Informed consent was obtained for this case report. Gastrointestinal Pathology and Its Clinical Implications 2nd ed2014;190225. [13] The endoscopic appearance of g-NENs consists of polypoid lesions, solitary or multiple. All polyps showed linear neuroendocrine cell hyperplasia within hyperplastic foveolar epithelium both at the surface and within deeper-situated glands. [15,41,42] Vanoli et al reported in 1 patient with type A-CAG and type I gastric NET treated by antrectomy a decrease in gastrin levels to undetectable levels, and a regression of ECL cell hyperplasia postoperatively, without NET recurrence. [27]. Meet the TRISH Team. [10] The classification system was updated in 2017 and 2018, and NENs were divided in neuroendocrine tumors (NETs), NECs, and mixed neuroendocrinenon-neuroendocrine neoplasms (MiNENs). your express consent. Tumor Biol 2014;35:595601. 2022 Dec 31;15(1):295. doi: 10.3390/cancers15010295. Histologic characterization and improved prognostic evaluation of 209 gastric neuroendocrine neoplasms. Rindi G, Klimstra DS, Abedi-Ardekani B, et al. MeSH 2 Previous studies have reported an annual incidence of type 1 GNET ranging from 0.4% to 0.68% in patients with AMAG. Editorial Board. Adv Anat Pathol. Eur J Gastroenterol Hepatol 2001;13:144956. [50] Octreotide showed good results in tumors regression in ZES or MEN-1. Endoscopic view of tumor with central ulceration in the gastric body (A) and a large duodenal ulcer (B). Data is temporarily unavailable. generally only neuroendocrine cell hyperplasia is seen; With MEN1, may have neuroendocrine cell dysplasia and neoplasia . AMAG is characterized by immune-mediated destruction of gastric parietal cells. In type 3 g-NET, detected in metastatic stage, oncologic therapy was performed. https://www.cancer.net/cancer-types/neuroendocrine-tumor-gastrointestina NCI CPTC Antibody Characterization Program, Modlin IM, Kidd M, Latich I, Zikusoka MN, Shapiro MD. Endocrinol Diabetes Metab Case Rep 2018;2018: 18-0048. The features are consistent with autoimmune gastritis in the appropriate clinical context. At the level of duodenum, multiple ulcers were found (Fig. Water-Clear Cell Hyperplasia. Their morphology, histogenesis, and natural history. Gastroenterol Res Pract 2014;2014: 253860. 2010 Nov;34(11):1591-8 MIB1 immunohistochemical stain showed a Ki-67 proliferation index of less than 3% within these aggregates, which was pathologically suggestive of a type 1 microneuroendocrine tumor (Figure 2). Histologic changes in type A chronic atrophic gastritis indicating increased risk of neuroendocrine tumor development: the predictive role of dysplastic and severely hyperplastic enterochromaffin-like cell lesions. Hum Pathol. Version: StomachNET 4.0.0.1 Protocol Posting Date: June 2017 Includes pTNM requirements from the 8th Edition, AJCC Staging Manual. Laparoscopic antrectomy for the treatment of type I gastric carcinoid tumors. Histidine decarboxylase, DOPA decarboxylase and vesicular monoamine transporter 2 expression in neuroendocrine tumors: immunohistochemical study and gene expression analysis. 6. All the 3 patients underwent upper endoscopy with targeted biopsies. Published by Wolters Kluwer Health, Inc. -, Taghavi S, Jayarajan SN, Powers BD, Davey A, Willis AI. Solcia E, Kloppel G, Sobin LH. As a result, the marked hypergastrinemia was considered to have been caused by G-cell hyperplasia related to a block in the negative feedback mechanism of somatostatin against achlorhydria with autoimmune gastritis. may email you for journal alerts and information, but is committed modify the keyword list to augment your search. Most of them are G1 NETs, rarely G2 NETs. Carolina Martinez Ciarpaglini, M.D., Ph.D. heterotopic pancreas / pancreatic acinar metaplasia, chemotherapy/radiation-induced gastropathy, poorly differentiated neuroendocrine carcinoma. [55,56] Another particularity of this case is the detection of a well-differentiated G2 tumor with liver metastases. Histologic changes in type A chronic atrophic gastritis indicating increased risk of neuroendocrine tumor development: the predictive role of dysplastic and severely hyperplastic enterochromaffin-like cell lesions. [52] In the case of metastatic liver disease, surgery, somatostatin analogues, chemotherapy (streptozocin, 5-fluorouracil with leucovorin, cyclophosphamide, doxorubicin, oxaplatin, dacarbazine), and locoregional control methods (targeted radionucleotide therapies, transarterial chemoembolization, radiofrequency ablation) are recommended. This case exemplifies a thorough diagnostic workup for AMAG with GNET and reviews the pathophysiology behind the development of this condition. Usefulness of laparoscope-assisted antrectomy for gastric carcinoids with hypergastrinemia. This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. NEUROENDOCRINE NEOPLASMS: CLINICOPATHOLOGIC ANALYSIS OF 132 CASES . Eur J Gastroenterol Hepatol. Close communication between the histopathologist and clinician is required, with an analysis of the relevant clinical data, correlated with histologic analysis of tumor and nontumor tissue. Gastrin represents a direct stimulus for parietal cells to produce acid, and also acts like a direct proliferative stimulus on ECL cells. [25]. [16]. The authors thank Ms. Stephanie Stebens, MLIS, AHIP, and Dr. Karla D. Passalacqua, PhD, at Henry Ford Hospital for editorial assistance and manuscript formatting. No Helicobacter pylori was identified in the specimens. [33]. Gastritis, Gastric Polyps and Gastric Cancer. [31,32], Type 2 ECL cell NETs represent 5% to 6% of all g-NETs and occur in patients with hypergastrinemia, secondary to ZES and MEN-1 syndrome. 1989 Mar 1;63(5):881-90. Less frequently, Caduet causes headache (5%). 1. Neuroendocrine cell proliferations of the stomach arise in various settings and show features ranging from hyperplasia to neoplasia; . Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare and poorly understood lung condition that is characterized by the abnormal overgrowth of certain cells in the lung (called pulmonary neuroendocrine cells) that receive signals from nerve cells (neurons) and produce hormones. EUS was grossly normal without findings of a primary tumor or mucosal abnormalities. Hypergastrinemia represents the stimulus for hyperplastic proliferation of ECL cells and the development of NETs. How to cite this article: Boeriu A, Dobru D, Fofiu C, Brusnic O, Onior D, Mocan S. Gastric neuroendocrine neoplasms and precursor lesions: case reports and literature review. Autoimmune gastritis: Pathologist's viewpoint. 2014. and transmitted securely. Endocrinol Metab Clin North Am 2018;47:485, Mixed neuroendocrine nonneuroendocrine neoplasm (MiNEN), Odze: Surgical Pathology of the GI Tract, Liver, Biliary Tract and Pancreas, 4th Edition, 2022, WHO Classification of Tumours Editorial Board: Digestive System Tumours, 5th Edition, 2019, The 5th edition of the WHO 2019 Digestive Tumors Classification definition of neuroendocrine tumor (NET): well differentiated, grade 1, 2 or 3 based on mitotic rate per 2 mm, Must lack features of carcinoma, which includes poorly differentiated morphology, tumoral necrosis, high N/C ratio and prominent nucleoli, Type I is the most common, followed by type III, with type II being very rare, Type I is typically indolent and type II and III have a higher malignant potential (, Grade 3 neoplasms are no longer automatically categorized as a carcinoma per the updated 2019 WHO classification, Can be a component of a mixed neuroendocrine nonneuroendocrine neoplasm (MiNEN); must represent 30% of tumor (, Gastric NETs often occur in the setting of certain diseases (, Derived from enterochromaffin-like (ECL) cells, Occurs in a setting of chronic atrophic gastritis (type A) and hypergastrinemia, Occurs in a setting of hypergastrinemia due to Zollinger-Ellison syndrome, Can be seen in patients with multiple endocrine neoplasia type 1 (MEN1) syndrome, Discussed in the literature but not currently recognized by the WHO, Usually nonfunctional but can cause Zollinger-Ellison syndrome and is then referred to as a gastric gastrinoma, All types tend to occur in the age range of 50 - 60 years, Type I and II are more common in the gastric body and are often multifocal; usually < 2 cm, Type III occurs anywhere in the stomach and is usually unifocal; usually > 2 cm, May arise in the setting of autoimmune gastritis (type I), Zollinger-Ellison syndrome (type II) or in the absence of a known precursor (type III) (, Tumor functionality is based on clinical symptoms, not on immunohistochemical expression of the hormone (, These tumors do not typically cause symptoms secondary to hormone secretion, Serum chromogranin A is used as biomarker to assess the bulk of disease and monitor treatment (, Neuroendocrine neoplasms test (NETest) is a multianalyte liquid biopsy that measures neuroendocrine tumor gene expression in blood and can be used as a diagnostic and disease surveillance test (, Type I and type II will typically have elevated serum gastrin levels, Dependent on tumor subtype, grade and stage at presentation, Type I: excellent prognosis with a 5 year survival of 90 - 95% (, Type II: good prognosis with a 5 year survival of 60 - 90% (, Type III: worse prognosis with a 5 year survival rate of < 35% (, 37 year old woman presented with upper gastrointestinal bleed and epigastric pain (, 45 year old woman with autoimmune pernicious anemia and Hashimoto thyroiditis monitored by upper endoscopy (, 56 year old woman with a history of cholelithiasis and irritable bowel syndrome presented with postprandial, colicky left upper quadrant pain radiating to the right shoulder lasting approximately 45 minutes (, 66 year old man with gastric NET presented as a subepithelial tumor mimicking a gastrointestinal stromal tumor (, 68 year old man with an incidental finding of a small nodule in the gastric fundus (, Dependent on size at time of endoscopic evaluation, Excision of tumors: endoscopic mucosal resection, local resection, antrectomy or total gastrectomy (, Small, sharply outlined, covered by flattened mucosa, Architecturally, arranged in nests, cords or trabeculae, Bland, round to oval cells with typical salt and pepper chromatin and amphophilic cytoplasm, Type I NET background oxyntic mucosa is atrophic with metaplasia (intestinal type most commonly), with ECL cell hyperplasia, Correlates with endoscopic impression of atrophy, Type II NET background oxyntic mucosa is hyperplastic with ECL cell hyperplasia, Correlates with endoscopic impression of hypertrophic mucosal folds, Type III NET background oxyntic mucosa is normal without ECL cell hyperplasia (, Small cohort studies show associations with germline mutation in the, Well differentiated neuroendocrine tumor, WHO grade 1, 1.8 cm, excised (see comment and synoptic table), Oxyntic mucosa with moderate chronic gastritis, Mild intestinal metaplasia, incomplete type, Comment: Histologic sections of the stomach demonstrate involvement by nests of tumor cells with round nuclei, variably prominent nucleoli and abundant clear to eosinophilic cytoplasm, consistent with a well differentiated neuroendocrine tumor. Li TT, Qiu F, Qian ZR, et al. Yao JC, Hassan M, Phan A, et al. It is a non-progressive disease of the interstitial tissues of the lungs. Dis Colon Rectum. [55]. Endoscopic view of multiple polypoid lesions and corporeal atrophic gastritis. [56]. Stanford Medicine School of Medicine Departments Surgical Pathology Criteria Gastric Endocrine Hyperplasia, Dysplasia and Neoplasia. Disclaimer, National Library of Medicine . 2020;59(6):799803. J Clin Pathol 2014;67:93848. These NENs can be preceded by ECL cells hyperplastic and dysplastic lesions, whose oncologic potential has not yet been fully elucidated. Pathol Oncol Res 2011;17:75963. Serum chromogranin A is the most commonly used biomarker to assess the bulk of disease and monitor treatment and is raised in both functioning and non-functioning neuroendocrine tumors. [58]. Considering the diversity of the endocrine population of cells and the hormonal complexity of the gastrointestinal system, it is surprising that the diseases of the system are so limited. Department of Pathology Stanford University School of Medicine Stanford CA 94305-5342 . World J Clin Cases 2021;9:797385. Li QL, Zhang YQ, Chen WF, Xu MD, Zhong YS, Ma LL, Qin WZ, Hu JW, Cai MY, Yao LQ, Zhou PH. The common feature and causal link is atrophic gastritis, which predisposed the gastric mucosa to the development of both neuroendocrine cell hyperplasia and tumours, and hyperplastic polyps. 6B), synaptophysin, cluster of differentiation X2 (corresponding with a primary gastric lesion), and negative staining with cytokeratin 7 and 20, and for prostate-specific antigen. Pathol Res Pract. Neuroendocrine nuttiness in the digestive system - Dr. Raul S. Gonzalez, GI neuroendocrine tumors classification - Dr. Vikram Deshpande. Ucella S, Ceritti R, Vigetti D, et al. Various treatment options are available for curative and palliative treatment of gastrointestinal neuroendocrine tumors. This website is intended for pathologists and laboratory personnel but not for patients. Pouessel D, Gallet B, Bibeau F, et al. Carcinoid syndrome; Colonic neuroendocrine tumors; Gastric neuroendocrine tumors; Gastrointestinal neuroendocrine tumors; Rectal neuroendocrine tumors; Small intestinal neuroendocrine tumors. Random mucosal biopsies confirmed neuroendocrine tumor of the gastric body composed of small clusters/nodules of neuroendocrine hyperplasia with an estimated proliferation index less than 3%. [39]. Oral cavity, esophagus and stomach. AMAG is associated with other autoimmune diseases and is a risk factor for the development of type 1 gastric neuroendocrine tumor (GNET). This site needs JavaScript to work properly. [32]. Scherulb H, Cadiot G, Jensen RT, Rosch T, Stolzel U, Kloppel G. Neuroendocrine tumors of the stomach (gastric carcinoids) are on rise, small tumors, small problems? The https:// ensures that you are connecting to the [11,12], In contrast with most of the digestive NENs, gastric tumors may have a specific clinical context, with distinct prognosis and therapeutic management. WHO International Histological Classification of Tumours 2nd edBerlin: Springer; 2000. Manneh R, Castellano D, Caso O, et al. BJU Int 2007;99:80711. Hoshino M, Omura N, Yano F, et al. The site is secure. Medical history and physical examination When your doctor takes a "medical history," he/she will ask you a series of . Endoscopic submucosal dissection for foregut neuroendocrine tumors: an initial study. They are classified into 4 types. Chen WF, Zhou PH, Li QL, et al. After a succinct outline of the types and distribution of the neuroendocrine cells in the normal gastric mucosa we discuss the most common situations in which the pathologist needs to think about gastric neuroendocrine cells. Given concern for type 1 GNET, she underwent a gallium-68 DOTATATE positron emission tomography scan, which was negative. Primary location was lung (40.57%, n=43), folowed by pancreas (16.04%, n=17), stomach (15.09%, n=16), appendix (8.49%, n=9), small . However, we cannot answer medical or research questions or give advice. The background gastric mucosa shows moderate chronic gastritis with pseudopyloric and intestinal metaplasia. The https:// ensures that you are connecting to the http://creativecommons.org/licenses/by/4.0. HHS Vulnerability Disclosure, Help [37] Aggressive tumors can infiltrate the muscularis propria with angio-invasion, lymph node, and liver metastases. Williams GT. Get new journal Tables of Contents sent right to your email inbox, http://creativecommons.org/licenses/by/4.0, Gastric neuroendocrine neoplasms and precursor lesions: Case reports and literature review, Articles in PubMed by Alina Boeriu, MD, PhD, Articles in Google Scholar by Alina Boeriu, MD, PhD, Other articles in this journal by Alina Boeriu, MD, PhD, Ossifying pilomatricoma and a novel hypothesis for its pathogenesis: A case report and comprehensive literature review, Gastritis in patients undergoing sleeve gastrectomy: Prevalence, ethnic distribution, and impact on glycemic, Clinical effectiveness of acupuncture on Parkinson disease: A PRISMA-compliant systematic review and meta-analysis, Gastrointestinal stromal tumors (GISTs) and second malignancies: A novel sentinel tumor? A risk factor for the treatment of gastrointestinal neuroendocrine tumors ; Rectal neuroendocrine tumors ; Small neuroendocrine! 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