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Síndrome Zollinger-Ellison
ORPHA:913CID-10 · E16.4CID-11 · 5A43.1DOENÇA RARA

A Síndrome de Zollinger-Ellison (SZE) é uma doença que causa úlceras graves no estômago e esôfago. Ela ocorre porque um tumor, chamado gastrinoma, libera muita gastrina no sangue. Esse excesso de gastrina faz com que o estômago produza uma quantidade elevada de ácido.

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Introdução

O que você precisa saber de cara

📋

A Síndrome de Zollinger-Ellison (SZE) é uma doença que causa úlceras graves no estômago e esôfago. Ela ocorre porque um tumor, chamado gastrinoma, libera muita gastrina no sangue. Esse excesso de gastrina faz com que o estômago produza uma quantidade elevada de ácido.

Publicações científicas
2.326 artigos
Último publicado: 2026 Mar 20

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 100 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Europe
Início
Adult
+ elderly
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: E16.4
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Entender a doença

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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

📏
Crescimento
11 sintomas
🫃
Digestivo
7 sintomas
😀
Face
2 sintomas
🧠
Neurológico
1 sintomas
🫘
Rins
1 sintomas

+ 12 sintomas em outras categorias

Características mais comuns

100%prev.
Síndrome de Zollinger-Ellison
100%prev.
Neoplasia neuroendócrina
90%prev.
Úlcera péptica
Muito frequente (99-80%)
90%prev.
Diarreia
Muito frequente (99-80%)
90%prev.
Náusea
Muito frequente (99-80%)
90%prev.
Úlcera duodenal
Muito frequente (99-80%)
34sintomas
Muito frequente (8)
Frequente (1)
Ocasional (24)
Muito raro (1)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 34 características clínicas mais associadas, ordenadas por frequência.

Síndrome de Zollinger-EllisonZollinger-Ellison syndrome
Muito frequente100%
Neoplasia neuroendócrinaNeuroendocrine neoplasm
Muito frequente100%
Úlcera pépticaPeptic ulcer
Muito frequente (99-80%)90%
DiarreiaDiarrhea
Muito frequente (99-80%)90%
NáuseaNausea
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico2.326PubMed
Últimos 10 anos200publicações
Pico202326 papers
Linha do tempo
2026Hoje · 2026🧪 1978Primeiro ensaio clínico📈 2023Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado1
3Fase 32
2Fase 24
1Fase 11
·Pré-clínico4
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 12 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Síndrome Zollinger-Ellison

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Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

50 ensaios clínicos encontrados.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

📖Melhor nível de evidência: Revisão
Timeline de publicações
197 papers (10 anos)
#1

Balancing therapeutic benefits and hidden risks of proton pump inhibitors in pediatric practice: a narrative review and update.

Clinical and experimental pediatrics2026 Mar

Proton pump inhibitors (PPIs) are drugs whose main effect is catalytic and long-lasting suppression of gastric acid secretion, with an anti-inflammatory effect. The main indications for PPIs use include the treatment of gastroesophageal reflux disease, peptic ulcer disease, functional dyspepsia, Barrett esophagus, eosinophilic esophagitis, and hypersecretory diseases such as gastrinoma and Zollinger-Ellison syndrome. Their contribution to eradication therapy for Helicobacter pylori is also important. However, the long-term use of PPIs has been associated with various adverse drug reactions and complications, such as the development of fundic gland polyposis, atrophic gastritis, gastric cancer, vitamin B12 deficiency, hypomagnesemia, osteoporosis/ fractures, changes in the microbiome, increased risk of infection, necrotizing enterocolitis, renal injury, and other conditions. Thus, administration of PPIs should be based on scientific evidence of their efficacy and safety. Their long-term administration should be cautious, and continuation reassessed frequently. Caution should be exercised when administering PPIs to neonates and infants. This narrative review and update aims to summarize and critically evaluate the current evidence, based on the most recent clinical guidelines, regarding the use of PPIs in children, with a focus on their indications, efficacy, safety, and limitations.

#2

Esophageal Perforation in Zollinger-Ellison Syndrome: A Scoping Review of Management and Outcomes.

JGH open : an open access journal of gastroenterology and hepatology2026 Jan

Esophageal perforation in Zollinger-Ellison syndrome (ZES) is an exceedingly rare and life-threatening manifestation of gastrinoma-related acid hypersecretion. While peptic ulceration and severe reflux are recognized complications of ZES, perforation represents the ultimate consequence of uncontrolled hyperacidity. Evidence remains confined to isolated case reports with no prior systematic synthesis. A scoping review was conducted in accordance with PRISMA-ScR and the Joanna Briggs Institute framework. A comprehensive search of PubMed, Embase, Scopus, and Web of Science was performed from database inception to September 2025. Eligible studies included any report describing esophageal perforation in confirmed or suspected ZES. Iatrogenic perforations were excluded. Data were extracted on patient demographics, clinical presentation, diagnostic modalities, management strategies, and outcomes. Seven eligible cases published between 2001 and 2025 were identified. Patients were aged 44-63 years (4 males, 3 females). The distal esophagus was affected in approximately 70% of cases, usually after chronic peptic injury; three patients (≈43%) presented with spontaneous Boerhaave-type rupture. Computed tomography was diagnostic in all cases, and endoscopy was used in six. Surgical repair with mediastinal drainage or T-tube repair was the mainstay of management, while conservative therapy failed. Endoscopic stenting achieved successful leak control in one patient, whereas overall survival across all cases was approximately 86%, with one death due to delayed diagnosis and sepsis. Esophageal perforation in ZES represents a predictable but preventable endpoint of chronic acid injury. Rapid imaging, decisive surgical or endoscopic repair, and definitive acid suppression are essential to survival. Awareness of this rare complication among surgeons and gastroenterologists may facilitate early recognition and improve outcomes.

#3

Surgical Management of Zollinger-Ellison Syndrome in Multiple Endocrine Neoplasia Type 1 an AFCE and GTE Cohort Study. (Association Francophone de Chirurgie Endocrinienne and Groupe d'étude des Tumeurs Endocrines).

World journal of surgery2026 Mar 20

To describe surgical indications, procedures and outcomes in patients operated for Zollinger-Ellison syndrome (ZES) in multiple endocrine neoplasia type 1 (MEN1) using a large nationwide cohort. Management of ZES in MEN1 remains controversial. All patients with ZES diagnosed through the MEN1 AFCE/GTE network from 1985 to 2015. Among 233 ZES patients, 66 (28%) were operated for ZES-related gastrinomas. Thirty-three (51%) procedures aimed to remove gastrinomas and associated pancreatic neuroendocrine tumors (pNET(s)) with appropriate resection. Thirty-two procedures (49%) aimed to remove gastrinomas alone (ZES group). Survival was decreased in patients metastatic at ZES diagnosis (p < 0.001). Fifteen-year survival among non-metastatic patients was not significantly better in operated patients (82% vs. 70%, p = 0.2). Perioperative mortality was nil. Metastatic lymph nodes were found in 30/42 lymphadenectomies (71%). The choice between pancreaticoduodenectomy versus duodenal focused surgery in the ZES group was associated with pre-operative detection of adenopathies (p > 0.001), leading to more frequent lymphadenectomies (p < 0.01). Previous pancreatic surgeries (30%) may have influenced the choice of ZES procedures. Gastrin levels were more frequently normalized when the duodenum and the head of pancreas were removed versus more localized duodenal surgeries (p < 0.01). The high rate of invaded nodes in lymphadenectomies in MEN1 patients operated for ZES, the absence of operative mortality, and the decreased survival in metastatic patients are indirect arguments for surgery. Pancreaticoduodenectomy may be indicated in young and fit individuals to better control hypergastrinemia and to prevent metastatic progression in the ZES group. Gastrinoma removal is justified when associated with large pNETs.

#4

Rendezvous-assisted endoscopic retrograde pancreatography using a dual-wire balloon technique for stenotic pancreaticojejunostomy in post-Whipple anatomy.

VideoGIE : an official video journal of the American Society for Gastrointestinal Endoscopy2026 Feb

Pancreatic duct (PD) stones and a stenotic pancreaticojejunal anastomosis (PJA) can make ERCP highly challenging, especially in post-Whipple anatomy. EUS-assisted rendezvous (RV) may serve as a salvage approach, but severe stenoses can prevent guidewire passage. We describe a novel dual-wire balloon technique to achieve PD drainage in this setting. A 67-year-old man with Zollinger-Ellison syndrome and previous Whipple surgery presented with recurrent pancreatitis. Imaging revealed PD stones impacted at the PJA. Multiple enteroscopy-assisted ERCPs and an initial EUS-assisted RV failed. On repeat EUS-assisted RV, a 22-gauge needle was used to puncture the PD, and a 0.018-inch guidewire was advanced antegrade into the jejunum. Standard devices could not achieve dual-wire access. A therapeutic gastroscope was introduced, and a 5.5F balloon catheter was used to dilate the anastomosis. Removing the wire stiffener allowed a second 0.021-inch wire to be placed alongside the first. This approach enabled retrograde cannulation, deep wire access beyond the rendezvous wire through a stenotic PJA precluding larger dual-wire devices, and stent placement. The patient was discharged without adverse events, with stone extraction planned at follow-up. This case demonstrates the utility of a dual-wire balloon technique for EUS-guided PD drainage in severe PJA stenosis.

#5

Microwave ablation as a therapeutic approach in Zollinger-Ellison syndrome.

Gastroenterologia y hepatologia2026 Jan 21

Publicações recentes

Ver todas no PubMed

📚 EuropePMC1.377 artigos no totalmostrando 198

2026

Surgical Management of Zollinger-Ellison Syndrome in Multiple Endocrine Neoplasia Type 1 an AFCE and GTE Cohort Study. (Association Francophone de Chirurgie Endocrinienne and Groupe d'étude des Tumeurs Endocrines).

World journal of surgery
2026

Balancing therapeutic benefits and hidden risks of proton pump inhibitors in pediatric practice: a narrative review and update.

Clinical and experimental pediatrics
2026

Rendezvous-assisted endoscopic retrograde pancreatography using a dual-wire balloon technique for stenotic pancreaticojejunostomy in post-Whipple anatomy.

VideoGIE : an official video journal of the American Society for Gastrointestinal Endoscopy
2025

Severe Recurrent Gastrointestinal Bleeding and Diagnostic Dilemmas in Zollinger-Ellison Syndrome: A Case Report.

GE Portuguese journal of gastroenterology
2026

Esophageal Perforation in Zollinger-Ellison Syndrome: A Scoping Review of Management and Outcomes.

JGH open : an open access journal of gastroenterology and hepatology
2026

Microwave ablation as a therapeutic approach in Zollinger-Ellison syndrome.

Gastroenterologia y hepatologia
2025

Current Medical Controversies in Zollinger-Ellison Syndrome.

Biomedicines
2025

Clinicopathological characteristics and prognosis analysis of gastrinoma based on the SEER database.

PloS one
2026

Lessons for the surgeon from the early history of the Zollinger Ellison syndrome: The 6th Alexander Walt Historical Lecture Presented at the Midwest Surgical Association 68th Annual Meeting Delavan, Wisconsin July 27, 2025.

American journal of surgery
2025

Robotic-assisted total gastrectomy for refractory hypoproteinemia in Menetrier's disease: a case report with operative video and literature review.

Frontiers in medicine
2025

Gastrointestinal surveillance in patients with multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome.

Scandinavian journal of gastroenterology
2025

Endoscopic ultrasound-guided lauromacrogol embolization for suspected Zollinger-Ellison syndrome: a novel approach.

Endoscopy
2025

Zollinger-Ellison Syndrome Unmasked during Hiatal Hernia Evaluation: A Case Report.

Case reports in gastroenterology
2025

Etiology and Therapeutic Strategies for Refractory Peptic Ulcers: A Comprehensive Review.

The Korean journal of helicobacter and upper gastrointestinal research
2025

Diarrhea, Weight Loss, and an Elevated Gastrin: A Case Report.

Case reports in gastroenterology
2025

Last decade of advances in gastric neuroendocrine tumors: Innovations, challenges, and future directions.

World journal of clinical oncology
2025

Case Report: PRRT in a patient with Zollinger-Ellison syndrome. The management of gastrointestinal complications.

Frontiers in oncology
2025

Gastrinoma: A Case of Chronic Diarrhea.

Cureus
2025

A Challenging Case of Biliary Obstruction in an 82-Year-Old Male With a History of Zollinger-Ellison Syndrome and Suspected Cholangiocarcinoma Secondary to Biliary Candidiasis.

Cureus
2024

[A Case of Type Ⅲ Gastric Neuroendocrine Tumor and Gastric Cancer Coexisting in the Same Lesion].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2024

First reported case of multiple endocrine neoplasia type 1 in an Australian Aboriginal.

Endocrinology, diabetes &amp; metabolism case reports
2024

Long-Term Proton Pump Inhibitor-Acid Suppressive Treatment Can Cause Vitamin B12 Deficiency in Zollinger-Ellison Syndrome (ZES) Patients.

International journal of molecular sciences
2024

Presentation, Diagnosis, and Treatment of a Sporadic Gastrinoma With Liver Metastases.

Cureus
2025

Natural language processing assisted detection of inappropriate proton pump inhibitor use in adult hospitalised patients.

European journal of hospital pharmacy : science and practice
2024

Middle-segment preserving pancreatectomy: a literature review and case report.

Langenbeck's archives of surgery
2024

Choledochoduodenostomy combined with Billroth II procedure for extrahepatic biliary obstruction and duodenal perforation in a cat.

JFMS open reports
2024

Gastric neuroendocrine neoplasms.

Nature reviews. Disease primers
2024

Severe Hypomagnesemia Caused by Proton-Pump Inhibitors in a Patient With an Ostomy.

Cureus
2024

Recurrent Duodenal Ulceration-More Than Simple Peptic Ulcer Disease?

Gastroenterology
2024

Sporadic gastrinoma with refractory benign esophageal stricture: A case report.

World journal of clinical cases
2024

Sporadic Zollinger-Ellison syndrome in a patient with isolated mesenteric gastrinoma.

International journal of surgery case reports
2023

Duodenal gastrinoma - case report.

Rozhledy v chirurgii : mesicnik Ceskoslovenske chirurgicke spolecnosti
2023

Primary gastrinoma of the gallbladder: a case report and review of the literature.

Frontiers in oncology
2024

The cckOMA syndrome and its relation to the Zollinger-Ellison syndrome: a diagnostic challenge.

Scandinavian journal of gastroenterology
2023

Chronic Use of Proton Pump Inhibitors: A Potential Link to Amino Acid Deficiency and the Development of Depression.

Cureus
2024

PBPK modeling to predict the pharmacokinetics of pantoprazole in different CYP2C19 genotypes.

Archives of pharmacal research
2023

Severe esophageal stricture after perforation and necrotizing esophagitis: unusual presentation of a duodenal gastrinoma.

Journal of surgical case reports
2024

Management of Gastric Neuroendocrine Tumors: A Review.

Annals of surgical oncology
2023

Complete Esophageal Obstruction: A Rare Complication of Zollinger-Ellison Syndrome.

ACG case reports journal
2023

Esophageal Perforation Unveiling the Diagnosis of Zollinger-Ellison Syndrome.

Journal of community hospital internal medicine perspectives
2023

Multiple endocrine neoplasia type 1 with Zollinger-Ellison syndrome: clinicopathological analysis of a Japanese family with focus on menin immunohistochemistry.

Frontiers in endocrinology
2023

AGA Clinical Practice Update on the Epidemiology, Evaluation, and Management of Exocrine Pancreatic Insufficiency: Expert Review.

Gastroenterology
2023

Typical Zollinger-Ellison syndrome-atypical location of gastrinoma and absence of hypergastrinemia: A case report and review of literature.

World journal of clinical cases
2023

A Rare Case of Hepatic Gastrinoma.

Cureus
2023

ACTH-secreting pancreatic neuroendocrine neoplasms: A case-series.

Journal of neuroendocrinology
2023

Over 30 Years of Omeprazole.

The Journal of the Association of Physicians of India
2024

Multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome.

Revista espanola de enfermedades digestivas
2023

[Prognostic and surgical technique factors for recurrence after resection of a sporadic gastrinoma].

Chirurgie (Heidelberg, Germany)
2023

Adverse Effects Associated with Long-Term Use of Proton Pump Inhibitors.

Chonnam medical journal
2023

Clinical, endoscopic, and demographic characteristics of idiopathic duodenal ulcers compared with helicobacter pylori positive ulcers.

Caspian journal of internal medicine
2023

Proton pump inhibitor-induced gut dysbiosis and immunomodulation: current knowledge and potential restoration by probiotics.

Pharmacological reports : PR
2023

Gastrinomas and non-functioning pancreatic endocrine tumors in multiple endocrine neoplasia syndrome type-1 (MEN-1).

Endocrine
2023

Closed-Loop Gastric Outlet Obstruction Secondary to Duodenal Ulcer in a Patient With Esophageal Stricture.

Cureus
2023

All you need to know about gastrinoma today | Gastrinoma and Zollinger-Ellison syndrome: A thorough update.

Journal of neuroendocrinology
2023

Insulinoma and subsequent Zollinger-Ellison syndrome as an expression of phenotype exchange in a pancreatic neuroendocrine tumor.

Medicina clinica
2023

Successful Lifetime/Long-Term Medical Treatment of Acid Hypersecretion in Zollinger-Ellison Syndrome (ZES): Myth or Fact? Insights from an Analysis of Results of NIH Long-Term Prospective Studies of ZES.

Cancers
2023

Recurrence after surgical resection of nonmetastatic sporadic gastrinoma: Which prognostic factors and surgical procedure?

Surgery
2023

Ga-68 DOTATATE PET/CT in a patient with Zollinger-Ellison syndrome.

Radiology case reports
2022

A survey on the incidence of common musculoskeletal side effects among the patients taking long-term anti-ulcerant therapies in Bangladesh.

Toxicology reports
2022

[Primary lymph node gastrinoma].

Revista de gastroenterologia del Peru : organo oficial de la Sociedad de Gastroenterologia del Peru
2023

[Gastroenteropancreatic neuroendocrine tumors : (GEP-NENs)].

Wiener medizinische Wochenschrift (1946)
2023

How to treat gastrinomas in patients with multiple endocrine neoplasia type1: surgery or long-term proton pump inhibitors?

Surgery today
2023

Sporadic and MEN1-related gastrinoma and Zollinger-Ellison syndrome: differences in clinical characteristics and survival outcomes.

Journal of endocrinological investigation
2022

The Zollinger-Ellison Syndrome.

The New England journal of medicine
2022

Radiological Features of Zollinger-Ellison Syndrome: A Report of Two Cases.

The Indian journal of radiology &amp; imaging
2022

Multiple endocrine neoplasia type 1 with suspicion of Zollinger Ellison syndrome in a family with history of renal stones and hypercalcemia in a limited resource setting: a case report.

Oxford medical case reports
2022

Sporadic, non-functional, gastrin-producing duodenal neuroendocrine tumors: A retrospective study of an infrequent disease.

Journal of digestive diseases
2022

Metastatic Grade 3 Neuroendocrine Tumor in Multiple Endocrine Neoplasia Type 1 Expressing Somatostatin Receptors.

Journal of the Endocrine Society
2022

Neurofibromatosis Type 1: A Rare Predisposition for Gastrinomas and Other Neuroendocrine Tumors.

Pancreas
2022

Zollinger Ellison Syndrome Refractory to Medical Therapy in the Setting of Multiple Endocrine Neoplasia Type I.

Cureus
2022

Conjunctival myxoma masquerading as conjunctival lymphoma: A case report.

International journal of surgery case reports
2022

Identification of Helicobacter pylori-related gastric cancer risk using serological gastritis markers and endoscopic findings: a large-scale retrospective cohort study.

BMC gastroenterology
2022

Double Peptic Ulcer Perforation due to Cumulative Effects of Post-surgery Stress and NSAIDs: A Rare Event in Surgical Practice.

Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
2022

Pancreaticoduodenectomy Is the Best Surgical Procedure for Zollinger-Ellison Syndrome Associated with Multiple Endocrine Neoplasia Type 1.

Cancers
2022

Gastric neuroendocrine neoplasms: a primer for radiologists.

Abdominal radiology (New York)
2022

Primary Hepatic Neuroendocrine Tumor: A Case Report and Literature Review.

Cureus
2022

Gastroesophageal Reflux Disease and the Zollinger-Ellison Syndrome.

The American journal of gastroenterology
2022

Vitamin B12 absorption and malabsorption.

Vitamins and hormones
2022

GIST. Duodenopancreatectomía. Tumor neuroendocrino no funcionante.

Revista espanola de enfermedades digestivas
2022

Conjunctival myxoid stromal tumor of the palpebral conjunctiva: A case report.

American journal of ophthalmology case reports
2022

A case of enterochromaffin-like cell neuroendocrine tumor associated with parietal cell dysfunction which was successfully treated with somatostatin analogue.

Clinical journal of gastroenterology
2022

Secretin Stimulation Test and Early Diagnosis of Gastrinoma in MEN1 Syndrome: Survey on the MEN1 Florentine Database.

The Journal of clinical endocrinology and metabolism
2021

Primary thoracic gastrinoma causing Zollinger-Ellison syndrome.

Indian journal of thoracic and cardiovascular surgery
2022

Somatostatin analogs in patients with Zollinger Ellison syndrome (ZES): an observational study.

Endocrine
2021

[Comparison of clinical characteristics between sporadic gastrinoma and multiple endocrine neoplasia type 1-related gastrinoma].

Zhonghua wei chang wai ke za zhi = Chinese journal of gastrointestinal surgery
2021

Gastrinoma and Zollinger Ellison syndrome: A roadmap for the management between new and old therapies.

World journal of gastroenterology
2021

Validity of Secretin Stimulation Testing on Proton Pump Inhibitor Therapy for Diagnosis of Zollinger-Ellison Syndrome.

The American journal of gastroenterology
2021

Benign Oesophageal Stricture and Chronic Diarrhoea As Atypical Presenting Symptoms of an Advanced Metastatic Pancreatic Gastrinoma: A Case Report and Review of Literature.

Cureus
2021

Successful endoscopic resection of an unusually enlarged and pedunculated type I gastric carcinoid tumour.

BMJ case reports
2021

Hypertrophic Gastric folds with Hypomagnesemia, linking the dots.

Pakistan journal of medical sciences
2022

Enterochromaffin-like Cell Hyperplasia-Associated Gastric Neuroendocrine Tumors May Arise in the Setting of Proton Pump Inhibitor Use.

Archives of pathology &amp; laboratory medicine
2021

Postsurgical Presentation of Zollinger-Ellison Syndrome After Resection of Endometrioid Neuroendocrine Tumor.

ACG case reports journal
2021

Pancreatoduodenectomy for Neuroendocrine Tumors in Patients with Multiple Endocrine Neoplasia Type 1: An AFCE (Association Francophone de Chirurgie Endocrinienne) and GTE (Groupe d'étude des Tumeurs Endocrines) Study.

World journal of surgery
2020

Somatostatin receptor molecular imaging in a misdiagnosed gastrinoma case.

World journal of nuclear medicine
2021

Adverse Effects Associated with Proton Pump Inhibitor Use.

Cureus
2021

Primary hyperparathyroidism and Zollinger Ellison syndrome during pregnancy: a case report.

Endocrinology, diabetes &amp; metabolism case reports
2021

Metastatic Gastrinoma Localized on Gallium-68 DOTATATE Imaging.

Mayo Clinic proceedings
2021

Gastric enterochromaffin-like cell changes in multiple endocrine neoplasia type 1.

Clinical endocrinology
2021

Acute oesophageal necrosis in multiple endocrine neoplasia type 1: an undescribed complication.

BMJ case reports
2020

A case report of diffuse hyperplastic gastropathy with multiple polypoid formations in a patient with pernicious anemia, Helicobacter pylori infection, hypergastrinemia and hypoalbuminaemia: Do not forget of Ménétrier's disease.

International journal of surgery case reports
2021

Total gastrectomy for severe proton pump inhibitor-induced hypomagnesemia in a MEN1/Zollinger Ellison syndrome patient.

Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.]
2020

Primary hepatic gastrinoma being diagnosed preoperatively: a case report and literature review.

Surgical case reports
2022

Ulcerative jejunitis secondary to Zollinger-Ellison syndrome following endoscopic ultrasound guided gastrojejunostomy.

Gastroenterologia y hepatologia
2021

Perspectives on the current pharmacotherapeutic strategies for management of functional neuroendocrine tumor syndromes.

Expert opinion on pharmacotherapy
2020

Duodenal perforation as presentation of gastric neuroendocrine tumour: A case report.

International journal of surgery case reports
2020

Multiple Endocrine Neoplasia: Spectrum of Abdominal Manifestations.

AJR. American journal of roentgenology
2020

Gastric neuroendocrine tumours from long-term proton pump inhibitor users are indolent tumours with good prognosis.

Histopathology
2020

Primary intracardiac gastrinoma causing Zollinger-Ellison syndrome.

European heart journal
2020

Delayed Management of Zollinger-Ellison Syndrome in a Noncompliant Patient.

Cureus
2020

De novo Gastrinoma: A Case Report.

GE Portuguese journal of gastroenterology
2020

The Neuroendocrine Neoplasms of the Digestive Tract: Diagnosis, Treatment and Nutrition.

Nutrients
2020

Cytopathological evaluation of potential malignancy of duodenal gastrinoma using aspiration smears from two patients' resected tumors (NET G1, NET G2): A case report.

Molecular and clinical oncology
2020

Management of Other Gastric and Duodenal Neuroendocrine Tumors.

Surgical oncology clinics of North America
2020

Jejunal Perforations in Zollinger-Ellison Syndrome: Expect the Unexpected.

The American surgeon
2020

Manifestation of Zollinger-Ellison Syndrome With Hypovolemic Shock.

The American journal of medicine
2019

Proton pump inhibitors from clinical pharmacists point of view.

Casopis lekaru ceskych
2020

Proton Pump Inhibitor Use, Hypergastrinemia, and Gastric Carcinoids-What Is the Relationship?

International journal of molecular sciences
2019

Atypical Thymic Carcinoid in a Patient with Zollinger-Ellison Syndrome.

The Korean journal of thoracic and cardiovascular surgery
2019

[Gastric neuroendocrine tumors].

Khirurgiia
2019

Roar Strøm - the Norwegian surgeon who was three years ahead of Zollinger and Ellison.

Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke
2019

Zollinger-Ellison syndrome.

CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne
2019

Case report: optimal tumor cytoreduction and octreotide with durable disease control in a patient with MEN-1 and Zollinger-Ellison syndrome-over a decade of follow-up.

World journal of surgical oncology
2020

Diagnostic accuracy of selective arterial calcium injection test for localization of gastrinoma.

Endocrine journal
2019

The Pancreatic Endocrine Tumors - Experience of First Surgical Clinic Iasi.

Chirurgia (Bucharest, Romania : 1990)
2019

Insights into Effects/Risks of Chronic Hypergastrinemia and Lifelong PPI Treatment in Man Based on Studies of Patients with Zollinger-Ellison Syndrome.

International journal of molecular sciences
2019

Occurrence of Acute Oesophageal Necrosis (Black Oesophagus) in a Single Tertiary Centre.

Journal of clinical medicine
2019

Surgical management of Zollinger-Ellison syndrome: Classical considerations and current controversies.

World journal of gastroenterology
2019

Contemporary surgical management of the Zollinger-Ellison syndrome in multiple endocrine neoplasia type 1.

Best practice &amp; research. Clinical endocrinology &amp; metabolism
2019

Low-Value Proton Pump Inhibitor Prescriptions Among Older Adults at a Large Academic Health System.

Journal of the American Geriatrics Society
2019

Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG).

Journal of surgical oncology
2019

Zollinger Ellison Syndrome in a Patient with Multiple Endocrine Neoplasia Type 1: A Classic Presentation.

Case reports in gastrointestinal medicine
2019

The Gastrinoma Saga Before Zollinger and Ellison: The Strøm Case Revisited.

Annals of surgery
2019

Classical features of Zollinger-Ellison syndrome, in images.

Gastrointestinal endoscopy
2018

A Young Man With Abdominal Pain, Shock, and Respiratory Distress.

Chest
2019

Management of Primary Lymph Nodal Gastrinoma With Liver Metastases Resulting in Zollinger-Ellison Syndrome.

Clinical nuclear medicine
2018

Gastrinoma of Cystic Duct: A Rare Association With Multiple Endocrine Neoplasia Type 1.

Journal of clinical medicine research
2018

Proton pump inhibitors: use and misuse in the clinical setting.

Expert review of clinical pharmacology
2019

Results of Duodenopancreatic Reoperations in Multiple Endocrine Neoplasia Type 1.

World journal of surgery
2018

Multiple Endocrine Neoplasia Type 1: A Case Report With Review of Imaging Findings.

Ochsner journal
2018

Gastrinomas: Medical or Surgical Treatment.

Endocrinology and metabolism clinics of North America
2018

Large primary hepatic gastrinoma in young patient treated with trisegmentectomy: A case report and review of the literature.

World journal of hepatology
2018

Bringing SASI back: Single session selective arterial secretin injection and transarterial embolization of intrahepatic pancreatic neuroendocrine metastasis in a MEN-1 patient.

Radiology case reports
2018

Gastrinoma and Zollinger-Ellison syndrome in canids: a literature review and a case in a Mexican gray wolf.

Journal of veterinary diagnostic investigation : official publication of the American Association of Veterinary Laboratory Diagnosticians, Inc
2018

The appropriate use of proton-pump inhibitors.

Minerva medica
2018

Old and New Gut Hormone, Gastrin and Acid Suppressive Therapy.

Digestion
2018

Prospective Evaluation of Results of Reoperation in Zollinger-Ellison Syndrome.

Annals of surgery
2018

A Rare Cause of Abdominal Pain and Mass in an 18-Year-Old Patient: A Diagnostic Dilemma.

Gastroenterology research
2018

Introduction and practical approach to exocrine pancreatic insufficiency for the practicing clinician.

International journal of clinical practice
2018

Incidence and Prognosis of Primary Gastrinomas in the Hepatobiliary Tract.

JAMA surgery
2017

Diagnosis of Zollinger-Ellison syndrome in the era of PPIs, faulty gastrin assays, sensitive imaging and limited access to acid secretory testing.

International journal of endocrine oncology
2018

[Functional diagnostics in endocrinology].

Der Internist
2017

Surgical Therapy of Sporadic Pancreatic Neuroendocrine Neoplasias G1/G2.

Visceral medicine
2018

The Zollinger-Ellison syndrome: is there a role for somatostatin analogues in the treatment of the gastrinoma?

Endocrine
2018

Diagnosis and management of Zollinger-Ellison syndrome in 2018.

Minerva endocrinologica
2017

Catching the Zebra: Clinical Pearls and Pitfalls for the Successful Diagnosis of Zollinger-Ellison Syndrome.

Digestive diseases and sciences
2017

The Effect of H2 Receptor Antagonist in Acid Inhibition and Its Clinical Efficacy.

The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi
2017

Zollinger-Ellison Syndrome in a 12-year-old Child.

Journal of Indian Association of Pediatric Surgeons
2017

Gastrointestinal: Zollinger-Ellison Syndrome: A rare cause of chronic diarrhea and abdominal pain.

Journal of gastroenterology and hepatology
2017

Occupational contact allergy to omeprazole and ranitidine.

Medycyna pracy
2017

Neuroendocrine Tumors of the Stomach.

The Surgical clinics of North America
2017

A Perplexing Case of Abdominal Pain That Led to the Diagnosis of Zollinger-Ellison Syndrome.

Case reports in gastrointestinal medicine
2017

Deprescribing versus continuation of chronic proton pump inhibitor use in adults.

The Cochrane database of systematic reviews
2017

A case of type 1 multiple endocrine neoplasia with esophageal stricture successfully treated with endoscopic balloon dilation and local steroid injection combined with surgical resection of gastrinomas.

BMC gastroenterology
2017

[Neuroendocrine neoplasia of the stomach : What is new?].

Der Pathologe
2016

Zollinger-Ellison Syndrome: A Rare Case of Chronic Diarrhea.

Gastroenterology research
2016

Zollinger-Ellison syndrome: an unusual case of chronic diarrhoea in a child.

The Malaysian journal of pathology
2017

Assessing for Multiple Endocrine Neoplasia Type 1 in Patients Evaluated for Zollinger-Ellison Syndrome-Clues to a Safer Diagnostic Process.

The American journal of medicine
2016

[Subtype classification and clinicopathological characteristics of gastric neuroendocrine neoplasms: an analysis of 241 cases].

Zhonghua wei chang wai ke za zhi = Chinese journal of gastrointestinal surgery
2016

[Basic and translational research progress of gastrointestinal neuroendocrine neoplasmas].

Zhonghua wei chang wai ke za zhi = Chinese journal of gastrointestinal surgery
2017

Operation for insulinomas in multiple endocrine neoplasia type 1: When pancreatoduodenectomy is appropriate.

Surgery
2016

Inhibiting the proton pump: mechanisms, benefits, harms, and questions.

BMC medicine
2016

Effective and safe proton pump inhibitor therapy in acid-related diseases - A position paper addressing benefits and potential harms of acid suppression.

BMC medicine
2017

The appropriate use of proton pump inhibitors (PPIs): Need for a reappraisal.

European journal of internal medicine
2016

Treatment of symptomatic neuroendocrine tumor syndromes: recent advances and controversies.

Expert opinion on pharmacotherapy
2017

Per-oral endoscopic tunneling for restoration of the esophagus: a novel endoscopic submucosal dissection technique for therapy of complete esophageal obstruction.

Gastrointestinal endoscopy
2016

Management of gastric and duodenal neuroendocrine tumors.

World journal of gastroenterology
2017

Drug-Induced Vitamin B12 Deficiency: A Focus on Proton Pump Inhibitors and Histamine-2 Antagonists.

Journal of pharmacy practice
2016

Sporadic Gastric Well-Differentiated Neuroendocrine Tumors Have a Higher Ki-67 Proliferative Index.

Endocrine pathology
2016

[Switching to a generic drugA blessing or a curse?].

Nederlands tijdschrift voor geneeskunde
2016

Zollinger-Ellison Syndrome Associated with von Recklinghausen Disease: Case Report and Literature Review.

The American journal of case reports
2016

[Management of gastrinoma].

Presse medicale (Paris, France : 1983)
2016

Synchronous Peripancreatic Lymph Node Gastrinoma and Gastric Neuroendocrine Tumor Type 2.

Clinical endoscopy
2016

Clinical treatment of gastrinoma: A case report and review of the literature.

Oncology letters
2016

Stereoselective Pharmacodynamics and Pharmacokinetics of Proton Pump Inhibitors.

Current drug metabolism
2016

[Endoscopic therapy for gastroduodenal neuroendocrine neoplasms].

Der Chirurg; Zeitschrift fur alle Gebiete der operativen Medizen
2016

Imaging in multiple endocrine neoplasia type 1: recent studies show enhanced sensitivities but increased controversies.

International journal of endocrine oncology
2016

Pancreatic Tumor in a Patient With Parathyroid and Pituitary Disease.

Mayo Clinic proceedings
2016

Syndromic versus non-syndromic sporadic gastrin-producing neuroendocrine tumors of the duodenum: comparison of pathological features and biological behavior.

Virchows Archiv : an international journal of pathology
2016

Primary Hepatic Gastrinoma.

Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract
2015

Gastric Hypersecretory States: Investigation and Management.

Current treatment options in gastroenterology
2015

Biochemical Testing in Patients with Neuroendocrine Tumors.

Frontiers of hormone research
2015

Diagnostic and Treatment Approaches for Refractory Peptic Ulcers.

Clinical endoscopy
2016

Intraoperative near-infrared fluorescence visualization of the duodenal gastrinoma in a patient with Zollinger-Ellison syndrome.

Surgery
2015

Intraoperative Use of a Portable Large Field of View Gamma Camera and Handheld Gamma Detection Probe for Radioguided Localization and Prediction of Complete Surgical Resection of Gastrinoma: Proof of Concept.

Journal of the American College of Surgeons
2016

Weight Gain in Zollinger-Ellison Syndrome After Acid Suppression.

Pancreas
2015

Somatostatin analogs for gastric carcinoids: For many, but not all.

World journal of gastroenterology
2015

Gastric neuroendocrine neoplasms and proton pump inhibitors: fact or coincidence?

Scandinavian journal of gastroenterology
2015

Treatment of Severe Metabolic Alkalosis with Continuous Renal Replacement Therapy: Bicarbonate Kinetic Equations of Clinical Value.

ASAIO journal (American Society for Artificial Internal Organs : 1992)
Ver todos os 1.377 no EuropePMC

Associações

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Balancing therapeutic benefits and hidden risks of proton pump inhibitors in pediatric practice: a narrative review and update.
    Clinical and experimental pediatrics· 2026· PMID 41736231mais citado
  2. Esophageal Perforation in Zollinger-Ellison Syndrome: A Scoping Review of Management and Outcomes.
    JGH open : an open access journal of gastroenterology and hepatology· 2026· PMID 41585576mais citado
  3. Surgical Management of Zollinger-Ellison Syndrome in Multiple Endocrine Neoplasia Type 1 an AFCE and GTE Cohort Study. (Association Francophone de Chirurgie Endocrinienne and Groupe d'&#xe9;tude des Tumeurs Endocrines).
    World journal of surgery· 2026· PMID 41862416mais citado
  4. Rendezvous-assisted endoscopic retrograde pancreatography using a dual-wire balloon technique for stenotic pancreaticojejunostomy in post-Whipple anatomy.
    VideoGIE : an official video journal of the American Society for Gastrointestinal Endoscopy· 2026· PMID 41727390mais citado
  5. Microwave ablation as a therapeutic approach in Zollinger-Ellison syndrome.
    Gastroenterologia y hepatologia· 2026· PMID 41577236mais citado
  6. Severe Recurrent Gastrointestinal Bleeding and Diagnostic Dilemmas in Zollinger-Ellison Syndrome: A Case Report.
    GE Port J Gastroenterol· 2025· PMID 41623552recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:913(Orphanet)
  2. MONDO:0019610(MONDO)
  3. GARD:7918(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Artigo Wikipedia(Wikipedia)
  6. Q218712(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Síndrome Zollinger-Ellison
Compêndio · Raras BR

Síndrome Zollinger-Ellison

ORPHA:913 · MONDO:0019610
Prevalência
1-9 / 100 000
Herança
Not applicable
CID-10
E16.4 · Secreção anormal de gastrina
CID-11
Início
Adult, Elderly
Prevalência
0.0 (Europe)
MedGen
UMLS
C0043515
Repurposing
5 candidatos
cimetidinehistamine receptor antagonist
esomeprazoleATPase inhibitor
pantoprazoleATPase inhibitor|gastrin inhibitor
+2 outros
EuropePMC
Wikidata
Wikipedia
Papers 10a
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