Zollinger-Ellison syndrome

Common Name(s)

Zollinger-Ellison syndrome

Zollinger-Ellison syndrome (ZES) is a condition in which tumors called gastrinomas in the pancreas and duodenum (part of the small intestine) cause high levels of the hormone gastrin in the blood. High levels of gastrin then cause production of too much stomach acid. Signs and symptoms may include abdominal pain, peptic ulcers, vomiting blood, and diarrhea. The tumors are sometimes cancerous and may spread to other areas of the body. In most cases, the cause of ZES is unknown. However, about 25-30% of gastrinomas are caused by an inherited condition called multiple endocrine neoplasia type 1 (MEN1). Treatment for ZES may include medication to reduce the production of stomach acid, and surgery for peptic ulcers or to remove tumors.
 

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Condition Specific Organizations

Following organizations serve the condition "Zollinger-Ellison syndrome" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Zollinger-Ellison syndrome" returned 98 free, full-text research articles on human participants. First 3 results:

A case of Zollinger-Ellison syndrome in multiple endocrine neoplasia type 1 with urolithiasis as the initial presentation.
 

Author(s): Na Eun Lee, Young Jae Lee, So Hee Yun, Jae Un Lee, Moon Sik Park, Joong Keun Kim, Ji Woong Kim, Jin Woong Cho

Journal: Korean J Gastroenterol. 2013 Jun;61(6):333-7.

 

Zollinger-Ellison syndrome (ZES) is characterized by gastrinoma and resultant hypergastrinemia, which leads to recurrent peptic ulcers. Because gastrinoma is the most common pancreatic endocrine tumor seen in multiple endocrine neoplasia type I (MEN 1), the possibility of gastrinoma ...

Last Updated: 23 Jul 2013

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Hypochlorhydria and achlorhydria are associated with false-positive secretin stimulation testing for Zollinger-Ellison syndrome.
 

Author(s): Pari Shah, Maneesh H Singh, Yu-Xiao Yang, David C Metz

Journal: Pancreas. 2013 Aug;42(6):932-6.

 

Secretin stimulation testing (SST) is used to evaluate patients with hypergastrinemia in the diagnosis of Zollinger-Ellison syndrome. Case series have documented false-positive SST in patients with achlorhydria. This study reviews our experience with SST in hypochlorhydric and achlorhydric patients.

Last Updated: 15 Jul 2013

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Causes of death and prognostic factors in multiple endocrine neoplasia type 1: a prospective study: comparison of 106 MEN1/Zollinger-Ellison syndrome patients with 1613 literature MEN1 patients with or without pancreatic endocrine tumors.
 

Author(s): Tetsuhide Ito, Hisato Igarashi, Hirotsugu Uehara, Marc J Berna, Robert T Jensen

Journal: Medicine (Baltimore). 2013 May;92(3):135-81.

 

Multiple endocrine neoplasia type 1 (MEN1) is classically characterized by the development of functional or nonfunctional hyperplasia or tumors in endocrine tissues (parathyroid, pancreas, pituitary, adrenal). Because effective treatments have been developed for the hormone excess ...

Last Updated: 9 May 2013

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Zollinger-Ellison syndrome" returned 7 free, full-text review articles on human participants. First 3 results:

Pharmacotherapy of Zollinger-Ellison syndrome.
 

Author(s): Tetsuhide Ito, Hisato Igarashi, Hirotsugu Uehara, Robert T Jensen

Journal: Expert Opin Pharmacother. 2013 Feb;14(3):307-21.

 

The role of pharmacotherapy in the management of patients with Zollinger-Ellison syndrome (ZES) is often equated with the medical management of acid hypersecretion. However, pharmacotherapy is also increasingly involved in the other management areas of these patients.

Last Updated: 4 Feb 2013

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Diagnosis of Zollinger-Ellison syndrome: increasingly difficult.
 

Author(s): Tetsuhide Ito, Guillaume Cadiot, Robert T Jensen

Journal: World J. Gastroenterol.. 2012 Oct;18(39):5495-503.

 

In the present paper the increasing difficulty of diagnosis of Zollinger-Ellison syndrome (ZES) due to issues raised in two recent papers is discussed. These issues involve the difficulty and need to withdraw patients suspected of ZES from treatment with Proton Pump Inhibitors (omeprazole, ...

Last Updated: 31 Oct 2012

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Treatment of Zollinger-Ellison syndrome.
 

Author(s): Paola Tomassetti, Davide Campana, Lydia Piscitelli, Elena Mazzotta, Emilio Brocchi, Raffaele Pezzilli, Roberto Corinaldesi

Journal: World J. Gastroenterol.. 2005 Sep;11(35):5423-32.

 

In this article, we have reviewed the main therapeutic measures for the treatment of Zollinger-Ellison syndrome (ZES). Review of the literature was based on computer searches (Pub-Med, Index Medicus) and personal experiences. We have evaluated all the measures now available for treating ...

Last Updated: 13 Oct 2005

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

A Phase I/III Study of D961H 10 mg and 20 mg in Japanese Paediatric Patients With Gastrointestinal Acid Related Diseases
 

Status: Recruiting

Condition Summary: Gastric Ulcer (GU); Duodenal Ulcer (DU); Anastomotic Ulcer (AU); Non-erosive Reflux Esophagitis Disease (NERD); Reflux Esophagitis (RE); Zollinger-Ellison Syndrome

 

Last Updated: 1 Aug 2014

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Last Updated: 17 Jun 2014

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Romidepsin in Treating Patients With Lymphoma, Chronic Lymphocytic Leukemia, or Solid Tumors With Liver Dysfunction
 

Status: Recruiting

Condition Summary: Adult Anaplastic Astrocytoma; Adult Anaplastic Ependymoma; Adult Anaplastic Oligodendroglioma; Adult Brain Stem Glioma; Adult Ependymoblastoma; Adult Giant Cell Glioblastoma; Adult Glioblastoma; Adult Gliosarcoma; Adult Mixed Glioma; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Adult Pineal Gland Astrocytoma; AIDS-related Diffuse Large Cell Lymphoma; AIDS-related Diffuse Mixed Cell Lymphoma; AIDS-related Diffuse Small Cleaved Cell Lymphoma; AIDS-related Immunoblastic Large Cell Lymphoma; AIDS-related Lymphoblastic Lymphoma; AIDS-related Peripheral/Systemic Lymphoma; AIDS-related Primary CNS Lymphoma; AIDS-related Small Noncleaved Cell Lymphoma; Anaplastic Large Cell Lymphoma; Anaplastic Thyroid Cancer; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extensive Stage Small Cell Lung Cancer; Extra-adrenal Paraganglioma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Gastrinoma; Glucagonoma; Hepatic Complications; Hepatosplenic T-cell Lymphoma; HIV-associated Hodgkin Lymphoma; Insular Thyroid Cancer; Insulinoma; Intraocular Lymphoma; Male Breast Cancer; Metastatic Gastrointestinal Carcinoid Tumor; Metastatic Pheochromocytoma; Metastatic Squamous Neck Cancer With Occult Primary Squamous Cell Carcinoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Pancreatic Polypeptide Tumor; Peripheral T-cell Lymphoma; Pulmonary Carcinoid Tumor; Recurrent Adenoid Cystic Carcinoma of the Oral Cavity; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Brain Tumor; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Basal Cell Carcinoma of the Lip; Recurrent Bladder Cancer; Recurrent Breast Cancer; Recurrent Colon Cancer; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Esthesioneuroblastoma of the Paranasal Sinus and Nasal Cavity; Recurrent Gastrointestinal Carcinoid Tumor; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Inverted Papilloma of the Paranasal Sinus and Nasal Cavity; Recurrent Islet Cell Carcinoma; Recurrent Lymphoepithelioma of the Nasopharynx; Recurrent Lymphoepithelioma of the Oropharynx; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Melanoma; Recurrent Metastatic Squamous Neck Cancer With Occult Primary; Recurrent Midline Lethal Granuloma of the Paranasal Sinus and Nasal Cavity; Recurrent Mucoepidermoid Carcinoma of the Oral Cavity; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Neuroendocrine Carcinoma of the Skin; Recurrent Non-small Cell Lung Cancer; Recurrent Pancreatic Cancer; Recurrent Pheochromocytoma; Recurrent Prostate Cancer; Recurrent Rectal Cancer; Recurrent Renal Cell Cancer; Recurrent Salivary Gland Cancer; Recurrent Small Cell Lung Cancer; Recurrent Small Lymphocytic Lymphoma; Recurrent Squamous Cell Carcinoma of the Hypopharynx; Recurrent Squamous Cell Carcinoma of the Larynx; Recurrent Squamous Cell Carcinoma of the Lip and Oral Cavity; Recurrent Squamous Cell Carcinoma of the Nasopharynx; Recurrent Squamous Cell Carcinoma of the Oropharynx; Recurrent Squamous Cell Carcinoma of the Paranasal Sinus and Nasal Cavity; Recurrent Thyroid Cancer; Recurrent Verrucous Carcinoma of the Larynx; Recurrent Verrucous Carcinoma of the Oral Cavity; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Regional Gastrointestinal Carcinoid Tumor; Regional Pheochromocytoma; Small Intestine Lymphoma; Somatostatinoma; Splenic Marginal Zone Lymphoma; Stage III Adenoid Cystic Carcinoma of the Oral Cavity; Stage III Adult Soft Tissue Sarcoma; Stage III Basal Cell Carcinoma of the Lip; Stage III Bladder Cancer; Stage III Esthesioneuroblastoma of the Paranasal Sinus and Nasal Cavity; Stage III Follicular Thyroid Cancer; Stage III Inverted Papilloma of the Paranasal Sinus and Nasal Cavity; Stage III Lymphoepithelioma of the Nasopharynx; Stage III Lymphoepithelioma of the Oropharynx; Stage III Midline Lethal Granuloma of the Paranasal Sinus and Nasal Cavity; Stage III Mucoepidermoid Carcinoma of the Oral Cavity; Stage III Neuroendocrine Carcinoma of the Skin; Stage III Pancreatic Cancer; Stage III Papillary Thyroid Cancer; Stage III Prostate Cancer; Stage III Renal Cell Cancer; Stage III Salivary Gland Cancer; Stage III Squamous Cell Carcinoma of the Hypopharynx; Stage III Squamous Cell Carcinoma of the Larynx; Stage III Squamous Cell Carcinoma of the Lip and Oral Cavity; Stage III Squamous Cell Carcinoma of the Nasopharynx; Stage III Squamous Cell Carcinoma of the Oropharynx; Stage III Squamous Cell Carcinoma of the Paranasal Sinus and Nasal Cavity; Stage III Verrucous Carcinoma of the Larynx; Stage III Verrucous Carcinoma of the Oral Cavity; Stage IIIA Breast Cancer; Stage IIIA Colon Cancer; Stage IIIA Melanoma; Stage IIIA Non-small Cell Lung Cancer; Stage IIIA Rectal Cancer; Stage IIIB Breast Cancer; Stage IIIB Colon Cancer; Stage IIIB Melanoma; Stage IIIB Non-small Cell Lung Cancer; Stage IIIB Rectal Cancer; Stage IIIC Breast Cancer; Stage IIIC Colon Cancer; Stage IIIC Melanoma; Stage IIIC Rectal Cancer; Stage IV Adult Soft Tissue Sarcoma; Stage IV Bladder Cancer; Stage IV Breast Cancer; Stage IV Lymphoepithelioma of the Nasopharynx; Stage IV Melanoma; Stage IV Neuroendocrine Carcinoma of the Skin; Stage IV Non-small Cell Lung Cancer; Stage IV Pancreatic Cancer; Stage IV Prostate Cancer; Stage IV Renal Cell Cancer; Stage IV Squamous Cell Carcinoma of the Hypopharynx; Stage IV Squamous Cell Carcinoma of the Nasopharynx; Stage IVA Adenoid Cystic Carcinoma of the Oral Cavity; Stage IVA Basal Cell Carcinoma of the Lip; Stage IVA Colon Cancer; Stage IVA Esthesioneuroblastoma of the Paranasal Sinus and Nasal Cavity; Stage IVA Follicular Thyroid Cancer; Stage IVA Inverted Papilloma of the Paranasal Sinus and Nasal Cavity; Stage IVA Lymphoepithelioma of the Oropharynx; Stage IVA Midline Lethal Granuloma of the Paranasal Sinus and Nasal Cavity; Stage IVA Mucoepidermoid Carcinoma of the Oral Cavity; Stage IVA Papillary Thyroid Cancer; Stage IVA Rectal Cancer; Stage IVA Salivary Gland Cancer; Stage IVA Squamous Cell Carcinoma of the Larynx; Stage IVA Squamous Cell Carcinoma of the Lip and Oral Cavity; Stage IVA Squamous Cell Carcinoma of the Oropharynx; Stage IVA Squamous Cell Carcinoma of the Paranasal Sinus and Nasal Cavity; Stage IVA Verrucous Carcinoma of the Larynx; Stage IVA Verrucous Carcinoma of the Oral Cavity; Stage IVB Adenoid Cystic Carcinoma of the Oral Cavity; Stage IVB Basal Cell Carcinoma of the Lip; Stage IVB Colon Cancer; Stage IVB Esthesioneuroblastoma of the Paranasal Sinus and Nasal Cavity; Stage IVB Follicular Thyroid Cancer; Stage IVB Inverted Papilloma of the Paranasal Sinus and Nasal Cavity; Stage IVB Lymphoepithelioma of the Oropharynx; Stage IVB Midline Lethal Granuloma of the Paranasal Sinus and Nasal Cavity; Stage IVB Mucoepidermoid Carcinoma of the Oral Cavity; Stage IVB Papillary Thyroid Cancer; Stage IVB Rectal Cancer; Stage IVB Salivary Gland Cancer; Stage IVB Squamous Cell Carcinoma of the Larynx; Stage IVB Squamous Cell Carcinoma of the Lip and Oral Cavity; Stage IVB Squamous Cell Carcinoma of the Oropharynx; Stage IVB Squamous Cell Carcinoma of the Paranasal Sinus and Nasal Cavity; Stage IVB Verrucous Carcinoma of the Larynx; Stage IVB Verrucous Carcinoma of the Oral Cavity; Stage IVC Adenoid Cystic Carcinoma of the Oral Cavity; Stage IVC Basal Cell Carcinoma of the Lip; Stage IVC Esthesioneuroblastoma of the Paranasal Sinus and Nasal Cavity; Stage IVC Follicular Thyroid Cancer; Stage IVC Inverted Papilloma of the Paranasal Sinus and Nasal Cavity; Stage IVC Lymphoepithelioma of the Oropharynx; Stage IVC Midline Lethal Granuloma of the Paranasal Sinus and Nasal Cavity; Stage IVC Mucoepidermoid Carcinoma of the Oral Cavity; Stage IVC Papillary Thyroid Cancer; Stage IVC Salivary Gland Cancer; Stage IVC Squamous Cell Carcinoma of the Larynx; Stage IVC Squamous Cell Carcinoma of the Lip and Oral Cavity; Stage IVC Squamous Cell Carcinoma of the Oropharynx; Stage IVC Squamous Cell Carcinoma of the Paranasal Sinus and Nasal Cavity; Stage IVC Verrucous Carcinoma of the Larynx; Stage IVC Verrucous Carcinoma of the Oral Cavity; T-cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Thyroid Gland Medullary Carcinoma; Tongue Cancer; Unspecified Adult Solid Tumor, Protocol Specific; Waldenström Macroglobulinemia

 

Last Updated: 23 Sep 2014

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