多发性内分泌腺瘤1型相关型与散发型胰腺神经内分泌肿瘤临床特征比较

Comparison of clinical features between sporadic pancreatic neuroendocrine tumors and those associated with multiple endocrine neoplasia type 1

目的比较多发内分泌腺瘤1型(MEN-1)相关型胰腺神经内分泌肿瘤(pNENs)与散发型pNENs的临床特征。方法收集2010年1月至2021年6月南京医科大学第一附属医院收治的28例散发型pNENs患者及10例MEN-1相关型pNENs患者的临床资料,并通过检索PubMed数据库,文献复习同期报道国外的20例MEN-1相关型pNENs患者的临床资料。比较分析MEN-1相关型pNENs与散发型pNENs患者的临床特征如家族史、血液检测、病理诊断指标、肿瘤分级、分期及转移、治疗及预后等方面的异同。结果纳入的58例pNENs患者中,MEN-1相关型pNENs患者30例,散发型pNENs患者28例,其中,MEN-1相关型pNENs患者中有家族史的为18例(60%),发病年龄为(35.3±13.0)岁,散发型pNENs患者中有家族史的为0例,发病年龄为(55.3±13.4)岁。两组相比,家族史、发病年龄、神经元特异性烯醇化酶(NSE)差异有统计学意义(P<0.05);病理诊断指标中,MEN1相关型pNENs中G2级的患者有19例(63.3%),生长抑素受体2(SSTR2)阴性的患者有25例(83.3%),散发型pNENs中G2级的患者有16例(57.1%),SSTR2阴性的患者有9例(32.1%),两组相比,患者病理分级和免疫组化(嗜铬粒蛋白A、CD56、SSTR2)差异有统计学意义(P<0.05);肿瘤分期及转移方面,MEN-1相关型pNENs患者中转移的有21例(70%),AJCC分期中Ⅰ期和Ⅱ期共20例(71%),散发型pNENs中转移的有8例(26.7%),AJCC分期中Ⅰ期和Ⅱ期共8例(28.6%),两组相比,总转移率及AJCC分期、远处转移差异有统计学意义(P<0.05);在治疗方式及预后方面,手术治疗及预后差异无统计学意义(P>0.05),两者之间的生存率差异亦无统计学意义(P>0.05)。结论MEN-1相关型pNENs和散发型pNENs在治疗方式、预后及生存率方面无显著差异,但在临床特征、病理学特征、肿瘤分级分期方面存在显著差异,散发型pNENs肿瘤分级、分期和转移率更高。

Objective To compare the clinical features of multiple endocrine adenoma type 1(MEN-1)associated pancreatic neuroendocrine neoplasms(pNENs)as well as sporadic pNENs.Methods The clinical data of 28 sporadic pNENs patients and 10 MEN-1-related pNENs patients admitted to the First Affiliated Hospital of Nanjing Medical University from January 2010 to June 2021 were collected.Meanwhile,by searching PubMed database and reviewing the clinical data of 20 foreign patients with MEN-1-related pNENs which were reported at the same time.Compare and analyze the similarities and differences between MEN1-associated pNENs and sporadic pNENs in clinical features,such as family history,blood tests,pathological diagnostic indicators,tumor grade,stage and metastasis,treatment and prognosis and so on.Results A total of 58 pNENs patients were included,and there were 30 MEN1-related pNENs patients and 28 sporadic pNENs patients.Eighteen patients(60%)had a family history of MEN1-related pNENs,and the mean age of onset was(35.3±13.0)years.There were no patients(0)with family history of sporadic pNENs,and the mean age of onset was(55.3±13.4)years.In contrast,the differences in family history,age of onset and NSE were statistically significant(all P<0.05).Among the pathological diagnostic indicators,there were 19 patients(63.3%)with Grade G2 of MEN1-related pNENs,and 25 patients(83.3%)with somatostatin receptor 2(SSTR2)negative.In sporadic pNENs,there were 16 patients(57.1%)with Grade G2 and 9 patients(32.1%)with SSTR2 negative.The differences in pathological grade,immunohistochemistry(Chromogranin A,CD56,and somatostatin receptor 2,SSTR2)between the two groups were statistically significant(all P<0.05).In terms of tumor staging and metastasis,21 patients with MEN-1-related pNENs had metastasis(70%)and 20 patients with stageⅠandⅡAJCC(71%)in all.Eight patients with sporadic pNENs had metastasis(26.7%)and 8 patients were with stageⅠandⅡAJCC(28.6%).By contrast,the differences in total metastasis rate,AJCC stage and distant metastasis between the two groups were statistically significant(all P<0.05).In terms of treatment and prognosis,there was no statistical significance in the differences between surgical treatment and prognosis(P>0.05),and the difference was also not statistically significant in survival rate between them(P>0.05).Conclusions There are no significant differences between MEN1-related pNENs and sporadic pNENs in terms of treatment,prognosis,and survival rate,but there are significant differences in clinical features,pathological features and the staging and grading of tumors.The rate of tumor grade,stage and metastasis of sporadic pNENs is higher.