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World J Surg Oncol 2019 Apr 11;17(1):66. Epub 2019 Apr 11.

Department of Integrative Oncology, China-Japan Friendship Hospital, 2 Yinghuadong Road, Chaoyang District, Beijing, 100029, China.

Background: Well-differentiated pancreatic neuroendocrine tumors (PanNETs) usually have a good prognosis; however, there are patients that experience recurrence after curative resection.

Aim: To explore recurrence-related risk factors by analyzing clinicopathological data of PanNETs after radical surgery.

Methods: Clinical and pathological data from 47 patients with well-differentiated PanNETs at China-Japan Friendship Hospital from January 2012 to March 2016 were analyzed retrospectively. Read More

Chirurg 2019 Mar;90(Suppl 2):100

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

Am J Surg 2019 May 28;217(5):937-942. Epub 2018 Dec 28.

Digestive Disease Institute, Virginia Mason Medical Center, Seattle, WA, USA. Electronic address:

Background: Gastric carcinoids are rare neuroendocrine tumors of the gastrointestinal tract. They are typically managed according to their etiology. However, there is little known about the impact of surgical strategy on the long-term outcomes of these patients. Read More

Ann Surg 2018 Dec 21. Epub 2018 Dec 21.

INSERM, U1231, Epidemiology and Clinical Research in Digestive Cancers Team, Dijon, France.

Objective: To assess the distant metastatic potential of duodeno-pancreatic neuroendocrine tumors (DP-NETs) in patients with MEN1, according to functional status and size.

Summary Background Data: DP-NETs, with their numerous lesions and endocrine secretion-related symptoms, continue to be a medical challenge; unfortunately they can become aggressive tumors associated with distant metastasis, shortening survival. The survival of patients with large nonfunctional DP-NETs is known to be poor, but the overall contribution of DP-NETs to metastatic spread is poorly known. Read More

J Clin Endocrinol Metab 2019 Apr;104(4):1336-1344

Department of Internal Medicine, Sector of Endocrinology, Erasmus Medical Center and Erasmus MC Cancer Institute, Rotterdam, Netherlands.

Purpose: Peptide receptor radionuclide therapy (PRRT) with the radiolabeled somatostatin analogue [Lutetium-177-DOTA0-Tyr3]octreotate (177Lu-DOTATATE) is widely applied for inoperable metastatic small intestinal and nonfunctioning pancreatic neuroendocrine tumors (pNETs). The aim of this study is to describe the safety and efficacy of the treatment of functioning pNETs.

Methods: Patients were treated with up to four cycles of 177Lu-DOTATATE with an intended dose of 7. Read More

Pancreas 2019 01;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

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

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

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.

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

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

J Visc Surg 2019 Feb 27;156(1):69-71. 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:

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

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

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

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

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

Expert Rev Anticancer Ther 2018 09 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

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

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

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

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

Chirurg 2018 Jun;89(6):479

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

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

Endocrinol Diabetes Nutr 2018 May 11;65(5):255-264. Epub 2018 Apr 11.

Servicio de Endocrinología y Nutrición, Hospital Universitario Puerta de Hierro, Majadahonda, Madrid, España.

Introduction: Ectopic Cushing's syndrome (ECS) is a rare condition caused by ACTH secretion by extrapituitary tumors. Its low frequency makes it difficult to acquire experience in its management. The aim of this study was to describe patients with ECS seen at the endocrinology department of a tertiary hospital over 15 years. Read More

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

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

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

Rev Endocr Metab Disord 2018 06;19(2):145-158

Department of Internal Medicine IV, University-Hospital Campus Grosshadern, Ludwig-Maximilian University of Munich, Munich, Germany.

Neuroendocrine tumors (NETs) are a group of rare and heterogeneous malignancies that can develop in various organs. A significant number of gastroenteropancreatic neuroendocrine tumours (GEP-NETs) is functionally active and presents with symptoms related to the secretion of biologically active substances, leading to the development of distinct clinical syndromes. There are various therapeutic approaches for GEP-NETs, including curative surgery, palliative surgery, local-ablative and loco-regional therapies as well as systemic therapeutic options including peptide receptor radionuclide therapy, cytotoxic therapy, and molecularly targeted therapies. Read More

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

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

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.).

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

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

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

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

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

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

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:

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

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

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

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

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

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