摘要
目的分析散发型与多发性内分泌腺瘤病1型(MEN1)相关型胃泌素瘤的临床特征,为临床诊治提供帮助。方法采用回顾性队列研究方法。纳入有卓-艾综合征的临床表现且经病理确诊为神经内分泌肿瘤(NEN)的临床及随访资料完整的患者,排除仅有高胃酸分泌表现而无NEN证据者以及合并其他非NEN肿瘤者。根据上述标准,收集2003年4月至2020年12月期间,在中山大学附属第一医院就诊的52例胃泌素瘤患者的临床病理资料。符合胃泌素瘤诊断标准且满足以下条件之一的患者,被诊断为MEN1相关型胃泌素瘤:(1)经基因检测证实存在MEN1基因致病突变;(2)含有两个或两个以上内分泌腺体的NEN,即垂体、甲状旁腺、胸腺、胰腺以及肾上腺NEN;(3)发生NEN且至少一个一级亲属诊断为MEN1。其余胃泌素瘤定义为散发型胃泌素瘤。通过t检验和χ^(2)检验进行统计学分析,比较散发型与MEN1相关型胃泌素瘤的临床病理特征、内镜下表现、影像学特征、治疗及预后情况。结果 52例胃泌素瘤患者中,散发型33例,MEN1相关型19例,散发型和MEN1相关型的主要临床表现均为腹泻(24/33,72.7%;17/19)和腹痛(19/33,57.6%;9/19)。与散发型胃泌素瘤相比,MEN1相关型诊断推迟时间更长[(7.4±4.9)年比(3.9±5.2)年,t=-2.355,P=0.022];多发肿瘤比例更高[9/19比15.2%(5/33),χ^(2)=6.361,P=0.012];肿瘤最大径更短[(1.7±1.0)cm比(3.1±1.8)cm,t=2.942,P=0.005];肿瘤分级更低[G1:15/18比39.4%(13/33);G2:2/18比54.5%(18/33);G3:1/18比6.1%(2/33),Z=-2.766,P=0.006];血清胃泌素高于正常值10倍的比例更低[2/17比56.0%(14/25),χ^(2)=8.396,P=0.004];合并2型胃神经内分泌肿瘤(g-NET)的概率更高[6/19比3.0%(1/33),χ^(2)=6.163,P=0.013];出现肝转移比例更低[4/19比51.5%(17/33),χ^(2)=4.648,P=0.031]。散发型与MEN1相关型的胃泌素瘤在内镜下无明显区别,均为高胃酸刺激下胃黏膜粗大、红肿,可见胃、十二指肠多发溃疡。合并2型g-NET
Objective To compare clinical characteristics of sporadic gastrinoma and multiple endocrine neoplasia type 1(MEN1)-related gastrinoma.Methods A retrospective cohort study was conducted.Patients with clinical manifestations of Zollinger-Ellison syndrome,pathological diagnosis as neuroendocrine neoplasm(NEN)and complete clinical and follow-up data were enrolled.Patients with only high gastric acid secretion but without evidence of NEN,or with other concurrent non-NEN tumors were excluded.According to the above criteria,the clinicopathological data of 52 cases of gastrinoma diagnosed from April 2003 to December 2020 in the First Affiliated Hospital,Sun Yat-sen University,were collected.Patients who met the diagnostic criteria of gastrinoma and met one of the following conditions were diagnosed as MEN1-related gastrinoma:(1)the presence of pathogenic mutations in the MEN1 gene confirmed by genetic testing;(2)NENs involving two or more endocrine glands,namely,pituitary,parathyroid,thymic,pancreatic,and adrenal NENs;(3)NEN and at least one first-degree relatives diagnosed as MEN1.The remaining gastrinomas were defined as sporadic gastrinoma.Student's t test and chi-square test were used for statistical analysis.Clinicopathological characteristics,endoscopic findings,imaging characteristics,treatment,and prognosis of sporadic and MEN1-related gastrinoma were compared.Results Among 52 patients with gastrinoma,33 were sporadic gastrinoma and 19 were MEN1-related gastrinoma.The common symptoms of both sporadic and MEN1-related gastrinomas were diarrhea(24/33,72.7%;17/19,89.5%)and abdominal pain(19/33,57.6%;9/19,47.4%).Compared with sporadic gastrinoma,MEN1-related gastrinoma needed longer time for diagnosis[(7.4±4.9)years vs.(3.9±5.2)years,t=-2.355,P=0.022],were more likely multiple tumors[47.4%(9/19)vs.15.2%(5/33),χ^(2)=6.361,P=0.012],had smaller diameter[(1.7±1.0)cm vs.(3.1±1.8)cm,t=2.942,P=0.005],presented the lower tumor grade[G1:83.3%(15/18)vs.39.4%(13/33);G2:11.1%(2/18)vs.54.5%(18/33);G3:5.6%(1/18)vs.6.1%(2/33),
作者
郭宇
陈洛海
刘曼
张艺璇
张宁
陈洁
Guo Yu;Chen Luohai;Liu Man;Zhang Yixuan;Zhang Ning;Chen Jie(Department of Gastroenterology,the First Affiliated Hospital,Sun Yat-sen University,Guangzhou 510080,China;Center for Neuroendocrine Tumors,Fudan University Shanghai Cancer Center,Department of Oncology,Shanghai Medical College,Fudan University,Shanghai Pancreatic Cancer Institute,Pancreatic Cancer Institute,Fudan University,Shanghai 200032,China)
出处
《中华胃肠外科杂志》
CSCD
北大核心
2021年第10期875-882,共8页
Chinese Journal of Gastrointestinal Surgery
基金
广州市科技计划项目(201804010078)
广东省自然科学基金(2019A1515011373)。
