原发性肝胆系统神经内分泌肿瘤病例回顾及文献复习

摘要/Abstract

摘要： 目的：分析原发性肝胆神经内分泌肿瘤的临床特点,并进一步明确其诊断方法和有效的治疗策略。方法：对16例原发性肝胆神经内分泌肿瘤患者的临床病例资料进行回顾性的分析;结合文献综述,探讨原发性肝胆神经内分泌肿瘤的疾病临床特点及治疗选择。结果：所有患者甲胎蛋白（alpha fetoprotein, AFP）、癌胚抗原（carcinoembryonic antigen,CEA）均无明显升高。计算机断层扫描（computed tomography,CT）上动脉期明显增强,门脉期和延迟期减弱。在磁共振成像（magnetic resonance imaging,MRI）上,肿瘤在T1WI上表现为低信号,T2WI上表现为高信号,DWI上表现为局限性扩散,动脉期表现为明显的环状或不均匀增强,门脉期和延迟期表现为低信号。所有患者均经病理证实为原发性肝胆神经内分泌肿瘤。免疫组化结果显示所有肿瘤嗜铬粒蛋白A（chromograninA,CgA）均为阳性、11例突触素（synaptophysin, Syn）为阳性。随访3~96个月,14例无复发。无病生存期最长为96个月。患者在随访过程中,全部患者均未发现其他部位神经内分泌瘤（neuroendocrine tumor, NET）。结论：目前对于肝胆系统神经内分泌肿瘤患者,应根据患者的具体情况制定治疗方案,并充分完善相关检查,减少误诊的发生。对于可切除的病变,手术切除是首选。

关键词: 原发性肝胆系统神经内分泌肿瘤, 影像学, 治疗, 预后

Abstract: Objective: To investigate the clinical features, diagnosis and effective treatment strategies of primary hepatobiliary neuroendocrine tumor. Methods: The clinical data of 16 patients with primary hepatobiliary neuroendocrine tumor were analyzed retrospectively, and the clinical characteristics and treatment options of primary hepatobiliary neuroendocrine tumor were discussed combined with literature review. Results: There was no obvious increase in AFP and CEA in all patients. On CT, the arterial phase was markedly enhanced, while the portal phase and the delayed phase were weakened. On MRI, the tumors showed low signal on T1-weighted MRI, high signal on T2-weighted MRI, limited diffusion on DWI, and marked annular or heterogeneous enhancement in arterial phase, and decreased enhancement in portal phase and delayed phase. All patients were diagnosed as primary hepatobiliary neuroendocrine tumors by pathological results. The results of immunohistochemistry showed that chromogranin A (CgA) was positive in all tumors and synaptophysin (Syn) was positive in 11 cases. Follow up for 3 to 96 months showed no recurrence in 14 cases. The longest disease-free survival period was 96 months. No other neuroendocrine tumors were found in all patients. Conclusions: At present, for patients with neuroendocrine tumor of hepatobiliary system, the treatment plan should be formulated according to the specific situation of patients, and the relevant examination should be fully improved to reduce the occurrence of misdiagnosis. For resectable lesions, surgical resection is the first choice.

Key words: Primary hepatobiliary neuroendocrine tumors, Medical imageology, Treatment, Prognosis

葛大壮, 毕新宇, 赵宏, 李智宇, 张业繁, 罗治文, 魏哲文, 陈启晨, 李星辰, 蔡建强. 原发性肝胆系统神经内分泌肿瘤病例回顾及文献复习[J]. 肝癌电子杂志, 2020, 7(4): 23-28.

Ge Dazhuang, Bi Xinyu, Zhao Hong, Li Zhiyu, Zhang Yefan, Luo Zhiwen, Wei Zhewen, Chen Qichen, Li Xingchen, Cai Jianqiang. Primary hepatobiliary neuroendocrine tumors: a case review and literature review[J]. Electronic Journal of Liver Tumor, 2020, 7(4): 23-28.

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