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

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

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

Refractory peptic ulcers are defined as ulcers that do not heal completely after 8 to 12 weeks of standard anti-secretory drug treatment. The most common causes of refractory ulcers are persistent Helicobacter pylori infection and use of nonsteroidal anti-inflammatory drugs (NSAIDs). Simultaneous use of two or more H. pylori diagnostic methods are recommended for increased sensitivity. Serologic tests may be useful for patients currently taking proton pump inhibitors (PPIs) or for suspected false negative results, as they are not affected by PPI use. NSAID use should be discontinued when possible. Platelet cyclooxygenase activity tests can confirm surreptitious use of NSAIDs or aspirin. Cigarette smoking can delay ulcer healing. Therefore, patients who smoke should be encouraged to quit. Zollinger-Ellison syndrome (ZES) is a rare but important cause of refractory gastroduodenal ulcers. Fasting plasma gastrin levels should be checked if ZES is suspected. If an ulcer is refractory despite a full course of standard PPI treatment, the dose should be doubled and administration of another type of PPI considered.
Anti-Inflammatory Agents, Non-Steroidal ; Aspirin ; Blood Platelets ; Fasting ; Gastrins ; Helicobacter pylori ; Humans ; Peptic Ulcer* ; Plasma ; Prostaglandin-Endoperoxide Synthases ; Proton Pump Inhibitors ; Serologic Tests ; Smoke ; Smoking ; Ulcer ; Zollinger-Ellison Syndrome

Zollinger-Ellison syndrome (ZES) is characterized by gastrinoma and resultant hypergastrinemia, which leads to recurrent peptic ulcers. Because gastrinoma is the most common pancreatic endocrine tumor seen in multiple endocrine neoplasia type I (MEN 1), the possibility of gastrinoma should be investigated carefully when patients exhibit symptoms associated with hormonal changes. Ureteral stones associated with hyperparathyroidism in the early course of MEN 1 are known to be its most common clinical manifestation; appropriate evaluation and close follow-up of patients with hypercalcemic urolithiasis can lead to an early diagnosis of gastrinoma. We report a patient with ZES associated with MEN 1, and urolithiasis as the presenting entity. A 51-year-old man visited the emergency department with recurrent epigastric pain. He had a history of calcium urinary stone 3 years ago, and 2 years later he had 2 operations for multiple jejunal ulcer perforations; these surgeries were 9 months apart. He was taking intermittent courses of antiulcer medication. Multiple peripancreatic nodular masses, a hepatic metastasis, parathyroid hyperplasia, and a pituitary microadenoma were confirmed by multimodal imaging studies. We diagnosed ZES with MEN 1 and performed sequential surgical excision of the gastrinomas and the parathyroid adenoma. The patient received octreotide injection therapy and close follow-up.
Gastrinoma/metabolism/pathology/ultrasonography ; Gastrins/metabolism ; Humans ; Immunohistochemistry ; Liver/radiography ; Magnetic Resonance Imaging ; Male ; Mesenteric Artery, Superior/radiography ; Middle Aged ; Multimodal Imaging ; Multiple Endocrine Neoplasia Type 1/complications/*diagnosis/radiography ; Pancreas/radiography/radionuclide imaging ; Pituitary Gland/radiography ; Positron-Emission Tomography ; Radiopharmaceuticals/diagnostic use ; Thyroid Gland/ultrasonography ; Tomography, X-Ray Computed ; Urolithiasis/*diagnosis/etiology ; Zollinger-Ellison Syndrome/complications/*diagnosis

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

Zollinger-Ellison syndrome (ZES) is a clinical syndrome caused by excessive gastric acid secretion by gastrinoma, characteristically causing peptic disease and/or gastroesophageal reflux disease. Approximately one third of patients with gastrinoma have multiple endocrine neoplasia type 1 (MEN-1). A 56-year-old man was admitted for abdominal pain and diarrhea lasting for 2 weeks. The endoscopic findings revealed severe reflux esophagitis and multiple ulcers at the bulb and second portion of the duodenum. He was diagnosed as ZES based on typical clinical features such as markedly elevated fasting gastrin level (> or =1,263 pg/mL) and findings from a CT scan and somatostatin receptor scan. Pathologic findings after the operation revealed malignant gastrinoma. He was confirmed to have parathyroid adenoma and MEN-1. Despite antisecretory therapy with proton pump inhibitors, an esophageal stricture developed, and we performed esophageal balloon dilatation and stent insertion.
Abdominal Pain ; Diarrhea ; Dilatation ; Duodenum ; Esophageal Stenosis ; Esophagitis, Peptic ; Fasting ; Gastric Acid ; Gastrinoma ; Gastrins ; Gastroesophageal Reflux ; Humans ; Middle Aged ; Multiple Endocrine Neoplasia Type 1 ; Parathyroid Neoplasms ; Proton Pump Inhibitors ; Receptors, Somatostatin ; Stents ; 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*

Isolated obstruction of the splenic vein leads to segmental portal hypertension, which is mainly originated from pancreatic disease, such as chronic pancreatitis, pancreatic pseudocyst, pancreatic cancer. The clinical manifestation are devoid of ascites and encephalopathy, but presented with gastric variceal bleeding. We experienced 27-year-old male patient who complained of hematochezia and melena. After work-up with CT and angiography, we performed operation with the impression of pancreatic tumor associated with splenic vein occlusion. It resulted nonfunctioning pancreatic endocrine tumor. Often called islet cell tumor, pancreatic endocrine tumor has various names according to secreted hormones, such as insulinoma, Zollinger-Ellison syndrome and glucagonoma. It was classified as nonfunctioning tumor if there were any evidence of hormone secretion by radioimmunoassay and immunohistochemistry. Surgical excision and adjuvant medical therapy is the mainstay of therapy. We performed tumor resection and splenectomy for control of gastric variceal bleeding due to splenic vein occlusion.
Adenoma, Islet Cell ; Adult ; Angiography ; Ascites ; Esophageal and Gastric Varices* ; Gastrointestinal Hemorrhage ; Glucagonoma ; Humans ; Hypertension, Portal ; Immunohistochemistry ; Insulinoma ; Male ; Melena ; Pancreas* ; Pancreatic Diseases ; Pancreatic Neoplasms ; Pancreatic Pseudocyst ; Pancreatitis, Chronic ; Radioimmunoassay ; Splenectomy ; Splenic Vein ; 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