原发性胆囊神经内分泌肿瘤临床病理特点分析

目的 探讨原发性胆囊神经内分泌肿瘤的诊断和治疗，提高对其的诊疗水平。方法 回顾性分析2013~2018年中国医学科学院肿瘤医院行手术切除病理学证实的5例原发性胆囊神经内分泌肿瘤患者的临床资料。结果 5例原发性胆囊神经内分泌肿瘤患者，其中男性2例，女性3例，患者年龄51~73岁，中位年龄56岁，其中病理分级G₂1例，G₃4例，肿瘤直径1.5~5.8cm，平均3.3cm。2例位于底部，2例位于体部，1例位于胆囊颈部。4例开腹行胆囊切除术+肝部分切除+肝门淋巴结清扫术，1例行腹腔镜辅助胆囊癌根治术。术后1、2、3年生存期分别为75%、45%、20%，中位生存期为22个月。结论 原发性胆囊神经内分泌肿瘤较罕见，手术是主要的治疗方法，术后辅助化疗、靶向治疗以及生长抑素治疗能提高术后疗效。

Clinicopathological Characteristics of Gallbladder Neuroendocrine Neoplasms

Objective To investigate the clinicopathological features and prognosis of gallbladder neuroendocrine neoplasms (GB-MEN),and to improve diagnosis and management of GB-MEN. Methods A retrospective clinical analysis of 5 rare primary GB-MEN cases diagnosed by postoperative pathological examination during October 2013 to September 2018 was performed. Results There were 5 cases with GB-MEN(2males, 3 females). The median age was 56 years (ranging 51 to 73). 1 case was classified G₂ by pathological grading, 4 cases were classified G₃.The median size of tumor was 3.3cm (ranging 1.5cm to 5.8cm). The lesions located in fundus of gallbladder in 2 cases,in bodies of gallbladder in 2 cases and in the neck in 1 case. 4 cases underwent cholecystotomy+hepatectomy+hilar lymph node dissection. 1 case underwent operation by laparoscopy assistance. The 1-, 2-, 3-year cumulative survival rates for this group were 75%, 45%, 20% respectively. The median survival time was 22 months. Conclusion Primary gallbladder neuroendocrine neoplasm is a rare malignancy pathological subtype of gallbladder neoplasm. Surgical resection is main treatment. The adjuvant systematic chemotherapy, targeted medicine therapy and Somatostatin treatment will improve the prognosis.