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1.
Clin J Gastroenterol ; 2024 Jun 12.
Artigo em Inglês | MEDLINE | ID: mdl-38865017

RESUMO

Although gastric neuroendocrine tumors (NETs) are uncommon compared with gastric carcinomas, the incidence of NETs has been recently increasing. Gastric NETs are classified into three subgroups, and among these, gastrin-independent sporadic type 3 gastric NETs have a poor prognosis because of frequent lymph node or distant metastasis. We experienced a case of an early-stage type 3 gastric NET associated with lymphovascular and submucosal invasion. In a 54 year-old woman, esophagogastroduodenoscopy performed during a health screening identified an elevated lesion of the upper body of the stomach. The results of immunohistochemical analyses of endoscopic biopsy specimens obtained from the lesion were positive for chromogranin A and synaptophysin, indicating an NET. Because the patient's serum gastrin level was normal and she had no predisposing conditions for NET development, the tumor was diagnosed as a type 3 gastric NET. The patient underwent local resection of the tumor and regional lymph node dissection. The resected specimen indicated a diagnosis of type 3 gastric NET with invasion into the submucosa and lymphatic duct. This is an extremely rare case of an early-stage type 3 gastric NET. Our discussion provides insight into the pathogenesis and development of these tumors and the appropriate therapeutic strategy.

2.
Cancers (Basel) ; 16(7)2024 Mar 28.
Artigo em Inglês | MEDLINE | ID: mdl-38610988

RESUMO

Autoimmune atrophic gastritis (AAG) is a chronic condition characterized by the presence of atrophy in the oxyntic mucosa due to anti-parietal cell antibodies. This review provides a comprehensive and up-to-date overview of autoimmune atrophic gastritis, reporting recent evidence on epidemiology, pathogenesis, diagnosis, clinical presentation, risk of malignancies, and management. The prevalence of AAG has been estimated at between 0.3% and 2.7% in the general population. The diagnosis of AAG is based on a combination of the serologic profile and the histological examination of gastric biopsies. Patients with AAG are often asymptomatic but can also have dyspeptic or reflux symptoms. The atrophy of the oxyntic mucosa leads to iron and vitamin B12 malabsorption, which may result in anemia and neurological affections. Autoimmune atrophic gastritis is associated with an increased risk of type I neuroendocrine tumors (NETs) and gastric cancer, with an incidence rate of 2.8% and 0.5% per person/year, respectively. Management is directed to reinstate vitamins and iron and to prevent malignancies with endoscopic surveillance. In conclusion, atrophic autoimmune gastritis is an infrequent condition, often asymptomatic and misdiagnosed, that requires an early diagnosis for appropriate vitamin supplementation and endoscopic follow-up for the early diagnosis of NETs and gastric cancer.

3.
Front Med (Lausanne) ; 11: 1327864, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38357651

RESUMO

Gastric neuroendocrine neoplasms (g-NENs) are rare tumors arising from the gastric enterochromaffin-like cells. Recent data suggests an increased detection rate, attributed to more frequent esophagogastroduodenoscopies. While type 3 g-NENs were historically deemed aggressive, emerging research indicates potential for conservative management, especially endoscopic resection, in well-differentiated, small tumors. European guidelines now advocate for endoscopic intervention in selected cases, but North American guidelines remain more conservative. Key factors influencing outcomes are tumor size, grading, and depth of gastric wall infiltration. Endoscopic resection has shown promise for tumors confined to submucosal layers without lymphovascular invasion. Given the complexities, a multidisciplinary team approach is essential for management decisions. Current insights are largely based on retrospective studies, underscoring the need for prospective research to optimize endoscopic approaches.

4.
Dig Liver Dis ; 56(4): 589-600, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38216439

RESUMO

The present paper reflects the position of the Italian Association for Neuroendocrine Tumors (Itanet), the Italian Society of Gastroenterology (SIGE), and the Italian Society of Digestive Endoscopy (SIED) regarding the management of patients affected by gastric, duodenal, and rectal neuroendocrine neoplasms (NENs) amenable to endoscopic treatment. The key questions discussed in this paper are summarized in Table 1. Data were extracted from the MEDLINE database through searches; expert opinions and recommendations are provided in accordance with the available scientific evidence and the authors' expertise. Recommendations are presented alongside a level of evidence and grade of recommendation based on the GRADE system. This paper specifically focuses on subgroups of NENs considered suitable for endoscopic management according to current international guidelines: i. well-differentiated gastric neuroendocrine tumors (gNET) type 1 < 2 cm and selected cases of type 3; ii. well-differentiated duodenal, non-functioning, non-ampullary NET with size < 2 cm; and well-differentiated rectal NET with size < 2 cm.


Assuntos
Gastroenterologia , Tumores Neuroendócrinos , Neoplasias Gástricas , Humanos , Tumores Neuroendócrinos/patologia , Endoscopia Gastrointestinal , Neoplasias Gástricas/patologia , Itália
5.
J Neuroendocrinol ; 35(8): e13306, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-37401795

RESUMO

The aim of the present guidance paper was to update the previous ENETS guidelines on well-differentiated gastric and duodenal neuroendocrine tumours (NETs), providing practical guidance for specialists in the diagnosis and management of gastroduodenal NETs. Type II gastric NETs, neuroendocrine carcinomas (NECs), and functioning duodenal NETs are not covered, since they will be discussed in other ENETS guidance papers.


Assuntos
Carcinoma Neuroendócrino , Tumores Neuroendócrinos , Neoplasias Gástricas , Humanos , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/terapia , Tumores Neuroendócrinos/patologia , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/patologia , Sociedades
6.
Biomedicines ; 11(3)2023 Mar 13.
Artigo em Inglês | MEDLINE | ID: mdl-36979851

RESUMO

BACKGROUND: Type 1 gastric neuroendocrine tumors (GC-1) represent an uncommon subtype of neoplasms. Endoscopic resection has been proposed as the treatment of choice; active surveillance may be performed in those smaller than 1 cm, while gastric surgery may be performed for those with frequent recurrences. The antiproliferative effect of somatostatin analogues (SSA) is well known, and their action on GC-1s has been postulated as a chronic treatment to reduce recurrence. METHODS: A two-centered, retrospective, observational study that included nine patients (55.6% women) diagnosed with GC-1, receiving long-term treatment with SSA, with a median follow-up from baseline of 22 months, was undertaken. Endoscopic follow-up, extension study, and analytical values of chromogranin A (Cg A) and gastrin were collected. RESULTS: In total, 88.9% of patients presented partial or complete response. Treatment with SSA was the only independent factor with a trend to prevent tumor recurrence (Odds Ratio 0.054; p = 0.005). A nonsignificant tendency toward a decrease in CgA and gastrin was observed; lack of significance was probably related to concomitant treatment with proton pump inhibitors in some patients. CONCLUSIONS: Chronic treatment with SSA is a feasible option for recurrent GC-1s that are difficult to manage using endoscopy or gastrectomy. Randomized clinical trials to provide more scientific evidence are still needed.

7.
J Clin Med ; 11(6)2022 Mar 16.
Artigo em Inglês | MEDLINE | ID: mdl-35329967

RESUMO

Background: Type I gastric neuroendocrine neoplasia (gNEN) is a rare and low-grade tumor in which the therapeutic strategy is almost always endoscopic. For this reason, the use of radiology or nuclear medicine imaging is not recommended by guidelines. Conversely, in a small number of cases, locoregional or distant metastases may develop, thus suggesting a role for imaging techniques. This retrospective study was performed to explore the usefulness of [68Ga]Ga-DOTA-SST PET/CT in the management of patients with T1gNENs. Patients and Method: Single-center retrospective analysis, in an ENETS Center of Excellence, of patients with type I gNEN who underwent [68Ga]Ga-DOTA-SST PET/CT. The indication for performing [68Ga]Ga-DOTA-SST PET/CT was generally based on the presence of at least one of the following criteria: (1) polyps > 10 mm; (2) endoscopic positive (R1) margin after previous endoscopic resection; and (3) Ki-67 > 3%. Results: A total of 120 patients with T1gNEN were evaluated. Overall, 15 out of 120 (13%) patients had performed [68Ga]Ga-DOTA-SST PET/CT. The median Ki-67 value was 6% (IQR 1−9): 9 out of 15 (60%) were G1 tumors, and 6 out of 15 (40%) were G2 tumors. Ninety-three percent of patients were treated by tumor endoscopic resection, whereas surgery was performed in two patients (13%) after incomplete endoscopic resection; the remaining patients (6.6%) received somatostatin analogs due to the presence of multiple recurrent tumors. Overall, [68Ga]Ga-DOTA-SST PET/CT was positive in 8 out of 15 patients (53%). Following the [68Ga]Ga-DOTA-SST PET/CT findings, the clinical management was modified in 6 out of 15 (40%) patients. Conclusion: [68Ga]Ga-DOTA-SST PET/CT can be useful in a restricted and selected group of patients with gastric neuroendocrine neoplasia with relevant risk factors to establish the most appropriate therapeutic strategy.

8.
Dig Surg ; 38(1): 38-45, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33260173

RESUMO

BACKGROUND: Current guidance for type 1 gastric neuroendocrine neoplasms (gNENs) recommends either resection of all visible lesions or selective resection of gNENs >10 mm. We adopt a selective strategy targeting lesions approaching 10 mm for endoscopic mucosal resection (EMR) and provide surveillance for smaller lesions. OBJECTIVES: This study aimed to describe the incidence of type 1 gNENs requiring endoscopic/surgical resection and the risk of disease progression (both considered significant disease) on endoscopic surveillance. The secondary objective was to assess the risk factors for disease progression during surveillance and the incidence of gastric dysplasia/adenoma/adenocarcinoma. METHODS: We collected consecutive patients with type 1 gNENs and obtained demographic and clinical data through the electronic patient record. RESULTS: In our cohort of 57 patients, 12 patients had EMR at index gastroscopy; 7 patients had surgery (4: large/multiple gNENs and 3: nodal metastases) (5.2% [3/57] risk of nodal metastases); and a patient with nodal and liver metastases (1.8% [1/57] risk of distant metastases). The prevalence of gastric adenocarcinoma in our study was 3.5% with an incidence rate of 9.59 per 1,000 persons per year. For patients undergoing surveillance, 29.5% (13/44) of patients progressed requiring resection. Serum gastrin was significantly higher in patients who progressed to resection (p value = 0.023). CONCLUSION: We concluded that up to a third of patients with type 1 gNENs have significant disease requiring resection. Hence, endoscopic surveillance and resect strategy would benefit patients.


Assuntos
Tumores Neuroendócrinos/cirurgia , Neoplasias Gástricas/cirurgia , Estômago/patologia , Adenocarcinoma/patologia , Adenoma/patologia , Assistência ao Convalescente , Progressão da Doença , Ressecção Endoscópica de Mucosa , Gastroscopia , Humanos , Tumores Neuroendócrinos/patologia , Vigilância da População , Fatores de Risco , Estômago/cirurgia , Neoplasias Gástricas/patologia
9.
Rev. cir. (Impr.) ; 72(3): 245-249, jun. 2020.
Artigo em Espanhol | LILACS | ID: biblio-1115550

RESUMO

Resumen Introducción: Los tumores neuroendocrinos (TNE), son tumores compuestos por células productoras de péptidos y aminas. Los TNE gástricos, representan el 1% de todas las neoplasias, sin embargo su incidencia ha ido en aumento. Son generalmente asintomáticos y no funcionantes. El tratamiento es generalmente la resección local. Caso Clínico: paciente de 48 años con sospecha de cáncer gástrico; su estudio demuestra un TNE gástrico bien diferenciado tipo 1. Se realiza etapificación y se define en comité oncológico la vigilancia endoscópica. El hallazgo de un TNE, en el estudio de cáncer gástrico, es un hallazgo poco frecuente. Debido al aumento progresivo en la realización de endoscopías digestivas altas, secundario a la alta prevalencia de cáncer gástrico en nuestro país, se espera que aumenten hallazgos como un TNE. Es por esto que realizamos una revisión de la literatura y planteamos algunas conclusiones al respecto.


Introduction: Neuroendocrine tumors (NETs) are composed of cells that produce peptides and amines. Gastric NETs represent 1% of all neoplasms; however their incidence has been increasing. They are usually asymptomatic and non-functioning. The treatment is usually local resection. Case Report: We present the case of a 48-year-old patient who was suspected of gastric cancer; her study shows a well-differentiated type 1 gastric NET. Staging is performed and endoscopic surveillance is defined in the oncology board. The finding of a NET, in the study of gastric cancer, is a rare finding. Due to the progressive increase in the performance of upper gastrointestinal endoscopies, secondary to the high prevalence of gastric cancer in our country, it is expected to increase findings as a NET. That is why we conducted a review of the literature and made some conclusions about it.


Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/etiologia , Tumores Neuroendócrinos/terapia , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/fisiopatologia , Neoplasias Gástricas/terapia , Incidência , Oncologia/métodos , Estadiamento de Neoplasias
10.
United European Gastroenterol J ; 8(2): 140-147, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-32213066

RESUMO

BACKGROUND: Type-1 gastric neuroendocrine tumors represent a recurring disease and long-acting somatostatin analogs can inhibit both gastrin release and endocrine cell proliferation. The efficacy and timing of this treatment are still unclear. We performed a systematic review of the literature to clarify the role of somatostatin analog treatment in type-1 gastric neuroendocrine tumors. METHODS: A computerized literature search was performed using relevant keywords to identify all the pertinent articles published in the last 15 years. RESULTS: Eight studies were included in this systematic review on somatostatin analogs in type-1 gastric neuroendocrine tumors. A complete response rate ranged from 25-100%. When only the six prospective studies were considered, no significant heterogeneity was observed, and the pooled cumulative complete response rate was 84.5% (confidence interval 73.8-92.8). Three studies evaluated the type-1 gastric neuroendocrine tumor recurrence, with a cumulative relapse rate of 30.2% (confidence interval 13.1-50.6) after 34 months. CONCLUSION: Somatostatin analogs, namely lanreotide and octreotide, have an excellent response rate, with a good safety profile in selected type-1 gastric neuroendocrine tumors, which cannot be safely managed by endoscopic follow-up or resection due to multiple or frequently recurring disease. After therapy discontinuation, the cumulative relapse rate observed after a median 34-month follow-up was relatively high (30.2%).


Assuntos
Tumor Carcinoide/tratamento farmacológico , Neoplasias Intestinais/tratamento farmacológico , Recidiva Local de Neoplasia/epidemiologia , Tumores Neuroendócrinos/tratamento farmacológico , Octreotida/administração & dosagem , Neoplasias Pancreáticas/tratamento farmacológico , Peptídeos Cíclicos/administração & dosagem , Somatostatina/análogos & derivados , Neoplasias Gástricas/tratamento farmacológico , Tumor Carcinoide/mortalidade , Tumor Carcinoide/patologia , Intervalo Livre de Doença , Esquema de Medicação , Seguimentos , Humanos , Neoplasias Intestinais/mortalidade , Neoplasias Intestinais/patologia , Recidiva Local de Neoplasia/prevenção & controle , Tumores Neuroendócrinos/mortalidade , Tumores Neuroendócrinos/patologia , Octreotida/efeitos adversos , Neoplasias Pancreáticas/mortalidade , Neoplasias Pancreáticas/patologia , Peptídeos Cíclicos/efeitos adversos , Estudos Prospectivos , Somatostatina/administração & dosagem , Somatostatina/efeitos adversos , Estômago/efeitos dos fármacos , Estômago/patologia , Neoplasias Gástricas/mortalidade , Neoplasias Gástricas/patologia
11.
Autoimmun Rev ; 18(3): 215-222, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30639639

RESUMO

Chronic autoimmune atrophic gastritis (CAAG) is an organ-specific autoimmune disease, which affects the corpus-fundus gastric mucosa. Although it has been described for several years, the real pathophysiological mechanisms, the natural history and the possible neoplastic complications are not completely known. Atrophy of the gastric mucosa is the endpoint of the chronic processes, with the loss of glandular cells and their replacement by intestinal-type epithelium, pyloric-type glands, and fibrous tissue. As a consequence, hydrochloric acid, pepsin and intrinsic-factor is impaired resulting in pernicious anemia. The exact causal agent is not yet known, but both genetic and environmental factors seem to play a decisive role. Moreover, the clinical onset may assume different characteristics; differently from what previously observed, recent evidence has reported the onset of CAAG at a younger age, frequently with iron deficiency anemia or upper gastro-intestinal symptoms. The diagnosis of CAAG might be challenging and usually requires the combination of clinical, serological and histopathologic data; moreover, CAAG patients are often misdiagnosed as refractory to HP eradication therapy, probably because achlorhydria might allow urease-positive bacteria other than H pylori to colonize the stomach, causing positive 13C-urea breath test results. However, biopsy is the most reliable method to evaluate the presence of metaplastic atrophic gastritis. In order to assess the severity of gastric atrophy and intestinal metaplasia, OLGA and OLGIM staging systems have been proposed and seem to correlate with the risk of developing gastric adenocarcinoma. Indeed, CAAG represents a pre-neoplastic condition, as patients with CAAG are very likely to develop either type-1 gastric neuroendocrine tumors and gastric adenocarcinomas, as well as several other neoplastic diseases. To date, the need, the intervals and cost-effectiveness of endoscopic/histological surveillance for patients with CAAG/pernicious anemia are yet to be established.


Assuntos
Doenças Autoimunes , Gastrite Atrófica , Animais , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/patologia , Endoscopia Gastrointestinal , Gastrite Atrófica/complicações , Gastrite Atrófica/diagnóstico , Gastrite Atrófica/patologia , Humanos , Neoplasias/etiologia
12.
Gastroenterology Res ; 11(1): 75-78, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29511413

RESUMO

We present a case of an 18-year-old male who presented with complains of abdominal pain, nausea and vomiting for 2 years. An esophagogastroduodenoscopy (EGD) revealed a 3 mm nodule on the lesser curvature of the stomach and prominent gastric folds. Biopsy of the nodule revealed a well-differentiated neuroendocrine tumor (NET) in lamina prop with focal extension into muscularis mucosa consistent with a gastric carcinoid. Tumor cells stained with neuron-specific enolase (NSE), chromogranin and synaptophysin only. The prominent gastric fold biopsy revealed gastric fundic mucosa with mucosal edema and focal mild chronic inflammation. Serum gastrin level was found to be 2,083 pg/mL. Abdomen CT and endoscopic ultrasound (EUS) revealed a mass near the pancreatic neck. These findings were consistent with a functional gastrin producing well-differentiated grade 1 neuroendocrine neoplasm (gastrinoma). The patient underwent exploratory laparotomy with resection of the mass and resulting in normalization of gastrin levels.

13.
Int J Epidemiol ; 46(3): 914-923, 2017 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-28369403

RESUMO

Background: Gastrin, which induces gastric acid secretion, and a structurally similar hormone, cholecystokinin (CCK)-a potent acid inhibitor, may each play a role in gastric cancer. However, few studies have investigated this hypothesis in humans. We therefore investigated whether serum gastrin or CCK concentrations at baseline were associated with the incidence of gastric non-cardia adenocarcinomas (GNCA), oesophagogastric junctional adenocarcinomas (EGJA) or gastric carcinoid tumours over 24 years of follow-up in a study nested within the all-male Alpha-Tocopherol, Beta-Carotene Cancer Prevention (ATBC) Study of Finnish smokers. Methods: Totals of 283 incident GNCA, 96 EGJA and 10 gastric carcinoid cases, and 778 matched controls, were included in our analysis. Gastrin and CCK were measured using specific radioimmunoassays. Odds ratios (OR) and 95% confidence intervals (95% CI) were estimated by multivariable logistic regression with adjustment for all known or suspected confounding factors, including Helicobacter pylori seropositivity. Results: Those with high gastrin (Q4 vs Q1), had an increased risk of GNCA (fully adjusted OR: 1.92; 95% CI: 1.21, 3.05) and gastric carcinoids, though the small number of carcinoid cases meant the fully adjusted model was unstable (age-adjusted continuous model OR: 4.67; 95% CI: 2.67, 8.15). CCK was associated with risk of GNCA only for those in Q3 relative to Q1 (OR: 0.56; 95% CI: 0.33, 0.96), and no significant trend was observed. Conclusions: Our data suggest that high serum concentrations of gastrin may be associated independently with an increased risk of gastric cancer; the role of CCK in cancer risk is less clear.


Assuntos
Adenocarcinoma/epidemiologia , Colecistocinina/sangue , Neoplasias Esofágicas/epidemiologia , Gastrinas/sangue , Fumantes , Neoplasias Gástricas/epidemiologia , Adenocarcinoma/sangue , Estudos de Casos e Controles , Neoplasias Esofágicas/sangue , Finlândia/epidemiologia , Seguimentos , Infecções por Helicobacter/epidemiologia , Helicobacter pylori , Humanos , Incidência , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Estudos Prospectivos , Fatores de Risco , Neoplasias Gástricas/sangue
14.
Dis Model Mech ; 9(9): 975-84, 2016 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-27491072

RESUMO

By whole exome sequencing, we recently identified a missense mutation (p.R703C) in the human ATP4a gene, which encodes the proton pump responsible for gastric acidification. This mutation causes an aggressive familial type I gastric neuroendocrine tumor in homozygous individuals. Affected individuals show an early onset of the disease, characterized by gastric hypoacidity, hypergastrinemia, iron-deficiency anemia, gastric intestinal metaplasia and, in one case, an associated gastric adenocarcinoma. Total gastrectomy was performed as the definitive treatment in all affected individuals. We now describe the generation and characterization of a knockin mouse model for the ATP4a(R703C) mutation to better understand the tumorigenesis process. Homozygous mice recapitulated most of the phenotypical alterations that were observed in human individuals, strongly suggesting that this mutation is the primary alteration responsible for disease development. Homozygous mice developed premalignant condition with severe hyperplasia, dysplasia and glandular metaplasia in the stomach. Interestingly, gastric acidification in homozygous mice, induced by treatment with 3% HCl acid in the drinking water, prevented (if treated from birth) or partially reverted (if treated during adulthood) the development of glandular metaplasia and dysplasia in the stomach and partially rescued the abnormal biochemical parameters. We therefore suggest that, in this model, achlorhydria contributes to tumorigenesis to a greater extent than hypergastrinemia. Furthermore, our mouse model represents a unique and novel tool for studying the pathologies associated with disturbances in gastric acid secretion.


Assuntos
Técnicas de Introdução de Genes , ATPase Trocadora de Hidrogênio-Potássio/genética , Mutação/genética , Tumores Neuroendócrinos/genética , Tumores Neuroendócrinos/terapia , Neoplasias Gástricas/genética , Neoplasias Gástricas/terapia , Anemia/sangue , Anemia/complicações , Anemia/patologia , Animais , Modelos Animais de Doenças , Ácido Gástrico/metabolismo , Gastrinas/sangue , Homozigoto , Humanos , Ácido Clorídrico/farmacologia , Hiperplasia , Camundongos Endogâmicos C57BL , Camundongos Mutantes , Tumores Neuroendócrinos/sangue , Tumores Neuroendócrinos/prevenção & controle , Fenótipo , Estômago/patologia , Neoplasias Gástricas/sangue , Neoplasias Gástricas/prevenção & controle
15.
Scand J Gastroenterol ; 51(11): 1398-405, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27309188

RESUMO

OBJECTIVES: To review the presentation, treatment and outcome of patients with type 1 gastric carcinoid tumours. MATERIAL AND METHODS: We retrospectively reviewed medical records and re-evaluated histopathological specimens of 26 patients with type 1 gastric carcinoids treated at a single tertiary referral centre from 1993 to 2013, with median time of follow-up 52.5 months (IQR 90.8). RESULTS: Seven patients (27%) had single tumours and 19 patients (73%) multiple tumours at the time of diagnosis. The median number of tumours and median diameter of largest tumour were 2.5 (IQR 3.2) and 6.0 mm (IQR 9.5) respectively. Median serum gastrin was 321.0 pmol/l (IQR 604.0) and median serum chromogranin A 7.7 nmol/l (IQR 5.3). Three patients had metastatic disease at the time of diagnosis and two developed metastases during follow-up. Patients with metastatic disease had larger primary tumours than the others (20.0 mm (IQR 28.5) vs. 5.0 mm (IQR 5.5), p = 0.04). There was a positive correlation between age and tumour size (r = 0.44, p = 0.03) and between serum chromogranin A and serum gastrin at diagnosis (r = 0.76, p = 0.001). Patients were either treated with surgery (n = 8 (31%)), a long-acting somatostatin analogue and/or gastrin receptor antagonist (n = 10 (39%)) for a period of time, or were observed without treatment (n = 8 (31%) with close endoscopic follow up. CONCLUSIONS: Although gastric carcinoids have an overall good prognosis, a significant proportion develops metastatic disease. As partial and total gastrectomy is associated with major side effects, treatment with long-acting a somatostatin analogue or gastrin antagonist should be considered.


Assuntos
Tumor Carcinoide/patologia , Tumor Carcinoide/terapia , Celulas Tipo Enterocromafim/patologia , Neoplasias Gástricas/patologia , Neoplasias Gástricas/terapia , Idoso , Antineoplásicos Hormonais/uso terapêutico , Tumor Carcinoide/mortalidade , Cromogranina A/sangue , Comorbidade , Feminino , Seguimentos , Gastrectomia , Mucosa Gástrica/patologia , Gastrinas/sangue , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Metástase Neoplásica , Noruega , Octreotida/uso terapêutico , Receptor de Colecistocinina B/antagonistas & inibidores , Receptor de Colecistocinina B/uso terapêutico , Estudos Retrospectivos , Somatostatina/análogos & derivados , Somatostatina/uso terapêutico , Neoplasias Gástricas/mortalidade , Centros de Atenção Terciária , Tomografia Computadorizada por Raios X , Resultado do Tratamento
16.
Scand J Gastroenterol ; 50(7): 856-65, 2015 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-25645880

RESUMO

OBJECTIVE: Atrophic gastritis (AG) is a risk condition for gastric cancer and type I gastric carcinoids. Recent studies assessing the overall risk of gastric cancer and carcinoids in AG at long-term follow up are lacking. This study aimed to investigate in a prospective cohort of AG patients the occurrence of gastric cancer and carcinoids at long-term follow up. METHODS: A total of 200 AG patients from a prospective cohort (67% female, median age 55 years) with a follow up of 7.5 (range: 4-23.4) years were included. Inclusion criteria were presence of AG and at least one follow-up gastroscopy with biopsies at ≥4 years after AG diagnosis. Follow-up gastroscopies at 4-year intervals were performed. RESULTS: Overall, 22 gastric neoplastic lesions were detected (crude incidence 11%). Gastric cancer was diagnosed in four patients at a median follow up of 7.2 years (crude incidence 2%). Eleven type I gastric carcinoids were detected at a median follow up of 5.1 years (crude incidence of 5.5%). In seven patients, six low-grade and one high-grade dysplasia were found. The annual incidence rate person-year were 0.25% (95% confidence interval [CI]: 0.067-0.63%), 0.43% (95% CI: 0.17-0.89%), and 0.68% (95% CI: 0.34-1.21%) for gastric cancer, dysplasia, and type I-gastric carcinoids, respectively. The incidence rates of gastric cancer and carcinoids were not different (p = 0.07). CONCLUSION: This study shows an annual incidence rate of 1.36% person-year for gastric neoplastic lesions in AG patients at long-term follow up. AG patients are similarly exposed to gastric cancer and type I gastric carcinoids.


Assuntos
Tumor Carcinoide/patologia , Gastrite Atrófica/patologia , Helicobacter pylori/patogenicidade , Neoplasias Gástricas/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Biópsia , Estudos de Coortes , Feminino , Gastroscopia , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Estadiamento de Neoplasias , Análise de Regressão , Fatores de Risco , Adulto Jovem
17.
Scand J Gastroenterol ; 50(5): 550-9, 2015 May.
Artigo em Inglês | MEDLINE | ID: mdl-25665655

RESUMO

OBJECTIVE: Gastric carcinoids (neuroendocrine tumors) arise from enterochromaffin-like cells in the gastric mucosa. Most are caused by hypergastrinemia. The objectives were to determine if their prevalence in Europe, USA and Japan meets the criteria for an orphan disease and to justify treatment with a gastrin/CCK2 receptor antagonist. METHODS: We obtained data from European and USA cancer registries, and searched PubMed. RESULTS: Prevalence per 10,000 population obtained from cancer registries was: median 0.32 (range 0.09-0.92) for Europe; and 0.17 for the USA, equivalent to 4812 for the whole population. A PubMed search for gastric carcinoids yielded prevalence for Japan only, which was 0.05 per 10,000 population, equivalent to 665 for the entire population. A further search for gastric carcinoids in patients with pernicious anemia (PA) or autoimmune chronic atrophic gastritis (CAG), two presentations of about 80% of gastric carcinoids, produced prevalence rates of 5.2-11%. Prevalence of PA itself was 0.12-1.9%. Data on CAG epidemiology were sparse. CONCLUSION: Prevalence of gastric carcinoids varied widely. All sources probably underestimate prevalence. However, prevalence was below the limits required for recognition by drug regulatory authorities as an orphan disease: 5 per 10,000 population of Europe; 200,000 for the whole population of the USA; and 50,000 for the whole population of Japan. Because gastric carcinoids are an orphan disease, and nonclinical and healthy volunteer studies support treatment with netazepide, a gastrin/CCK2 antagonist, netazepide has been designated an orphan medicinal product in Europe and the USA for development as targeted treatment for gastric carcinoids.


Assuntos
Benzodiazepinonas/uso terapêutico , Tumor Carcinoide/tratamento farmacológico , Tumor Carcinoide/epidemiologia , Neoplasias Gastrointestinais/tratamento farmacológico , Neoplasias Gastrointestinais/epidemiologia , Compostos de Fenilureia/uso terapêutico , Receptor de Colecistocinina B/antagonistas & inibidores , Idoso , Idoso de 80 Anos ou mais , Anemia Perniciosa/epidemiologia , Celulas Tipo Enterocromafim/metabolismo , Europa (Continente)/epidemiologia , Feminino , Mucosa Gástrica/metabolismo , Gastrite Atrófica/epidemiologia , Humanos , Japão/epidemiologia , Masculino , Prevalência , Doenças Raras , Distribuição por Sexo , Estados Unidos/epidemiologia
18.
Scand J Gastroenterol ; 49(12): 1447-55, 2014 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-25309981

RESUMO

OBJECTIVE: Data on clinical presentation and associated features of patients with type 1 gastric carcinoids (T1-GCs) are scanty. This study aimed to provide detailed data on a series of patients with T1-GCs. MATERIAL AND METHODS: Clinical, laboratory, endoscopic, and histological data were assessed from 31 T1-GCs patients (cross-sectional design), consecutively diagnosed in a tertiary center according to a standardized diagnostic protocol. T1-GCs were diagnosed at baseline or follow-up gastroscopy for atrophic gastritis in 74.2% and 25.8% of patients, respectively. RESULTS: Seventy-one percent of T1-GC patients were female. Age ranged from 23 to 78 (median 58 years). T1-GCs were more frequently diagnosed between 40-49 years (35.5%) and 60-69 years (32.3%) (p = 0.0383). Thyroid disease was present in 54.8% (in 29% autoimmune). All 31 patients had either cobalamin or iron deficiency with or without anemia. Manifest pernicious anemia was present in 67.7% of patients and cobalamin deficiency without anemia in 9.7% patients. Iron deficiency anemia was present in 29% and iron deficiency without anemia in 12.9% of patients. In 48.4% of patients, T1-GCs appeared as polyps, which were single in all cases and had a median size of 4 mm (range 2-15 mm). In patients with polypoid T1-GCs, thyroid disease of autoimmune and nonautoimmune origin (p = 0.0181) was more frequently associated. CONCLUSION: This study shows that T1-GCs may be diagnosed at any age. Autoimmune features are frequently present as well as cobalamin and iron deficiency. The copresence of autoimmune diseases and micronutrient deficiencies should be accurately investigated, in particular in patients with polypoid T1-GCs.


Assuntos
Tumor Carcinoide , Neoplasias Gástricas , Adulto , Idoso , Anemia Ferropriva/etiologia , Anemia Perniciosa/etiologia , Tumor Carcinoide/complicações , Tumor Carcinoide/imunologia , Tumor Carcinoide/patologia , Estudos Transversais , Feminino , Gastrite Atrófica/etiologia , Gastroscopia , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Gástricas/complicações , Neoplasias Gástricas/imunologia , Neoplasias Gástricas/patologia , Deficiência de Vitamina B 12/etiologia
19.
Int J Surg ; 12 Suppl 1: S225-31, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-24862665

RESUMO

Gastric neuroendocrine tumors (g-NETs), which originate from gastric enterochromaffin-like (ECL) mucosal cells and account for 2.4% of all carcinoids, are increasingly recognized due to expanding indications of upper gastrointestinal endoscopy. Often silent and benign, g-NETs may however, be aggressive and sometimes they mimic the course of gastric adenocarcinoma. Current nosography distinguishes those occurring in chronic conditions with hypergastrinemia, as the type 1 associated with chronic atrophic gastritis, and the type 2 associated with Zollinger-Ellison syndrome in MEN1. Conversely, type 3 and 4 (according to some authors) are unrelated to hypergastrinemia and are frequently malignant, with a propension to develop distant metastases. While there is a general agreement concerning the treatment of malignant gastric neuroendocrine tumors, for types 1 and 2, current possibilities include surveillance, endoscopic polypectomy, surgical excision, associated or not with surgical antrectomy, or total gastrectomy. This report, based on our clinical experience, discusses how the size, number, depth, histological grading, staging with CT, MRI, and the use of recently developed somatostatin receptor tracers (68Ga-DOTATATE, 68Ga-DOTA-TOC) could allow the correct identification of a benign or malignant propensity of an individual tumor, thus avoiding underestimation or overtreatment of these uncommon neoplasms.


Assuntos
Gastrectomia/métodos , Tumores Neuroendócrinos/diagnóstico , Compostos Organometálicos , Tomografia por Emissão de Pósitrons/métodos , Neoplasias Gástricas/diagnóstico , Tomografia Computadorizada por Raios X/métodos , Idoso , Diagnóstico Diferencial , Feminino , Gálio , Humanos , Masculino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/cirurgia , Octreotida/análogos & derivados , Neoplasias Gástricas/cirurgia
20.
World J Gastroenterol ; 19(46): 8687-95, 2013 Dec 14.
Artigo em Inglês | MEDLINE | ID: mdl-24379587

RESUMO

AIM: To describe disease characteristics and treatment modalities in a group of rare patients with metastatic gastric carcinoid type 1 (GCA1). METHODS: Information on clinical, biochemical, radiological, histopathological findings, the extent of the disease, as well as the use of different therapeutic modalities and the long-term outcome were recorded. Patients' data were assessed at presentation, and thereafter at 6 to 12 monthly intervals both clinically and biochemically, but also endoscopically and histopathologically. Patients were evaluated for the presence of specific symptoms; the presence of autoimmune disorders and the presence of other gastrointestinal malignancies in other family members were also recorded. The evaluation of response to treatment was defined using established WHO criteria. RESULTS: We studied twenty consecutive patients with a mean age of 55.1 years. The mean follow-up period was 83 mo. Twelve patients had regional lymph node metastases and 8 patients had liver metastases. The primary tumor mean diameter was 20.13 ± 10.83 mm (mean ± SD). The mean Ki-67 index was 6.8% ± 11.2%. All but one patient underwent endoscopic or surgical excision of the tumor. The disease was stable in all but 3 patients who had progressive liver disease. All patients remained alive during the follow-up period. CONCLUSION: Metastatic GCA1 carries a good overall prognosis, being related to a tumor size of ≥ 1 cm, an elevated Ki-67 index and high serum gastrin levels.


Assuntos
Tumor Carcinoide/secundário , Neoplasias Hepáticas/secundário , Neoplasias Gástricas/patologia , Adulto , Idoso , Tumor Carcinoide/sangue , Tumor Carcinoide/química , Tumor Carcinoide/classificação , Tumor Carcinoide/terapia , Quimioterapia Adjuvante , Progressão da Doença , Europa (Continente) , Feminino , Gastrectomia , Derivação Gástrica , Gastrinas/sangue , Gastroscopia , Humanos , Israel , Antígeno Ki-67/análise , Neoplasias Hepáticas/classificação , Neoplasias Hepáticas/terapia , Excisão de Linfonodo , Metástase Linfática , Masculino , Pessoa de Meia-Idade , Imagem Multimodal , Tomografia por Emissão de Pósitrons , Estudos Retrospectivos , Neoplasias Gástricas/sangue , Neoplasias Gástricas/química , Neoplasias Gástricas/classificação , Neoplasias Gástricas/terapia , Fatores de Tempo , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Carga Tumoral
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