclusionThe incidence of primary GB-NENs is low. Clinical symptoms and imaging manifestations are non-specific. Final diagnosis relies on both pathology andimmunohistochemistry findings. Surgical excisionwas considered as the main and only curativetreatment.Aggressive multimodality therapy withadjuvant chemotherapy and biotherapy may improve the survival of GB-NENs.

关 键 词：胆囊肿瘤 神经内分泌肿瘤 外科治疗 病理分级 预后 

分 类 号：R6[医药卫生—外科学]