Zollinger-Ellison syndrome (ZES) involves hypergastinemia produced by a gastrin-secreting tumor. Not only can it cause an ulcer but may also behave as a malignant lesion, metastasizing to the liver or other organs. The development of potent antisecretory agents for controlling acid secretion as well as techniques for localizing these islet cell tumors, has led to greatly improved survival rates. We describe a case of Zollinger-Ellison syndrome, emphasising the radiologic findings, and including a review of the literature.
Adenoma, Islet Cell ; Liver ; Survival Rate ; Ulcer ; Zollinger-Ellison Syndrome*

In patient with Zollinger-Ellison syndrome, it is difficult to localize gastrinoma because the tumor is frequently small and multiple. However, accurate localization of the tumor is important for the treatment. Among various imaging modalities, somatostatin receptor scintigraphy (SRS) has been recognized to be the most sensitive tool for the detection of neuroendocrine tumors such as gastrinomas based on the presence of high-affinity binding sites for somatostatin. Recently, we experienced a case of Zollinger-Ellison syndrome caused by gastrinomas which was localized by SRS. This is the first case report of gastrinoma detected by SRS in Korea. SRS can facilitate tumor detection in patient with Zollinger-Ellison syndrome and should be considered as the first-line diagnostic method in the early course of the disease.
Binding Sites ; Gastrinoma* ; Humans ; Korea ; Neuroendocrine Tumors ; Radionuclide Imaging ; Receptors, Somatostatin ; Somatostatin ; Zollinger-Ellison Syndrome*

Atypical thymic carcinoid is an extremely rare tumor with a poor prognosis. In addition to its known association with multiple endocrine neoplasia type 1, its hallmark characteristics include local invasion and early distant metastasis. In this report, we share our experience treating atypical thymic carcinoid in a patient with Zollinger-Ellison syndrome.
Carcinoid Tumor ; Humans ; Multiple Endocrine Neoplasia Type 1 ; Neoplasm Metastasis ; Neuroendocrine Tumors ; Prognosis ; Zollinger-Ellison Syndrome

Multiple carcinoid tumors in stomach are very rare tumors and associated with pernicious anemia, chronic atrophic gastritis and Zollinger-Ellison syndrome. These are believed to be due to hypergastrinemia, resulting in chronic stimulation of enterochromaffine-like (ECL) cells, leading to hyperplasia, metaplasia and ultimately neoplasia. The carcinoid tumor is potentially malignant, even though it may grow slowly, so the treatment of choice must be endoscopic lumpectomy or surgical gastrectomy. The prognosis depends on the site of the tumor and its size. Most carcinoid tumors are less than 1 cm in size and metastasis is uncommon but metastasis in tumors with size over 2 cm is common. A case of multiple carcinoid tumors in stomach, which was successfully removed by endoscopy, is herein reported.
Anemia, Pernicious ; Carcinoid Tumor* ; Endoscopy ; Gastrectomy ; Gastrins ; Gastritis, Atrophic ; Hyperplasia ; Mastectomy, Segmental ; Metaplasia ; Neoplasm Metastasis ; Prognosis ; Stomach* ; Zollinger-Ellison Syndrome

Gastric carcinoid tumors are so rare that they constitute less than 1% of total primary gastric tumors. They are classified into 3 types: (1) gastric carcionoid associated with chronic atrophic gastritis (type I), (2) gastric carcinoids associated with Zollinger-Ellison syndrome (ZES) or multiple endocrine neoplasm (MEN) type 1 (type II), and (3) sporadic gastric carcinoid (type III). A 55-year-old man underwent a medical check-up. Five polypoid masses were incidentally detected in the cardia, fundus and midbody whose size ranged from 0.5 cm to 1.6 cm in diameter. Tumor cells were strongly positive for cytokeratin, synaptophysin and chromogranin staining. Biopsies from the body and fundus showed severe atrophy. Fasting serum gastrin level was highly increased, 1,098 pg/mL. There was, however, no evidence of the presence of ZES or MEN type 1. The patient underwent total gastrectomy for complete resection and has remained free of any complications with normal serum gastrin level.
Atrophy ; Biopsy ; Carcinoid Tumor* ; Cardia ; Fasting ; Gastrectomy ; Gastrins ; Gastritis, Atrophic* ; Humans ; Keratins ; Male ; Middle Aged ; Synaptophysin ; Zollinger-Ellison Syndrome

A 34-year-old man was referred to our hospital with gastric polypoid lesions and biopsy-confirmed neuroendocrine tumor (NET). Computed tomography (CT) revealed a 3×3.5×8-cm retroperitoneal mass behind the pancreas, with multiple hepatic metastases. His serum gastrin level was elevated to 1,396 pg/mL. We performed a wedge resection of the stomach, a right hemi-hepatectomy, and a retroperitoneal mass excision. After careful review of the clinical, radiological, histopathological, and immunohistochemical findings, peripancreatic gastrinoma, and synchronous gastric NET were ultimately diagnosed. We reviewed a CT scan that had been performed 6 years previously after surgery for a duodenal perforation. There was no evidence of gastric or hepatic lesions, but the retroperitoneal mass was present at the same site. Had gastrinoma been detected earlier, our patient could have been cured using less invasive treatment. This case demonstrates how important it is to consider Zollinger-Ellison syndrome in patients with a recurrent or aggressive ulcer.
Adult ; Gastrinoma* ; Gastrins ; Humans ; Lymph Nodes* ; Neoplasm Metastasis ; Neuroendocrine Tumors* ; Pancreas ; Stomach ; Tomography, X-Ray Computed ; Ulcer ; Zollinger-Ellison Syndrome

Zollinger-Ellison syndrome is a very rare disease that's caused by tumor having gastrin-producing cells, and this is accompanied by hypergastrinemia leading to gastric acid hypersecretion, peptic ulcer and their complications. A 60-year-old man presented with epigastric pain and soreness he had experienced for 7 days. The endoscopic findings showed reflux esophagitis and multiple active ulcers at the antrum, the duodenal bulb and the 2nd and 3rd portions of the duodenum. The fasting serum gastrin level was markedly elevated above 1,470 pg/mL and this was consistent with the findings of gastrinoma. We confirmed the presence of gastrinoma via the abdomen CT scan and selective intra-arterial calcium injection. This is the first Korean case report of gastrinoma that was localized by selective intra-arterial calcium injection.
Abdomen ; Calcium* ; Duodenum ; Esophagitis, Peptic ; Fasting ; Gastric Acid ; Gastrinoma* ; Gastrins ; Humans ; Middle Aged ; Peptic Ulcer ; Rare Diseases ; Tomography, X-Ray Computed ; Ulcer ; Zollinger-Ellison Syndrome*

The first histamine H₂ receptor antagonists (H₂RAs) were developed in the early 1970s. They played a dominant role in treating peptic ulcer disease and gastroesophageal reflux disease (GERD). H₂RAs block the production of acid by H⁺, K⁺-ATPase at the parietal cells and produce gastric luminal anacidity for varying periods. H₂RAs are highly selective, and they do not affect H₁ receptors. Moreover, they are not anticholinergic agents. Sequential development of H₂RAs, proton pump inhibitors (PPIs), and discovery of Helicobacter pylori infection changed the paradigm of peptic ulcer disease with marked decrease of morbidity and mortality. PPIs are known to be the most effective drugs that are currently available for suppressing gastric acid secretion. Many studies have shown its superiority over H₂RAs as a treatment for acid-related disorders, such as peptic ulcer disease, GERD, and Zollinger-Ellison syndrome. However, other studies have reported that PPIs may not be able to render stomach achlorhydric and have identified a phenomenon of increasing gastric acidity at night in individuals receiving a PPI twice daily. These nocturnal acid breakthrough episodes can be eliminated with an addition of H₂RAs at night. The effectiveness of nighttime dose of H₂RA suggests a major role of histamine in nocturnal acid secretion. H₂RAs reduce secretion of gastric acid, and each H₂RA also has specific effects. For instance, nizitidine alleviates not only symptoms of GERD, but also provokes gastric emptying, resulting in clinical symptom improvement of functional dyspepsia. The aim of this paper was to review the characteristics and role of H₂RAs and assess the future strategy and treatment of upper gastrointestinal disease, including acid related disorders.
Cholinergic Antagonists ; Dyspepsia ; Gastric Acid ; Gastric Emptying ; Gastroesophageal Reflux ; Gastrointestinal Diseases ; Helicobacter pylori ; Histamine ; Mortality ; Peptic Ulcer ; Phenobarbital ; Proton Pump Inhibitors ; Rabeprazole ; Stomach ; Treatment Outcome* ; Zollinger-Ellison Syndrome

Gastric neuroendocrine tumor (GNET) is rare, but increasing in incidence. GNET may be classified into three types on the basis of the background pathology. Type I GNET is related to autoimmune atrophic gastritis and hypergastrinemia. Type II is related to multiple endocrine neoplasia (MEN)-1, Zollinger-Ellison syndrome and hypergastrinemia and sporadic Type III is not related to any background pathology. Type I GNETs can be considered as benign tumors with unusual metastases. However, type II may be related to distant metastases, which is also common in type III GNETs. Type I and type II lesions can be treated by endoscopic excision or somatostatin analogues whereas surgical treatment should be considered for type III lesions. Hypergastrinemia is an essential precondition for the evolution of type I and II lesions, but hypergastrinemia alone is not enough for explanation of tumorigenesis. Furthermore, the pathogenesis of type III neuroendocrine tumors is still poorly understood. Despite improvements in our knowledge of GNET pathogenesis in diagnostic approach and treatment, further investigations and large scale clinical studies are warranted.
Cell Transformation, Neoplastic ; Gastritis, Atrophic ; Incidence ; Multiple Endocrine Neoplasia ; Multiple Endocrine Neoplasia Type 1 ; Neoplasm Metastasis ; Neuroendocrine Tumors ; Somatostatin ; Upper Gastrointestinal Tract ; Zollinger-Ellison Syndrome

Gasritis cystica profunda (GCP) is a rare disease in which hyperplasia of mature glandular epithelium extends into the tissues beneath the submucosa. It shows multiple small cysts in the mucosa and submucosa of the stomach. It was firstly reported by Littler and Gleibermann on 1972. GCP is mainly observed at the site of gastroenterostomy but, it may occur in the stomach without a previous history of surgery. The proposed pathogenesis of the these abnormalities are related to ischemia, chronic inflammation and the presence of a foreign body. GCP may present not only as a submucosal tumor or as solitary or diffuse polyps but also as a giant gastric mucosal fold rarely. It should be differentiated from Menetrier's disease, Zollinger-Ellison syndrome, inflammatory disease and malignancy. We present a case of gastritis cystica profunda without having had any previous surgery, suspiciously caused by gastric foreign body. We made a diagnosis based on findings from the esophagogastroduodenoscopy, endoscopic ultrasonography and histologic findings after surgery.
Diagnosis ; Endoscopy, Digestive System ; Endosonography ; Epithelium ; Foreign Bodies* ; Gastritis* ; Gastritis, Hypertrophic ; Gastroenterostomy ; Hyperplasia ; Inflammation ; Ischemia ; Mucous Membrane ; Polyps ; Rare Diseases ; Stomach ; Zollinger-Ellison Syndrome