Sporadic duodenal macrogastrinoma: a rare case report (original) (raw)
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De novo Gastrinoma: A Case Report
GE - Portuguese Journal of Gastroenterology, 2019
Gastrinomas are neuroendocrine tumors characterized by gastrin overexpression – 80% are sporadic and 20% are associated with multiple endocrine neoplasia type 1. A 75-year-old male patient, surgically treated at the age of 50 years for gastrinoma, followed on an outpatient basis because of chronic non-bloody diarrhea, was admitted to our hospital because of abdominal pain, watery diarrhea, and nonbiliary vomits. He was hypotensive and showed no response to fluids. Blood cultures were positive for Salmonella, and a diagnosis of septic shock due to Salmonella infection was made. The patient’s condition improved, but the history of chronic diarrhea was still not explained. To investigate chronic diarrhea, gastrinoma recurrence was considered. Serum gastrin measurement was five times higher than the upper limit of the normal range (536 pg/mL). A positive somatostatin receptor scintigraphy was diagnostic for neuroendocrine tumor. Metastases were excluded. The patient was proposed to cura...
Journal of Medical Case Reports, 2013
Introduction: Zollinger-Ellison syndrome is characterized by recurrent peptic ulcers and diarrhea that result from gastrin-secreting neuroendocrine tumors of the gastrointestinal tract; nevertheless, severe hypergastrinemia may also have alternative pathogenetic explanations. Case presentation: A 61-year-old woman of Caucasian origin presented with a history of epigastric pain and early satiety, severe hypergastrinemia (approximately 2000 pg/mL) and a neuroendocrine polyp in the corpus of her stomach. Chronic atrophic gastritis and intestinal metaplasia was present, but she denied use of acid suppressant drugs and the results of tests for Helicobacter pylori as well as gastric parietal cell and intrinsic factor antibodies were negative. She underwent a radical gastric tangential resection. Six months later, serum gastrin was still elevated despite lack of recurrence of tumor. Conclusion: The clinical picture was suggestive for a hypochlorhydria-related hypergastrinemia with subsequent development of a non-secreting carcinoid. We suggest a periodic endoscopic follow-up in patients with severe hypochlorhydria-related hypergastrinemia in order to earlier detect neuroendocrine polyps.
Sporadic Duodenal Gastrinoma causing Zollinger Ellison Syndrome – A Rare Case Report
International Journal of Contemporary Medical Research [IJCMR], 2019
Introduction: Gastrinoma is a rare gastrin secreting neuroendocrine tumor. Clinical manifestations are nonspecific gastrointestinal (GI) symptoms or as Zollinger Ellison Syndrome(ZES) which often delays the diagnosis in majority of patients. Hence a high clinical index of suspicion is warranted in patients who present with nonspecific GI symptoms to rule out gastrinoma. Case report: We report a case of 55 year old female who presented with long standing dyspepsia and chronic diarrhoea and was diagnosed with Sporadic Duodenal Gastrinoma on detailed evaluation. She was treated with surgical resection and pathologically diagnosed as well differentiated neuroendocrine tumor, Gastrinoma-grade I after IHC analysis. Patient was relieved of her symptoms and became eugastrinemic after surgery. Conclusion: Any patient with refractory dyspepsia associated with long standing non specific abdominal symptoms should be evaluated for Gastrinoma and treated accordingly to avoid misdiagnosis and complications in the long run.
Management and Outcome of Patients With Sporadic Gastrinoma Arising in the Duodenum
Annals of Surgery, 2003
Background: Primary duodenal gastrinomas are now recognized as a common etiology for patients with sporadic Zollinger Ellison Syndrome (ZES); however, the clinical and pathologic features of this condition and long-term outcome after operation are not well characterized. Methods: Between November 1982 and September 2000, 63 patients diagnosed with sporadic ZES underwent resection of a primary duodenal gastrinoma and regional nodal metastases with curative intent. Data from a prospectively maintained database were reviewed for clinical and pathologic parameters relating to primary tumor size, location, frequency of lymph node metastases, and disease-specific and disease-free survival. Results: There were 41 males and 22 females (mean age, 48.6 years). The majority of duodenal gastrinomas were in the first or second portions of the duodenum (83%). Tumor size ranged from 0.2 to 2.0 cm with 62% measuring less than 1.0 cm. Sixty percent of individuals had regional lymph node metastases identified primarily in proximity to the primary tumor. At a median 10-year follow-up, the overall disease-specific and disease-free survivals were 100% and 60%, respectively. Actuarial 10-year disease-free survival was significantly higher for patients without lymph node metastases versus those with lymph node metastases (78% versus 48%, P ϭ 0.0137). Conclusions: Duodenal gastrinomas in patients with sporadic ZES are frequently small, most commonly located in the proximal duodenum, and associated with regional lymph node metastases in 60%. Disease-free survival is lower for patients with regional lymph node metastases suggesting that a more systematic lymphadenectomy to extirpate occult disease may be indicated in this group.
Gastrinoma (Duodenal and Pancreatic)
Neuroendocrinology, 2006
The incidence of gastrinomas is 0.5-3/million population/ year. They are the most common functional and, malignant pancreatic endocrine tumor (PET) syndrome and comprise up to 30% of these tumors . Duodenal tumors, which were originally thought to be uncommon (i.e. ! 20%), now make up 50-88% of gastrinomas in sporadic ZES patients and 70-100% of gastrinomas in MEN1/ZES patients. In rare cases, gastrinomas occur in other nonpancreatic, nonduodenal abdominal (stomach, liver, bile duct, ovary) (5-15%) and extra-abdominal (heart, small cell lung cancer) locations .
Cancer Control, 1997
Management of the primary gastrinoma has replaced the focus on control of gastric ulceration. Background: The Zollinger-Ellison syndrome, implicating a gastrinoma, was first recognized as a disease entity in 1955. At that time, total gastrectomy was the most common treatment approach. Advances in several aspects of the disease have occurred since that time. Methods: The authors reviewed the changes that have developed since 1955 in the diagnosis, imaging studies, operative and nonoperative management, and follow-up of patients with this disease. Results: The presence of a gastrinoma can be confirmed by a secretin stimulation test. A variable number of patients have hyperparathyroidism as part of the multiple endocrine neoplasm syndrome type 1 (MEN 1). Localization of the primary gastrinoma has been assisted by selective angiography, endoscopic ultrasonography, and the octreotide scan. H2-blockers or omeprazole, sometimes at high doses, usually controls acid secretion. Surgical removal of the primary gastrinoma is performed when feasible, and parathyroidectomy is indicated in those patients with hyperparathyroidism in the MEN 1 syndrome. Follow-up is facilitated by measurement of fasting serum gastrin levels. Conclusions: Several innovations have improved our capability to diagnose and effectively manage patients with gastrinoma.
Endokrynologia Polska
This article is available in open access under Creative Common Attribution-Non-Commercial-No Derivatives 4.0 International (CC BY-NC-ND 4.0) license, allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially Guidelines Grażyna Rydzewska et al. Guidelines in the World Health Organization (WHO) and North American Neuroendocrine Tumor Society (NANETS) recommendations [12, 13] (Tab. 1). 1.1.1. Pathogenesis Type 1 and type 2 tumours develop from enterochromaffin-like (ECL) cells in the gastric mucosa in response to chronic oversecretion of gastrin. Secondary hypergastrinaemia-caused by achlorhydria in the course of chronic atrophic gastritis (CAG)-is responsible for the development of GNEN type 1. Primary hypergastrinaemia-in Zollinger-Ellison syndrome (ZES), sporadic or associated with multiple endocrine neoplasia 1 (MEN-1)-is responsible for GNEN type 2. Gastrin and its derivatives stimulate the proliferation, migration, and differentiation of ECL cells, which in turn leads to their hyperplasia and dysplasia. Hypergastrinaemia, without the interaction of the transforming factor/factors, does not cause the development of GNEN [4]. Menin dysfunction may constitute a transforming factor in patients with MEN-1. The literature also mentions other factors including the following: BCL2 apoptosis inhibiting protein, p53 protein, fibroblast growth factor (FGF), transforming growth factor (TGF), Regla protein dysfunction (inhibiting the proliferation of ECL cells) [14]. 1.1.2. Type 1 Gastric neuroendocrine neoplasms type 1 (70-80% GNEN) occur in patients with atrophic gastritis. They
An atypical type I gastric neuroendocrine tumor
Romanian Journal of Internal Medicine
Gastric neuroendocrine tumors(GI-NETs) are rare lesions, usually discovered incidentally during endoscopy. Based on their pathology, there are 4 types of GI-NETs. Type I are multiple small polypoid lesions with central ulceration located in the gastric body or the fundus, associated with atrophic gastritis usually noninvasive and very rarely metastatic. We report on a rare case of a gastric NET arising from the muscularis propria layer of the pyloric ring.We present the case of a 65-year old woman with a history of alcoholic cirrhosis, investigated for melena. Upper endoscopy revealed a 30 mm submucosal pedunculated polypoid lesion located on the pylorus protruding in the duodenum, with normal overlying mucosa, fundic gastric atrophy and multiple small polyps at this level, with no active bleeding. CT scan did not reveal any distant metastases. An ultrasound endoscopy was performed, and a round hypoechoic heterogeneous solitary mass, evolving from the pyloric muscle was described. C...
Unusual Presentation of a Neuroendocrine Tumor of Stomach as an Ulcer
Journal of case reports, 2021
Background: Gastric neuroendocrine tumors (G-NETs) originate from the enterochromaffin like (ECL) cells of the gastric mucosa and form less than 1% of all gastric tumors. These present as single to multiple polyps on endoscopy. Case Report: A 52-year-old female patient found to have an ulcer on esophagogastroduodenoscopy (EGD) which was reported as chronic gastritis on biopsy. She underwent distal gastrectomy with Billroth II anastomosis due to suspicion of malignancy only to be diagnosed as a grade II gastric neuroendocrine tumor on final histopathological examination. Conclusion: Majority of G-NETs are detected incidentally on endoscopy or present with gastrointestinal bleeding, anemia, fatigue, epigastric pain and vomiting. One should be mindful of this unusual presentation of a G-NET as a gastric ulcer and further evaluation should be considered if there is suspicion of malignancy even after endoscopic biopsy.