ArticleJournal of endocrinological investigation2023
Sporadic and MEN1-related gastrinoma and Zollinger-Ellison syndrome: differences in clinical characteristics and survival outcomes.
Article in Journal of endocrinological investigation, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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14 citing papers in PubMed, 16 citations in OpenAlex.
- Comprehensive Somatic Profiling of Gastroenteropancreatic Neuroendocrine Neoplasms.Endocrine oncology (Bristol, England) · 2026Article
- Distinct epigenetic aging in sporadic and hereditary neuroendocrine neoplasms.Clinical epigenetics · 2026Article
- Perspectives on gastric cancer: the central role of gastrin in carcinogenesis byFrontiers in oncology · 2026Review
- Severe Recurrent Gastrointestinal Bleeding and Diagnostic Dilemmas in Zollinger-Ellison Syndrome: A Case Report.GE Portuguese journal of gastroenterology · 2025Article
- Current Medical Controversies in Zollinger-Ellison Syndrome.Biomedicines · 2025Review
- Article
- From diagnosis to treatment: navigating the course for pancreatic neuroendocrine neoplasms.Annals of medicine · 2025Review
- Gastrinoma: A Case of Chronic Diarrhea.Cureus · 2025Article
- Progress report on multiple endocrine neoplasia type 1.Familial cancer · 2025Review
- Gastric Neuroendocrine Tumors and Pernicious Anemia: A Case Report and Literature Review.Cureus · 2024Article
- Review
- Article
- The association between jaundice and poorly differentiated pancreatic neuroendocrine neoplasms (Ki67 index > 55.0%).BMC gastroenterology · 2023Article
- Therapeutic Challenges for Gastric Neuroendocrine Neoplasms: Take It or Leave It?Medicina (Kaunas, Lithuania) · 2023Review
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Authors and funding
10 authors at 5 institutions in 1 country.
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Abstract
purposeGastrinoma 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.
methodsConsecutive patients with ZES were evaluated between 1992 and 2020 among a monocentric Italian patient cohort.
resultsOf 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.
conclusionMEN-1/ZES patients generally show better OS and PFS than Sp-ZES as well as better SSAs response.
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