1,629 results match your criteria Gastrinoma


Gastric Neuroendocrine Tumor and Duodenal Gastrinoma With Chronic Autoimmune Atrophic Gastritis.

Pancreas 2019 Jan;48(1):131-134

Division of Gastroenterology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York.

Our group observed the first case of synchronous gastric neuroendocrine tumor (NET) and duodenal gastrinoma with autoimmune chronic atrophic gastritis (CAG), in the absence of Helicobacter pylori infection. Demographic, clinical, endoscopic, and pathologic data were abstracted from the electronic medical record at Mount Sinai Hospital from 2013 to 2015. The patient's anonymity was carefully protected, and informed consent was obtained for publication of protected health information. Read More

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January 2019

The impact of failure to achieve symptom control after resection of functional neuroendocrine tumors: An 8-institution study from the US Neuroendocrine Tumor Study Group.

J Surg Oncol 2019 Jan 27;119(1):5-11. Epub 2018 Nov 27.

Division of Surgical Oncology, Department of Surgery, Winship Cancer Institute, Emory University, Atlanta, Georgia.

Background: The goals of resection of functional neuroendocrine tumors (NETs) are two-fold: Oncological benefit and symptom control. The interaction between the two is not well understood.

Methods: All patients with functional NETs of the pancreas, duodenum, and ampulla who underwent curative-intent resection between 2000 and 2016 were identified. Read More

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January 2019
5 Reads

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

Clin Nucl Med 2019 Jan;44(1):e36-e39

From the Departments of Nuclear Medicine.

Primary lymph node gastrinoma has been defined as gastrin-producing tumor present in lymph nodes and predominantly found in well-defined anatomical region known as gastrinoma triangle. They are usually localized preoperatively with imaging, and their surgical resection results in long-term relief. The authors report a case of unresectable primary lymph nodal gastrinoma with liver metastases in a 14-year-old adolescent boy with proven histopathology detected on Ga-DOTANOC whole-body PET/CT scan followed by preoperative multiple Lu-DOTATATE cycles for cytoreduction. Read More

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January 2019
19 Reads

A rare cause of upper gastrointestinal bleeding: Primary gastrinoma of the lesser omentum.

Presse Med 2018 10 22;47(10):913-915. Epub 2018 Oct 22.

University of Tunis el Manar, La Rabta University Hospital of Tunis, Department of Gastroenetrology A, Tunis, Tunisia.

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October 2018
2 Reads

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

J Clin Med Res 2018 Nov 9;10(11):843-847. Epub 2018 Oct 9.

Department of Internal Medicine and Endocrine Unit, Medical School and Clementino Fraga Filho University Hospital, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.

Neuroendocrine tumors (NETs) of cystic duct are extremely rare, accounting for less than 2% of NET cases. The association of biliary tree NET and multiple endocrine neoplasm type 1 (MEN1) are even more rare. In this report, we described a case of a 65-year-old woman who was referred to our neuroendocrine outpatient clinic to investigate MEN1 after an incidental diagnosis of gastrinoma. Read More

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November 2018
5 Reads

Prognostic relevance of proliferation-related miRNAs in pancreatic neuroendocrine neoplasms

Eur J Endocrinol 2018 10 1;179(4):219-228. Epub 2018 Oct 1.

‘Lendület’ Hereditary Endocrine Tumours Research Group, Hungarian Academy of Sciences – Semmelweis University, Budapest, Hungary

Objective: Pancreatic neuroendocrine neoplasms (PanNENs) are rare tumors arising from the endocrine pancreas; however, their prognosis differs significantly upon their proliferative state, which is characterized by histopathological grading. MiRNAs are small, noncoding RNAs posttranscriptionally regulating gene expression. Our aim was to identify miRNAs with altered expression upon proliferation which can be used as prognostic biomarkers in PanNENs. Read More

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October 2018
8 Reads

Surgical procedure for multifocal duodenopancreatic gastrinomas in a MEN-1 patient (with video).

J Visc Surg 2018 Sep 27. Epub 2018 Sep 27.

Department of Digestive and Endocrine Surgery, université Paris Descartes, Cochin Hospital, Assistance publique-Hôpitaux de Paris, 27, rue du Faubourg Saint-Jacques, 75014 Paris, France; Université Paris Descartes, Paris, France. Electronic address:

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September 2018

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

Ochsner J 2018 ;18(2):170-175

Department of Radiology, Ochsner Clinic Foundation, New Orleans, LA.

Background: Multiple endocrine neoplasia type 1 (MEN1) is a rare, autosomal dominant inherited syndrome caused by mutations in the MEN1 tumor suppressor gene. The diagnosis is defined clinically by the presence of 2 or more primary MEN1 tumors (parathyroid, anterior pituitary, and pancreatic islet). We describe the case of a patient who presented with classic history and imaging findings for MEN1. Read More

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January 2018
2 Reads

Gastric Carcinoids.

Endocrinol Metab Clin North Am 2018 09 11;47(3):645-660. Epub 2018 Jul 11.

1st Department of Propaedeutic Internal Medicine, National and Kapodistrian University of Athens, Mikras Asias 75, Athens 11527, Greece. Electronic address:

Gastric carcinoids, formally named gastric neuroendocrine neoplasms (NENs), are derived from enterochromaffin-like cells of the stomach and are increasingly diagnosed. A majority are designated as type I (related to autoimmune gastritis) and type II (related to gastrinoma) neoplasms that develop secondary to gastrin hypersecretion. Types I and II gastric carcinoids are mostly small-sized (1-2 cm), multiple, low-malignancy potential lesions mainly confined to the gastric mucosa/submucosa. Read More

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September 2018
3 Reads
3.400 Impact Factor

Gastrinomas: Medical or Surgical Treatment.

Endocrinol Metab Clin North Am 2018 09;47(3):577-601

Digestive Diseases Branch, NIDDK, National Institutes of Health, Building 10, Room 9C-103, Bethesda, MD 20892-1804, USA. Electronic address:

This article reviews the role of surgical and medical management in patients with Zollinger-Ellison syndrome (ZES) due to a gastrin-secreting neuroendocrine tumor (gastrinoma). It concentrates on the status at present but also briefly reviews the changes over time in treatment approaches. Generally, surgical and medical therapy are complementary today; however, in some cases, such as patients with ZES and multiple endocrine neoplasia type 1, the treatment approach remains controversial. Read More

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September 2018
3 Reads

A novel germline mutation at exon 10 of MEN1 gene: a clinical survey and positive genotype-phenotype analysis of a MEN1 Italian family, including monozygotic twins.

Hormones (Athens) 2018 Sep 6;17(3):427-435. Epub 2018 Aug 6.

EndOsmet Unit, Villa Donatello Private Hospital, Florence and Villalba Hospital, Bologna, Italy.

Context: Clinical phenotype variability in MEN1 syndrome exists and evidence for an established genotype-phenotype is lacking. However, a higher aggressiveness of MEN1-associated gastro-entero-pancreatic (GEP) (neuro)endocrine tumours (NETs) tumours has been reported when MEN1 gene truncating mutations are detected. We found a novel germline truncating mutation of MEN1 gene at exon 10 in a subject with an aggressive clinical behavior of GEP-NETs. Read More

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September 2018
1 Read

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

World J Hepatol 2018 Jul;10(7):517-522

Departamento de Gastroenterologia, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo 05403, Brazil.

Primary hepatic gastrinoma is a rare disease, with fewer than 40 cases reported in the medical literature. Because it is located in an organ in which metastases are common, its diagnosis is difficult. We report a case of a 19 years old male patient with a history of gastric ulcers since the age of nine. Read More

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July 2018
3 Reads

Imaging of pancreatic neuroendocrine tumors: recent advances, current status, and controversies.

Expert Rev Anticancer Ther 2018 Sep 17;18(9):837-860. Epub 2018 Jul 17.

b Digestive Diseases Branch , NIDDK, NIH , Bethesda , MD , USA.

Introduction: Recently, there have been a number of advances in imaging pancreatic neuroendocrine tumors (panNETs), as well as other neuroendocrine tumors (NETs), which have had a profound effect on the management and treatment of these patients, but in some cases are also associated with controversies. Areas covered: These advances are the result of numerous studies attempting to better define the roles of both cross-sectional imaging, endoscopic ultrasound, with or without fine-needle aspiration, and molecular imaging in both sporadic and inherited panNET syndromes; the increased attempt to develop imaging parameters that correlate with tumor classification or have prognostic value; the rapidly increasing use of molecular imaging in these tumors and the attempt to develop imaging parameters that correlate with treatment/outcome results. Each of these areas and the associated controversies are reviewed. Read More

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September 2018
8 Reads

Incidental Detection of Parathyroid Adenoma on Somatostatin Receptor PET/CT and Incremental Role of F-Fluorocholine PET/CT in MEN1 Syndrome.

Nucl Med Mol Imaging 2018 Jun 2;52(3):238-242. Epub 2018 May 2.

1Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, 110029 India.

Multiple endocrine neoplasia type 1 (MEN1) syndrome is characterized by combined occurrence of tumors of endocrine glands including the parathyroid, the pancreatic islet cells, and the anterior pituitary gland. Parathyroid involvement is the most common manifestation and usually the first clinical involvement in MEN1 syndrome, followed by gastroentero-pancreatic neuroendocrine tumors (NETs). Here we present a case where the patient initially presented with metastatic gastric NET and a single parathyroid adenoma was detected incidentally on Ga-DOTANOC PET/CT done as part of post Lu-DOTATATE therapy (PRRT) follow-up. Read More

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June 2018
13 Reads

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

Radiol Case Rep 2018 Apr 3;13(2):333-335. Epub 2018 Feb 3.

Department of Radiology, Division of Vascular and Interventional Radiology, University of Michigan Health Systems, 1500 East Medical Center Dr, Ann Arbor, MI 48109, USA.

SASI (selective arterial secretin injection) is a form of ASVS (arterial stimulation and venous sampling) used to localize pancreatic gastrinomas. This report aims to review the protocol for SASI and demonstrate its utility in localizing functional and nonfunctional gastrinomas. Even if a patient has a pancreatic mass and a laboratory profile fitting a specific endocrine syndrome, these may or may not be associated as has been previously demonstrated with adrenal vein sampling. Read More

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April 2018
4 Reads

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

J Vet Diagn Invest 2018 Jul 2;30(4):584-588. Epub 2018 Jun 2.

Animal Health Institute, Department of Pathology and Population Medicine, Midwestern University, Glendale, AZ (Struthers, Wong).

Gastrinoma, an infrequent diagnosis in middle-aged dogs, occurs with nonspecific gastrointestinal morbidity. Laboratory tests can yield a presumptive diagnosis, but definitive diagnosis depends on histopathology and immunohistochemistry. We describe a malignant pancreatic gastrinoma with lymph node metastases and corresponding Zollinger-Ellison syndrome in a Mexican gray wolf ( Canis lupus baileyi) and review this endocrine neoplasm in domestic dogs. Read More

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July 2018
2 Reads

Prospective evaluation of Ga-DOTATATE PET/CT in limited disease neuroendocrine tumours and/or elevated serum neuroendocrine biomarkers.

Clin Endocrinol (Oxf) 2018 May 22. Epub 2018 May 22.

Department of Nuclear Medicine, La Timone & North University Hospital, Aix-Marseille University, Marseille, France.

Context: The Ga-labelled somatostatin analogues ( Ga-DOTA-SSAs) is becoming popular as an important diagnostic tool in neuroendocrine tumours as evidenced by a growing number of reports detailing institutional experience with various DOTA peptides. However, only few prospective studies have compared Ga-DOTA-SSAs and somatostatin receptor scintigraphy (SRS) in gastroenteropancreatic neuroendocrine tumours (GEP-NETs) and pulmonary neuroendocrine tumours.

Objective: The aim of our prospective study was to perform head-to-head comparison between Ga-DOTATATE PET/CT and standard imaging work-up (SI) that included multiphasic CT, liver MRI and SRS using single photon emission computed tomography. Read More

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May 2018
8 Reads

[Primary lymph node gastrinoma].

Authors:
H Dralle

Chirurg 2018 May 7. Epub 2018 May 7.

Sektion Endokrine Chirurgie, Klinik für Allgemein‑, Viszeral- und Transplantationschirurgie, Universitätsklinikum Essen, Hufelandstr. 55, 45147, Essen, Deutschland.

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May 2018
1 Read

Duodenal neuroendocrine neoplasms: a still poorly recognized clinical entity.

Scand J Gastroenterol 2018 Jun - Jul;53(7):835-842. Epub 2018 May 4.

a Department of Gastroenterology and Endoscopy , Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico , Milan , Italy.

Background: Duodenal neuroendocrine neoplasms (dNENs) are rare tumors, which usually show good prognosis. The optimal management of these tumors is still far from being clearly understood because of their rarity and the poor level of knowledge about their natural history. Herein, we have reviewed the literature on dNENs to collect and analyze the current data on epidemiology, diagnosis and management of these rare tumors. Read More

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November 2018
2 Reads

[Surgical strategies for small sporadic neuroendocrine pancreatic tumors].

Authors:
K Holzer

Chirurg 2018 Jun;89(6):422-427

Sektionsleitung Endokrine Chirurgie, Klinik für Viszeral‑, Thorax- und Gefäßchirurgie, Universitätsklinikum Marburg, Baldingerstraße, 35043, Marburg, Deutschland.

Small (<2 cm) sporadic neuroendocrine pancreatic neoplasms (pNENs) are a heterogeneous group of neoplasms, which can be separated into functionally active or non-functional tumors. Functionally active pNENs release various hormones, such as insulin, gastrin, glucagon and vasoactive intestinal hormones and therefore lead to severe symptoms independent of their size. The main symptoms include hypoglycemia, coma (insulinoma), diarrhea (gastrinoma, VIPoma), intestinal ulcers (gastrinoma) and hyperglycemia (glucagonoma). Read More

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June 2018
2 Reads

A Rare Case of Colonic Metastases From Tonsillar Carcinoma: Case Report and Review of Literature.

World J Oncol 2018 Feb 8;9(1):35-37. Epub 2018 Mar 8.

Department of Medicine, Bronx Lebanon Hospital Center, Bronx, NY 10457, USA.

The incidence of tonsillar cancer has increased by four times in the United States over the last few decades likely due to recent increase in human papilloma virus (HPV) infections. The stage of the tumor predicts likelihood of metastasis, with advanced stages associated with higher chances of metastasis. The squamous cell carcinomas (SCCs) of the head and neck commonly metastasize to the lung, bone and liver in descending order. Read More

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February 2018
1 Read

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

Gastroenterology Res 2018 Feb 23;11(1):75-78. Epub 2018 Feb 23.

Department of Medicine, Bronx Lebanon Hospital Center, Bronx, NY 10457, USA.

We present a case of an 18-year-old male who presented with complains of abdominal pain, nausea and vomiting for 2 years. An esophagogastroduodenoscopy (EGD) revealed a 3 mm nodule on the lesser curvature of the stomach and prominent gastric folds. Biopsy of the nodule revealed a well-differentiated neuroendocrine tumor (NET) in lamina prop with focal extension into muscularis mucosa consistent with a gastric carcinoid. Read More

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February 2018
5 Reads

Disease Control on Lanreotide Autogel® 120 mg in a Patient with Metastatic Gastrinoma: A Case Report.

Case Rep Gastroenterol 2017 Sep-Dec;11(3):616-623. Epub 2017 Dec 6.

University Hospital, UZ Brussel, Department of Gastroenterology and Hepatology, Brussels, Belgium.

Gastrinomas are functionally active pancreatic neuroendocrine tumors (NETs) secreting gastrin and are associated with local or regional metastases in 60% of the cases. Somatostatin analogs (SSAs) are currently recommended as a first-line treatment for the symptomatic treatment of NETs. Although antiproliferative activity of SSAs has been demonstrated in various cancer types in several in vivo and in vitro studies, clinical benefits with SSAs have been only achieved in a small proportion of patients. Read More

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December 2017

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

Int J Endocr Oncol 2017 11;4(4):167-185. Epub 2017 Oct 11.

Digestive Diseases Branch, NIDDK, NIH, Bethesda, Maryland, 20817, USA.

In recent years the diagnosis of Zollinger-Ellison syndrome (ZES) has become increasingly controversial with several new approaches and criteria proposed, differing from the classical biochemical criterion of inappropriate hypergastrinemia (i.e., hypergastrinemia in the presence of hyperchlorhydria) (Table 1). Read More

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October 2017
10 Reads

Going from A to Z.

N Engl J Med 2018 01;378(1):73-79

From the Department of Medicine, Johns Hopkins Hospital and Johns Hopkins University School of Medicine, Baltimore (D.A., D.F., R.M.); and the Department of Medicine, University of California, San Francisco, and the Medical Service, San Francisco Veterans Affairs Medical Center - both in San Francisco (G.D., K.R.F.).

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January 2018
17 Reads

Hormone secreting gastro-entero-pancreatic neuroendocrine neoplasias (GEP-NEN): When to consider, how to diagnose?

Rev Endocr Metab Disord 2017 12;18(4):393-410

Schwerpunkt Endokrinologie und Stoffwechselerkrankungen, I. Medizinischen Klinik und Poliklinik; ENETS center of excellence, Universitätsmedizin der Johannes Gutenberg-Universität Mainz, Langenbeckstrasse 1, 55101, Mainz, Germany.

Neuroendocrine neoplasms of the digestive system (GEP-NEN) represent a heterogeneous group of malignancies with various clinical presentation and prognosis. GEP-NENs can potentially affect all organs of the gastrointestinal tract; characteristically they share the biological property to produce and secrete peptides and neuroamines. About 30% of GEP-NENs are hormonally active and can cause specific clinical syndromes. Read More

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December 2017
9 Reads

: Assessment of Response to Treatment and Follow-Up in Gastroenteropancreatic Neuroendocrine Neoplasms.

Endocr Metab Immune Disord Drug Targets 2018 ;18(5):419-449

Department of Endocrine Oncology, University Hospital, Uppsala, Sweden.

Well-established criteria for evaluating the response to treatment and the appropriate followup of individual patients are critical in clinical oncology. The current evidence-based data on these issues in terms of the management of gastroenteropancreatic (GEP) neuroendocrine neoplasms (NEN) are unfortunately limited. This document by the Italian Association of Clinical Endocrinologists (AME) on the criteria for the follow-up of GEP-NEN patients is aimed at providing comprehensive recommendations for everyday clinical practice based on both the best available evidence and the combined opinion of an interdisciplinary panel of experts. Read More

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January 2018
10 Reads

A primary hepatic gastrinoma accompanied by hyperplasia of multi-nodular Brunner's glands.

Surg Case Rep 2017 Nov 28;3(1):118. Epub 2017 Nov 28.

Department of Gastroenterological Surgery, Kansai Electric Power Hospital, 2-1-7 Fukushima, Fukushima-ku, Osaka, 553-0003, Japan.

Background: Primary hepatic gastrinoma causing severe ulcerogenic syndrome is extremely rare. Herein, we report a case of primary hepatic gastrinoma accompanied by hyperplasia of multi-nodular Brunner's glands in a patient who instead, preoperatively, was suspected of having multiple duodenal gastrinomas and hepatic metastasis.

Case Presentation: A 57-year-old woman consulted a clinic complaining of melena, intermittent abdominal pain, diarrhea, and vomiting which had persisted for about 3 years. Read More

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November 2017
6 Reads

Surgical Therapy of Sporadic Pancreatic Neuroendocrine Neoplasias G1/G2.

Visc Med 2017 Oct 4;33(5):344-350. Epub 2017 Oct 4.

Department of Visceral-, Thoracic- and Vascular Surgery, University Hospital Marburg, Marburg, Germany.

Background: Pancreatic neuroendocrine neoplasias (pNENs) are uncommon but fascinating tumors with an annual incidence of 1 per 100,000 people. pNENs present either as functional tumors, causing specific hormonal syndromes like Zollinger-Ellison syndrome (ZES) or organic hyperinsulinism, or as non-functional pancreatic tumors (NF-pNENs). The natural history of pNENs is highly variable. Read More

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October 2017
6 Reads

Early Gastroenteropancreatic Neuroendocrine Tumors: Endoscopic Therapy and Surveillance.

Visc Med 2017 Oct 10;33(5):332-338. Epub 2017 Oct 10.

Service d'Hépato-Gastroentérologie, Hôpital Robert Debré, Reims, France.

Neuroendocrine neoplasias (NEN) of the stomach, duodenum, pancreas, appendix, or rectum that are ≤1 cm in size as well as well-differentiated with World Health Organization grade 1 (G1) can be considered 'early' neuroendocrine tumors; they have a very good prognosis. Regarding prognosis, neuroendocrine tumors (NET) G1 must be distinguished from well-differentiated NET G2 and poorly differentiated neuroendocrine carcinomas (NEC) G3. NET are increasing, with a rise in the age-adjusted incidence in the USA by about 700% in the last 40 years. Read More

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October 2017
3 Reads

Effect of gastric acid-suppressive therapy and biological variation of serum gastrin concentrations in dogs with chronic enteropathies.

BMC Vet Res 2017 Nov 7;13(1):321. Epub 2017 Nov 7.

Gastrointestinal Laboratory, Texas A&M University, TAMU 4474, College Station, TX, 77843-4474, USA.

Background: Serum gastrin concentration can help diagnose gastrinomas in dogs if >3-10× the upper reference limit (URL), but antisecretory therapy and other conditions can also cause hypergastrinemia. Effects of antisecretory therapy (famotidine or ranitidine, omeprazole) on serum gastrin concentration in dogs with chronic enteropathy (CE) and its biological variation (BV) are unknown. Aim of the study was to evaluate serum gastrin in acid-suppressant-treated or -naïve CE dogs; test the association between serum gastrin and histopathologic findings in acid-suppressant-naïve CE dogs; and evaluate the BV of serum gastrin in dogs not receiving any gastric acid suppressive therapy. Read More

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November 2017
17 Reads

Enteric Glia: The Origin of Duodenal Gastrinomas?

Gastroenterology 2017 12 31;153(6):1473-1475. Epub 2017 Oct 31.

Neuroscience Program and Department of Physiology, Michigan State University, East Lansing, Michigan. Electronic address:

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December 2017
5 Reads

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

Endocrine 2018 04 10;60(1):15-27. Epub 2017 Oct 10.

Thyroid and Parathyroid Surgery Unit, Istituto Nazionale per lo studio e la cura dei tumori "Fondazione G. Pascale" - IRCCS, Naples, Italy.

Purpose: Analyze the role of somatostatin analogues (SSAs) in the treatment of sporadic and MEN1-related gastrinomas, trying to define whether recent trials have changed the landscape of gastrinoma therapy.

Methods: We evaluate the rationale of SSA use in the treatment of gastrinomas, summarize the current literature concerning the effect of SSAs on the control of Zollinger-Ellison syndrome (ZES) and gastrinomas tumor progression and discuss their role in the most recent guidelines.

Results: The medical treatment of gastrinoma and related ZES is aimed at controlling acid hypersecretion and tumor progression, in inoperable patients. Read More

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April 2018
31 Reads

Diagnosis and management of Zollinger-Ellison syndrome in 2018.

Minerva Endocrinol 2018 Jun 25;43(2):212-220. Epub 2017 Sep 25.

Department of Gastroenterology and Digestive Endoscopy, AOU Città della Salute e della Scienza, University of Turin, Turin, Italy.

Zollinger-Ellison syndrome (ZES) is a clinical syndrome characterized by gastric acid hypersecretion due to the ectopic secretion of gastrin by a gastrinoma, a neuroendocrine tumor (NET) which mostly develops in the duodenum and in the pancreas. This syndrome was first described by Zollinger and Ellison in 1964; if left untreated, ZES can lead to multiple complications mainly due to gastric hypersecretion and some patients can suffer from the complications of an advanced metastatic disease. Although its clinical features are considered typical, the diagnosis of ZES is often challenging for the clinician. Read More

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June 2018
35 Reads

Gastrin Induces Nuclear Export and Proteasome Degradation of Menin in Enteric Glial Cells.

Gastroenterology 2017 12 30;153(6):1555-1567.e15. Epub 2017 Aug 30.

Department of Internal Medicine, Division of Gastroenterology, University of Michigan, Ann Arbor, Michigan; Department of Molecular and Integrative Physiology, University of Michigan, Ann Arbor, Michigan. Electronic address:

Background & Aims: The multiple endocrine neoplasia, type 1 (MEN1) locus encodes the nuclear protein and tumor suppressor menin. MEN1 mutations frequently cause neuroendocrine tumors such as gastrinomas, characterized by their predominant duodenal location and local metastasis at time of diagnosis. Diffuse gastrin cell hyperplasia precedes the appearance of MEN1 gastrinomas, which develop within submucosal Brunner's glands. Read More

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December 2017
14 Reads

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

Dig Dis Sci 2017 09 3;62(9):2258-2265. Epub 2017 Aug 3.

Division of Gastroenterology and Hepatology, Department of Internal Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Zollinger-Ellison syndrome (ZES) results from an ectopic gastrin-secreting tumor leading to peptic ulcer disease, reflux, and chronic diarrhea. While early recognition portends an excellent prognosis with >80% survival at 15 years, symptoms are often nonspecific making the diagnosis difficult to establish. Diagnosis involves a series of tests, including fasting gastrin, gastric pH, chromogranin A, and secretin stimulation. Read More

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September 2017
15 Reads

The future: medical advances in MEN1 therapeutic approaches and management strategies.

Endocr Relat Cancer 2017 10 2;24(10):T179-T193. Epub 2017 Aug 2.

Department of Endocrine OncologyUniversity Medical Center Utrecht, Utrecht, The Netherlands.

Multiple endocrine neoplasia type 1 is a rare autosomal inherited disorder associated with a high risk for patients to simultaneously develop tumors of the parathyroid glands, duodenopancreatic neuroendocrine tumors and tumors of the anterior pituitary gland. Early identification of in patients enables presymptomatic screening of manifestations, which makes timely interventions possible with the intention to prevent morbidity and mortality. Causes of death nowadays have shifted toward local or metastatic progression of malignant neuroendocrine tumors. Read More

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October 2017
8 Reads

Pancreatic Imaging.

Authors:
Mark Masciocchi

Endocrinol Metab Clin North Am 2017 09 12;46(3):761-781. Epub 2017 Jun 12.

Department of Radiology, UMass Memorial Medical Center, University of Massachusetts Medical School, 55 Lake Avenue North, Worcester, MA 01655, USA. Electronic address:

Imaging of the endocrine pancreas is dominated by neuroendocrine tumors, a diverse category of neoplasms that may or may not cause symptoms from hormone hypersecretion. These tumors may also be evidence of several different genetic syndromes. Understanding the usefulness of different imaging modalities and entities that simulate neuroendocrine tumors is key for both radiologists and referring physicians. Read More

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September 2017
8 Reads

Primary lymph node gastrinoma: A single institution experience.

Surgery 2017 11 10;162(5):1088-1094. Epub 2017 Jul 10.

Department of Surgery, Massachusetts General Hospital, Boston, MA. Electronic address:

Background: Gastrinomas are rare neuroendocrine tumors that ectopically secrete gastrin and classically originate within the duodenum or pancreas. The presence of primary lymph node gastrinoma is controversial. We report on a single institution's experience with gastrinoma, with focus on primary lymph node tumors. Read More

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November 2017
12 Reads

Clinicopathological features and prognosis of gastroenteropancreatic neuroendocrine neoplasms in a Chinese population: a large, retrospective single-centre study.

BMC Endocr Disord 2017 Jul 13;17(1):39. Epub 2017 Jul 13.

Department of Gastroenterology, the First Affiliated Hospital of Zhengzhou University, No.1, East Jianshe Road, Zhengzhou, 450052, China.

Background: Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are the most common type of neuroendocrine tumors, accounting for more than half of neuroendocrine neoplasms (NENs). We performed a retrospective study in our center to investigate the clinicopathological features, risk factors of metastasis, and prognosis of GEP-NENs in a Chinese population.

Methods: Four hundred forty patients with GEP-NENs treated at the First Affiliated Hospital of Zhengzhou University between January 2011 and March 2016 were analyzed retrospectively. Read More

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July 2017
29 Reads

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

J Indian Assoc Pediatr Surg 2017 Jul-Sep;22(3):168-169

Department of Pediatric Surgery, Dr. D. Y. Patil Hospital and Research Centre, Navi Mumbai, Maharashtra, India.

The syndrome described by Zollinger and Ellison in 1955 is a rare clinical entity which is even rarer in children. This report describes a 12-year-old boy who presented with refractory peptic ulcer disease which was finally diagnosed to be due to a gastrinoma and was successfully treated. Read More

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July 2017
8 Reads

Long-Term Efficacy of S-1 Chemotherapy plus Administration of Octreotide for a Patient with Metastatic Neuroendocrine Tumor (Gastrinoma).

Case Rep Oncol 2017 May-Aug;10(2):420-427. Epub 2017 May 8.

Department of Clinical Oncology, Iwate Prefectural Central Hospital, Morioka, Japan.

Metastatic neuroendocrine tumors (gastrinomas) have a poor prognosis. Octreotide can reduce gastrin levels and alleviate hormonal symptoms, and possibly slow tumor growth as well. No drugs were available except streptozocin for the treatment of metastatic pancreatic neuroendocrine tumor (PNET) in 2008. Read More

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May 2017
6 Reads

Diagnostic and therapeutic guidelines for gastro-entero-pancreatic neuroendocrine neoplasms (recommended by the Polish Network of Neuroendocrine Tumours).

Endokrynol Pol 2017 ;68(2):79-110

Klinika Endokrynologii i Nowotworów Neuroendokrynnych, Katedra Patofizjologii i Endokrynologii, Śląski Uniwersytet Medyczny.

Progress in the diagnostics and therapy of gastro-entero-pancreatic (GEP) neuroendocrine neoplasms (NEN), the published results of new randomised clinical trials, and the new guidelines issued by the European Neuroendocrine Tumour Society (ENETS) have led the Polish Network of Neuroendocrine Tumours to update the 2013 guidelines regarding management of these neoplasms. We present the general recommendations for the management of NENs, developed by experts during the Third Round Table Conference - Diagnostics and therapy of gastro-entero-pancreatic neuroendocrine neoplasms: Polish recommendations in view of current European recommenda-tions, which took place in December 2016 in Żelechów near Warsaw. Drawing from the extensive experience of centres dealing with this type of neoplasms, we hope that we have managed to develop the optimal management system, applying the most recent achievements in the field of medicine, for these patients, and that it can be implemented effectively in Poland. Read More

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July 2017
34 Reads

Challenging Differential Diagnosis of Hypergastremia and Hyperglucagonemia with Chronic Renal Failure: Report of a Case with Multiple Endocrine Neoplasia Type 1.

Intern Med 2017 1;56(11):1375-1381. Epub 2017 Jun 1.

Department of Diabetes and Endocrinology, Osaka Red Cross Hospital, Japan.

A 53-year-old woman developed end-stage renal failure during a 15-year clinical course of primary hyperparathyroidism and was referred to our hospital for evaluation of suspected multiple endocrine neoplasia type 1 (MEN1). Genetic testing revealed a novel deletion mutation at codon 467 in exon 10 of the MEN1 gene. Systemic and selective arterial calcium injection (SACI) testing revealed hyperglucagonemia and hypergastrinemia with positive gastrin responses. Read More

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November 2017
18 Reads

Gastroduodenal neuroendocrine neoplasms, including gastrinoma - management guidelines (recommended by the Polish Network of Neuroendocrine Tumours).

Endokrynol Pol 2017 ;68(2):138-153

This paper presents the updated Polish Neuroendocrine Tumour Network expert panel recommendations on the management of neuroendocrine neoplasms (NENs) of the stomach and duodenum, including gastrinoma. The recommendations discuss the epidemiology, pathogenesis, and clinical presentation of these tumours as well as their diagnosis, including biochemical, histopathological, and localisation diagnoses. The principles of treatment are discussed, including endoscopic, surgical, pharmacological, and radionuclide treatments. Read More

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July 2017
43 Reads

Management of the hormonal syndrome of neuroendocrine tumors.

Arch Med Sci 2017 Apr 1;13(3):515-524. Epub 2016 Jun 1.

Department of Endocrinology, Poznan University of Medical Sciences, Poznan, Poland.

Gastroenteropancreatic neuroendocrine tumors (GEP/NET) are unusual and rare neoplasms that present many clinical challenges. They characteristically synthesize store and secrete a variety of peptides and neuroamines which can lead to the development of distinct clinical syndrome, however many are clinically silent until late presentation with mass effects. Management strategies include surgery cure and cytoreduction with the use of somatostatin analogues. Read More

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April 2017
23 Reads