目的:胃泌素瘤伴Zollinger-Ellison综合征(ZES)可偶尔发生(Sp)或作为多发性内分泌肿瘤1(MEN-1)的遗传综合征的一部分。比较Sp和MEN-1/ZES的数据很少。我们旨在识别和比较它们的临床特征。
方法:在1992年至2020年期间,在单中心意大利患者队列中评估了连续的ZES患者。
结果:在76例MEN-1患者中,41例胃肠胰腺神经内分泌肿瘤(GEP-NEN),其中18人患有ZES;在320Sp-GEP-NEN中,19有Sp-ZES。MEN-1/ZES患者较年轻(p=0.035),原发性MEN-1/ZES胃泌素瘤小于Sp-ZES(p=0.030)。两组均发生肝转移,但只有Sp-ZES发生肝外转移。13Sp-ZES和8MEN-1/ZES接受了手术。8个Sp-ZES和7个MEN-1/ZES接受生长抑素类似物(SSA)。MEN-1/ZES的中位总生存期(OS)高于Sp-ZES(310vs168个月,p=0.034)。在单变量逻辑回归中,诊断年龄(p=0.01,OR=1.1),G3分级(p=0.003,OR=21.3),Sp-ZES(p=0.02,OR=0.3)和肝外转移的存在(p=0.001,OR=7.2)与OS显着相关。在多变量COX分析中,没有一个变量与OS显著相关。在单变量逻辑回归中,年龄(p=0.04,OR=1.0),尺寸(p=0.039,OR=1.0),G3分级(p=0.008,OR=14.6)和肝外转移(p=0.005,OR=4.6)与无进展生存期(PFS)独立相关。在多变量COX分析中,只有肝外转移(p=0.05,OR=3.4)与PFS显著相关.在接受SSA治疗的患者中,MEN-1/ZES显示更好的PFS(p=0.0227)。手术后,MEN-1和Sp的中位PFS为126个月和96个月,分别。
结论:MEN-1/ZES患者通常表现出比Sp-ZES更好的OS和PFS以及更好的SSA反应。
OBJECTIVE: Gastrinoma with Zollinger-Ellison syndrome (ZES) may occur sporadically (Sp) or as part of the inherited syndrome of multiple endocrine neoplasia 1 (MEN-1). Data comparing Sp and MEN-1/ZES are scanty. We aimed to identify and compare their clinical features.
METHODS: Consecutive patients with ZES were evaluated between 1992 and 2020 among a monocentric Italian patient cohort.
RESULTS: Of 76 MEN-1 patients, 41 had gastroenteropancreatic neuroendocrine neoplasm (GEP-NEN), 18 of whom had ZES; of 320 Sp-GEP-NEN, 19 had Sp-ZES. MEN-1/ZES patients were younger (p = 0.035) and the primary MEN-1/ZES gastrinoma was smaller than Sp-ZES (p = 0.030). Liver metastases occurred in both groups, but only Sp-ZES developed extrahepatic metastases. 13 Sp-ZES and 8 MEN-1/ZES underwent surgery. 8 Sp-ZES and 7 MEN-1/ZES received somatostatin analogs (SSAs). Median overall survival (OS) was higher in MEN-1/ZES than in Sp-ZES (310 vs 168 months, p = 0.034). At univariate-logistic regression, age at diagnosis (p = 0.01, OR = 1.1), G3 grading (p = 0.003, OR = 21.3), Sp-ZES (p = 0.02, OR = 0.3) and presence of extrahepatic metastases (p = 0.001, OR = 7.2) showed a significant association with OS. At multivariate-COX-analysis, none of the variables resulted significantly related to OS. At univariate-logistic regression, age (p = 0.04, OR = 1.0), size (p = 0.039, OR = 1.0), G3 grade (p = 0.008, OR = 14.6) and extrahepatic metastases (p = 0.005, OR = 4.6) were independently associated with progression-free survival (PFS). In multivariate-COX-analysis, only extrahepatic metastases (p = 0.05, OR = 3.4) showed a significant association with PFS. Among SSAs-treated patients, MEN-1/ZES showed better PFS (p = 0.0227). After surgery, the median PFS was 126 and 96 months in MEN-1 and Sp, respectively.
CONCLUSIONS: MEN-1/ZES patients generally show better OS and PFS than Sp-ZES as well as better SSAs response.