Gu D Y, Fu Q, Xue B Y, Kan J B, Bai J A, Tang Q Y
Department of Geriatric Gastroenterology,the First Affiliated Hospital of Nanjing Medical University, Nanjing 210000, China.
Department of Endocrinology, the First Affiliated Hospital of Nanjing Medical University, Nanjing 210000, China.
Zhonghua Yi Xue Za Zhi. 2022 Apr 12;102(14):1014-1019. doi: 10.3760/cma.j.cn112137-20210822-01906.
To compare the clinical features of multiple endocrine adenoma type 1 (MEN-1) associated pancreatic neuroendocrine neoplasms (pNENs) as well as sporadic pNENs. 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. 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 <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 <0.05). In terms of tumor staging and metastasis, 21 patients with MEN-1-related pNENs had metastasis (70%) and 20 patients with stage Ⅰ and Ⅱ AJCC (71%) in all. Eight patients with sporadic pNENs had metastasis (26.7%) and 8 patients were with stage Ⅰ and Ⅱ AJCC (28.6%). By contrast, the differences in total metastasis rate, AJCC stage and distant metastasis between the two groups were statistically significant(all <0.05). In terms of treatment and prognosis, there was no statistical significance in the differences between surgical treatment and prognosis (>0.05), and the difference was also not statistically significant in survival rate between them (>0.05). 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.
比较1型多发性内分泌腺瘤（MEN-1）相关胰腺神经内分泌肿瘤（pNENs）与散发性pNENs的临床特征。收集2010年1月至2021年6月在南京医科大学第一附属医院收治的28例散发性pNENs患者和10例MEN-1相关pNENs患者的临床资料。同时，通过检索PubMed数据库并回顾同期报道的20例国外MEN-1相关pNENs患者的临床资料。比较分析MEN1相关pNENs与散发性pNENs在家族史、血液检查、病理诊断指标、肿瘤分级、分期及转移情况、治疗及预后等临床特征方面的异同。共纳入58例pNENs患者，其中MEN1相关pNENs患者30例，散发性pNENs患者28例。18例（60%）MEN1相关pNENs患者有家族史，平均发病年龄为（35.3±13.0）岁。散发性pNENs患者无家族史（0例），平均发病年龄为（55.3±13.4）岁。相比之下，家族史、发病年龄和神经元特异性烯醇化酶（NSE）的差异具有统计学意义（均<0.05）。在病理诊断指标方面，MEN1相关pNENs患者中19例（63.3%）为G2级，25例（83.3%）生长抑素受体2（SSTR2）阴性。散发性pNENs中，16例（57.1%）为G2级，9例（32.1%）SSTR2阴性。两组间病理分级、免疫组化（嗜铬粒蛋白A、CD56和生长抑素受体2，SSTR2）的差异具有统计学意义（均<0.05）。在肿瘤分期和转移方面，MEN-1相关pNENs患者中21例发生转移（70%），所有患者中20例为美国癌症联合委员会（AJCC）Ⅰ期和Ⅱ期（71%）。散发性pNENs患者中8例发生转移（26.7%），8例为AJCCⅠ期和Ⅱ期（28.6%）。相比之下，两组间总转移率、AJCC分期和远处转移的差异具有统计学意义（均<0.05）。在治疗和预后方面，手术治疗与预后的差异无统计学意义（>0.05），两者生存率的差异也无统计学意义（>0.05）。MEN1相关pNENs与散发性pNENs在治疗、预后和生存率方面无显著差异，但在临床特征、病理特征以及肿瘤的分期和分级方面存在显著差异。散发性pNENs的肿瘤分级、分期和转移率更高。
