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Cancers (Basel) 2022 Apr 11;14(8). Epub 2022 Apr 11.

Department of Surgery, Philipps-University, 35041 Marburg, Germany.

Aim: The aim of this research was to evaluate the long-term outcome of pancreaticoduodenectomy (PD) versus other duodenopancreatic resections (non-PD) for the surgical treatment of the Zollinger-Ellison syndrome (ZES) in patients with multiple endocrine neoplasia type 1 (MEN1).

Methods: Prospectively recorded patients with biochemically confirmed MEN1-ZES who underwent duodenopancreatic surgery were retrospectively analyzed in terms of clinical characteristics, complications, cure rate, and long-term morbidity, including quality of life assessment (EORTC QLQ-C30).

Results: 35 patients (16 female, 19 male) with MEN1-ZES due to duodenopancreatic gastrinomas with a median age of 42 (range 30-74) years were included. Read More

Am J Case Rep 2022 Apr 8;23:e936029. Epub 2022 Apr 8.

Department of Surgery, King Abdulaziz Medical City, Jeddah, Saudi Arabia.

BACKGROUND Carcinoid tumors are well-differentiated tumors that develop from neuroendocrine cells. They are rare tumors and occur most commonly in the gastrointestinal tract, followed by the pulmonary system. They usually present with abdominal pain or cough or persistent pneumonia. Read More

Endokrynol Pol 2022 ;73(1):171-172

Department of Endocrinology and Neuroendocrine Tumours, Department of Pathophysiology and Endocrinology, Medical University of Silesia, Katowice, Poland.

Not required for Clinical Vignette. Read More

Function (Oxf) 2022 26;3(1):zqab062. Epub 2021 Nov 26.

Department of Medicine, Division of Gastroenterology and Hepatology, Arizona Comprehensive Cancer Center, University of Arizona, Tucson, AZ 85724, USA.

Abetted by widespread usage of acid-suppressing proton pump inhibitors (PPIs), the mitogenic actions of the peptide hormone gastrin are being revisited as a recurring theme in various gastrointestinal (GI) malignancies. While pathological gastrin levels are intricately linked to hyperplasia of enterochromaffin-like cells leading to carcinoid development, the signaling effects exerted by gastrin on distinct cell types of the gastric mucosa are more nuanced. Indeed, mounting evidence suggests dichotomous roles for gastrin in both promoting and suppressing tumorigenesis. Read More

Biomedicines 2022 Feb 24;10(3). Epub 2022 Feb 24.

Department of Hepatology and Gastroenterology, Charité-Universitätsmedizin Berlin, 13353 Berlin, Germany.

Secretin was originally discovered as a gastrointestinal peptide that stimulates fluid secretion from the pancreas and liver and delays gastric emptying. In disease, a secretin receptor (SCTR) was found to occur as a splice variant in gastrinoma and pancreatic adenocarcinoma. Overexpression of SCTR has been described for gastrinomas, carcinoid tumors of the lung and cholangiocarcinoma. Read More

Hered Cancer Clin Pract 2022 Mar 7;20(1):10. Epub 2022 Mar 7.

Department of General Surgery, Shengjing Hospital of China Medical University, Shenyang, China.

Background: Multiple endocrine neoplasia type 1 (MEN1) is a hereditary cancer syndrome caused by germline variants in the MEN1 gene located on chromosome 11q13. We found a Chinese woman who had a pancreatic tumor, parathyroid tumor, adrenal tumor, and suspicion of gastrinoma.

Case Presentation: The proband and her immediate family members underwent genetic detection. Read More

J Neuroendocrinol 2022 Apr 13;34(4):e13097. Epub 2022 Feb 13.

University of Winchester, Winchester, UK.

Pancreatic neuroendocrine tumours (panNET) are heterogeneous neoplasms usually characterised by slow growth and secretion of hormones, which often cause symptoms. The effect of these symptoms on quality of life (QoL) has not previously been examined in detail. EORTC (European Organisation for Research and Treatment of Cancer) guidelines were followed in phases 1-3 to produce a potential module of questions usable for trials in panNET, focusing on three common types of panNET. Read More

World J Clin Cases 2022 Jan;10(3):1032-1040

Department of Thyroid Surgery, The First Hospital of Jilin University, Changchun 130012, Jilin Province, China.

Background: Multiple endocrine neoplasia type 1 (MEN1) is a rare hereditary tumor syndrome inherited in an autosomal dominant manner and presents mostly as parathyroid, endocrine pancreas (such as gastrinoma) and anterior pituitary tumors. At present, papillary thyroid carcinoma (PTC) and nodular goiter are not regarded as components of MEN1.

Case Summary: A 35-year-old woman presented with MEN1 accompanied by coinstantaneous PTC and nodular goiter. Read More

Cir Cir 2022 ;90(1):109-113

Servicio de Anatomía Patológica, Unidad Médica de Alta Especialidad, IMSS, Puebla, Puebla. México.

The gastrinomas are rare functional neuroendocrine neoplasms, most are localized to the duodenum (70-90%) or the pancreas (2-30%), but less common ectopic sites have been reported. The primary hepatic gastrinoma is extremely rare, with less tan 40 cases reported in the medical literature. Its low incidence and its non specific clinical presentation make it a difficult disease to diagnose. Read More

J Small Anim Pract 2022 Jan 23. Epub 2022 Jan 23.

BluePearl Veterinary Partners, Eden Prairie, MN, 55344, USA.

Gastrinomas are gastrin-secreting pancreatic tumours rarely diagnosed in cats. A 12-year-old female spayed cat was presented for vomiting, anorexia and weight loss. Physical exam revealed lethargy, dehydration and thin body condition. Read More

Medicine (Baltimore) 2022 Jan;101(2):e28550

Pathology Department, University of Medicine, Pharmacy, Science, and Technology of Targu Mures, Romania.

Rationale: Gastric neuroendocrine neoplasms (g-NENs) represent a distinctive group of gastric tumors, stratified into different prognostic categories according to different histological characteristics, put forth in the 2018 World Health Organization classification system. The clinical presentations, as well as pathological features, represent important data in establishing the type of the tumor, in estimating the tumor behavior, and in selecting the best therapeutic strategy. In our case series we presented different clinical scenarios that may be encountered in practice regarding gastric NENs. Read More

Zhonghua Nei Ke Za Zhi 2022 Jan;61(1):116-120

Department of Gastroenterology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.

J Clin Endocrinol Metab 2022 Apr;107(5):e2110-e2123

F.I.R.M.O. Italian Foundation for Research on Bone Diseases, Florence, Italy.

Context: Multiple endocrine neoplasia type 1 (MEN1) is a rare inherited endocrine cancer syndrome. Multiple gastro-entero-pancreatic neuroendocrine tumors (GEP-NETs) affect 30% to 80% of MEN1 patients, with the most common functioning GEP-NET being gastrinoma. Biochemical identification of hypergastrinemia may help to recognize the presence of gastrinomas before they are detectable by instrumental screening, enabling early diagnosis and start of therapy, preferably before tumor progression and metastases occurrence. Read More

Front Endocrinol (Lausanne) 2021 18;12:741887. Epub 2021 Nov 18.

Institute of Systems, Molecular and Integrative Biology, University of Liverpool, Liverpool, United Kingdom.

Background: Hypergastrinaemia occasionally indicates the presence of a gastrinoma. However it is much more commonly associated with various benign causes including proton pump inhibitor (PPI) use, infection and/or atrophic gastritis. The extent to which these factors interact to influence fasting serum gastrin concentrations remains incompletely understood. Read More

Gastrointest Endosc 2022 Mar 17;95(3):589-590. Epub 2021 Nov 17.

Department of Gastroenterology and Hepatology, Maadi Armed Forces Medical Complex, Military Medical Academy, Cairo, Egypt.

Indian J Thorac Cardiovasc Surg 2021 Nov 9;37(6):706-709. Epub 2021 Jun 9.

General Surgery Ward, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran.

Gastrinomas are located largely in the pancreaticoduodenal region. However, gastrinomas have also been found in non-pancreaticoduodenal regions. Our study is a rare report of gastrinomas within the thoracic cavity. Read More

BMJ Open Gastroenterol 2021 11;8(1)

Department of Medicine, University of Arizona Medical Center - University Campus, Tucson, Arizona, USA

Objective: Gastroenteropancreatic neuroendocrine tumours (GEP-NETs) encompass a diverse group of neoplasms that vary in their secretory products and in their location within the gastrointestinal tract. Their prevalence in the USA is increasing among all adult age groups.

Aim: To identify the possible derivation of GEP-NETs using genome-wide analyses to distinguish small intestinal neuroendocrine tumours, specifically duodenal gastrinomas (DGASTs), from pancreatic neuroendocrine tumours. Read More

Endocrine 2022 Mar 29;75(3):942-948. Epub 2021 Oct 29.

Division of Gastroenterology, Department of Medicine and Surgery, University of Milano-Bicocca, San Gerardo School of Medicine, Monza, Italy.

Purpose: Zollinger Ellison syndrome (ZES) is a rare syndrome caused by gastrin hypersecretion from a gastrinoma. Gastrinoma treatment has two goals: the control of acid hypersecretion and the control of tumor growth. While therapy for the syndrome is univocally based on proton pump inhibitors, the one for disease control is still debated. Read More

Zhonghua Wei Chang Wai Ke Za Zhi 2021 Oct;24(10):875-882

Department of Gastroenterology, the First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, China Chen Jie now is working at the Center for Neuroendocrine Tumors, Fudan University Shanghai Cancer Center, Department of Oncology, Shanghai Medical College, Fudan University, Shanghai Pancreatic Cancer Institute, Pancreatic Cancer Institute, Fudan University, Shanghai 200032, China.

To compare clinical characteristics of sporadic gastrinoma and multiple endocrine neoplasia type 1 (MEN1)-related gastrinoma. A retrospective cohort study was conducted. Patients with clinical manifestations of Zollinger-Ellison syndrome, pathological diagnosis as neuroendocrine neoplasm (NEN) and complete clinical and follow-up data were enrolled. Read More

Zhonghua Wei Chang Wai Ke Za Zhi 2021 Oct;24(10):849-853

Department of Gastric Surgery, State Key Laboratory of Oncology in South China, Collaborative Innovation Center of Cancer Medicine, Sun Yat-sen University Cancer Center, Guangzhou 510060, China.

Neuroendocrine neoplasms (NEN) are rare neoplasms originating from all major systems, in which gastric neuroendocrine neoplasms (G-NEN) is rarely malignant neoplasm originated in stomach. In 2019, the 5th WHO classification of digestive system tumors updated the classification of G-NEN and solved several naming problems. Since the classification of G-NEN has become more specific and more scientific, the surgical treatment of G-NEN is becoming more individual and more precise. Read More

World J Gastroenterol 2021 Sep;27(35):5890-5907

Division of Gastroenterology and Center for Autoimmune Liver Diseases, Department of Medicine and Surgery, University of Milano-Bicocca, Monza 20900, Italy.

Zollinger-Ellison syndrome (ZES) associated with pancreatic or duodenal gastrinoma is characterized by gastric acid hypersecretion, which typically leads to gastroesophageal reflux disease, recurrent peptic ulcers, and chronic diarrhea. As symptoms of ZES are nonspecific and overlap with other gastrointestinal disorders, the diagnosis is often delayed with an average time between the onset of symptoms and final diagnosis longer than 5 years. The critical step for the diagnosis of ZES is represented by the initial clinical suspicion. Read More

Endocr J 2022 Mar 9;69(3):243-251. Epub 2021 Oct 9.

Department of Endocrinology, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, Anhui Province, 230001, China.

Pancreatic neuroendocrine tumors (P-NETs) secreting ectopic adrenocorticotropic hormones (ACTH) are rare and often delayed in diagnosis due to their atypical clinical characteristics. Here, we describe a case of P-NET in the pancreatic tail. The tumor had metastasized to the liver and secreted gastrin and ACTH. Read More

World J Clin Cases 2021 Sep;9(27):7973-7985

Department of Gastroenterology, Hitit University, Faculty of Medicine, Çorum 19200, Turkey.

Gastric neuroendocrine neoplasms (g-NENs) or neuroendocrine tumors are generally slow-growing tumors with increasing incidence. They arise from enterochromaffin like cells and are divided into four types according to clinical characteristic features. Type 1 and 2 are gastrin dependent, whereas type 3 and 4 are sporadic. Read More

World J Gastroenterol 2021 Aug;27(32):5322-5340

Department of Gastroenterology and Hepatology, "Grigore T. Popa" University of Medicine and Pharmacy, Iasi 700115, Romania.

The presence of pancreatic cancer during childhood is extremely rare, and physicians may be tempted to overlook this diagnosis based on age criteria. However, there are primary malignant pancreatic tumors encountered in pediatric patients, such as pancreatoblastoma, and tumors considered benign in general but may present a malignant potential, such as the solid pseudo-papillary tumor, insulinoma, gastrinoma, and vasoactive intestinal peptide secreting tumor. Their early diagnosis and management are of paramount importance since the survival rates tend to differ for various types of these conditions. Read More

Am J Gastroenterol 2021 11;116(11):2216-2221

Digestive Disease Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, USA.

Introduction: Zollinger-Ellison syndrome (ZES) is characterized by gastrinoma-induced hypergastrinemia causing excessive gastric acid secretion. Secretin stimulation tests (SSTs) are required for diagnosis in the majority of patients. Two case reports suggest that proton pump inhibitors (PPIs) cause false SST results. Read More

J Gastrointest Surg 2021 12 10;25(12):3244-3257. Epub 2021 Sep 10.

Department of Nuclear Medicine, The Christie Hospital NHS Foundation Trust, Manchester, UK.

Background: Radioguided surgery (RGS) for gastroenteropancreatic neuroendocrine tumours (GEP-NETs) has been suggested as a way to improve intraoperative lesion detection. This systematic literature review of reports of the use of RGS for GEP-NETs was performed to determine if there is a benefit.

Methods: A literature search was conducted using Google Scholar and PubMed, and snowballing from any relevant literature. Read More

Cureus 2021 Jul 23;13(7):e16593. Epub 2021 Jul 23.

Gastroenterology, United Lincolnshire Hospitals National Health Service (NHS) Trust, Boston, GBR.

Gastrinoma or otherwise known as Zollinger-Ellison syndrome is characterised by hypersecretion of gastrin and gastric acid leading to the formation of recurrent atypical ulcers along the upper gastrointestinal tract. It is extremely difficult to diagnose during an acute presentation both due to its rarity and its lack of pathognomonic symptoms. Its symptoms range from mild to severe to life-threatening and often get mistaken for a different condition such as viral gastroenteritis as seen in our case report. Read More

Clin Chem 2021 09;67(9):1220-1229

Department of Clinical Laboratory, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China.

Background: Two major forms of gastrin, gastrin-17 (G17) and gastrin-34 (G34), exist in blood. However, conventional immunoassay methods can only quantify total gastrin or G17 alone. Here, we aimed to establish a liquid chromatography-tandem mass spectrometry (LC-MS/MS) method to quantify G17 and G34 simultaneously. Read More

Quant Imaging Med Surg 2021 Aug;11(8):3898-3903

Department of Radiology, Wakayama Medical University, Wakayama, Japan.