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A Clinical Perspective on Gastric Neuroendocrine Neoplasia
Current Gastroenterology Reports, 2010
The incidence of gastric neuroendocrine tumors (NETs) has increased exponentially based on widespread use of endoscopy and a greater pathological awareness of the condition. A key concern is the potential association with hypergastrinemia induced by proton pump inhibitor administration. Previous confusion regarding diagnosis and therapy has been diminished by a series of international consensus statements defining the biology and management strategies for the disease. Overall, gastric NETs are categorized as welldifferentiated or poorly differentiated neoplasms. Welldifferentiated gastric NETs are enterochromaffin-like (ECL) cell tumors subclassified into three types based on their relationship to gastrin, a key regulator of ECL cell neoplastic transformation. The treatment of type 1 and type 2 tumors depends on the size and invasiveness of the tumor, whereas type 3 tumors and poorly differentiated neuroendocrine carcinomas warrant aggressive surgical resection. The disease-specific 5-year survival ranges from about 95% in type 1 gastric carcinoids to about 25% in poorly differentiated gastric NECs. Elucidation of the precise biology of a gastric NET is critical to diagnosis and delineation of a typespecific management strategy.
Neuroendocrine tumor presenting like lymphoma: a case report
Journal of Medical …, 2011
Neuroendocrine tumors are a rare but diverse group of malignancies that arise in a wide range of organ systems, including the mediastinum. Differential diagnosis includes other masses arising in the middle mediastinum such as lymphoma, pericardial, bronchogenic and enteric ...
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...
Neuroendocrine gastric carcinoma in a young patient
Vojnosanitetski pregled, 2010
Background. Neuroendocrine tumors cover a spectrum of neoplasms showing wide variations in their clinicopathological and pathogenetic features, as well as prognosis. They may develop throughout the whole gastrointestinal tract. Case report. We described a case of gastric neuroendocrine carcinoma in a 29-year-old male. The patient presented with chronic continuous abdominal pain and weight loss over a 6month period. Preoperative diagnosis, operative findings, histology and immunohistochemistry of the tumor confirmed the diagnosis of the rare neuroendocrine gastric carcinoma, stage T2N1. Conclusion. Case reports of this rare tumor are important, because of the paucity of studies noted in the gastrointestinal literature as a result of poor identification prior to the advent of modern immunohistochemistry. Significance of accurately diagnosing gastrointestinal neuroendocrine tumors is crucial for an appropriate treatment.
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
Gastric neuroendocrine neoplasias: manifestations and comparative outcomes
Endocrine-Related Cancer
Although gastric neuroendocrine neoplasias (gNEN) are an orphan disease, their incidence is rising. The heterogeneous clinical course powers the ongoing discussion of the most appropriate classification system and management. Prognostic relevance of proposed classifications was retrospectively analyzed in 142 patients from a single tertiary referral centre. Baseline, management and survival data were acquired for statistical analyses. The distribution according to the clinicopathological typification were: gNEN-1 (n=86/60.6%), gNEN-2 (n=7/4.9%) gNEN-3 (n= 24/16.9%) and gNEN-4 (n=25/17.6%); while hypergastrinemia-associated gNEN-1 and -2 were all low grade tumours (NET-G1/2), formerly termed sporadic gNEN-3 could be subdivided into gNEN-3 with grade 1 or 2 and gNEN-4 with grade 3 (NEC-G3). During follow-up 36 patients died (25%). The mean overall survival (OS) of all gNEN was 14.2 years. The OS differed statistically significant across all subgroups with either classification system....
Gastric neuroendocrine tumors Chapter 1 Overview on Gastric Cancer
2017
Gastric Neuroendocrine Tumors (NET)s are classified on the basis of criteria that are common to all gastrointestinal and pancreatic neuroendocrine neoplasms. Most neuroendocrine neoplasms of the stomach are NETs –well differentiated, nonfunctioning enterochromaffin–like(ECL) cell carcinoids (ECL cell NETs)-arise predominantly in the corpus-fundus region [1]. Three distinct types are recognized :
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.
Neuroendocrine carcinoma of stomach causing gastric outlet obstruction: a diagnostic dilemma
International Surgery Journal
Gastric neuroendocrine tumour (GNET), also known as carcinoids, are a very rare cause of gastric outlet obstruction and arise from entero-chromaffin like cells of the mucosa. They account for 0.2/1000001 population and 8.7% of all Gastrointestinal carcinoids. They are four types based on the degree of differentiation, cell of origin and other pathologic features. Type 4 GNET are referred to as neuroendocrine carcinoma accounting for <1% of GNET. They are aggressive, poorly differentiated, locally invasive tumours with metastatic potential. Hence, they require an aggressive approach in terms of surgery and multimodality adjuvant treatment. Our patient is an elderly hypertensive who had complaints of projectile vomiting over ten days associated with weight loss over the last two years. A diagnosis of gastric outlet obstruction was made, and she underwent a contrast CT scan and endoscopy twice, both being suggestive of malignancy involving the antrum region however, the endoscopic b...