中国实用外科杂志

中国实用外科杂志 ›› 2022, Vol. 42 ›› Issue (07): 790-793.DOI: 10.19538/j.cjps.issn1005-2208.2022.07.18

• 论著 • 上一篇    下一篇

胆囊原发神经内分泌肿瘤5例临床特征及诊治分析

杜    倩a，梁    霄a，王观宇a，沈    波a，吴峥嵘a，王翀韡b，曹利平a   

  1. 浙江大学医学院附属邵逸夫医院 a.肝胆胰外科， b.病理科，浙江杭州 310000 
  • 出版日期:2022-07-01 发布日期:2022-07-05

  • Online:2022-07-01 Published:2022-07-05

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。

关键词: 胆囊肿瘤, 神经内分泌肿瘤, 外科治疗, 病理分级, 预后

Abstract: Clinical features, diagnosis and treatment analysis of 5 cases of primary gallbladder neuroendocrine neoplasms        DU Qian*，LIANG Xiao，WANG Guan-yu，et al. *Department of Hepatobiliary & Pancreatic Surgery，Sir Run Run Shaw Hospital，School of Medicine，Zhejiang University，Hangzhou 310000，China
Corresponding author：CAO Li-ping，E-mail：caolipingzju@zju.edu.cn
Abstract    Objective    To explore the clinical features，pathology grading，diagnosis，treatment and prognosis of gallbladder neuroendocrine neoplasm（GB-NEN）. Methods    The clinicopathological features and follow-up data of 5 cases with GB-NEN admitted to Sir Run Run Shaw Hospital from January 2014 to May 2021 were analyzed retrospectively. Results    There 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 imaging examination had no specific features， and no distant metastasis was found in all cases. Based on the 2019 WHO classification，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 CgA，100%（5/5）expressed Syn and 100%（5/5）expressed CD56. 1 case of well-differentiated NET G1 underwent laparoscopic cholecystectomy(LC). The other 4 cases were treated with radical cholecystectomy including 4 cases of laparoscopy and 1 case of laparotomy. There were no postoperative complications in all cases. 2 patients with NEC and 1 patient with NET G3 received adjuvant chemotherapy after the operation.The patient with LCNEC was finding lung metastasis after 9 months of surgery，then she was treated with chemotherapy combined with immunosuppressive agents. The patient with MANEC was treated with TACE because of liver metastasis after 5.5 months of radical cholecystectomy. Follow-up period was 4.5 to78 months，all patients survived till the end of the follow-up. Conclusion    The incidence of primary GB-NENs is low. Clinical symptoms and imaging manifestations are non-specific. Final diagnosis relies on both pathology and immunohistochemistry findings．Surgical excision was considered as the main and only curative treatment.Aggressive multimodality therapy with adjuvant chemotherapy and biotherapy may improve the survival of GB-NENs.

Key words: gallbladder neoplasm；neuroendocrine neoplasm；surgical option；pathology grading；prognosis ,