المؤلفون: Elena Guidetti, Monica Cevenini, Maria Luigia Cipollini, Martina Ferrata, Paola Tomassetti, Roberto Corinaldesi

المصدر: Clinical Management Issues, Vol 6, Iss 1S, Pp 23-28 (2015)

مصطلحات موضوعية: Multiple endocrine neoplasia type 1, MEN1 Gene, Genetic testing, Gastrinoma, Pancreatic neuroendocrine tumors, Medicine (General), R5-920

الوصف: Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant endocrine disorder and is characterised by the concurrent appearance of adenomas of the parathyroid glands, neuroendocrine-enteropancreatic tumours, and pituitary adenomas, as well as other types of less frequent tumours, such as adrenal cortical tumours, carcinoid tumours, lipomas, etc. Two different forms, familial and sporadic, have been described. The gene responsible, MEN1, consists of 10 exons encoding a 610-amino acid protein known as menin. The MEN1 syndrome is caused by inactivating mutations in MEN1 tumour suppressor gene. The combination of clinical and genetic investigation helps in the diagnosis. Genetic testing has been advocated to identify MEN1 carriers of the MEN1 families for the purpose of earlier detection of tumours. We present a patient with traditionally described manifestations of MEN1 (a parathyroid hyperplasia associated with a pancreatic neuroendocrine tumour and a gastrinoma), but with a negative genetic test for the MEN1 mutation.

وصف الملف: electronic resource

العلاقة: https://journals.seedmedicalpublishers.com/index.php/cmi/article/view/493Test; https://doaj.org/toc/1973-4832Test; https://doaj.org/toc/2283-3137Test

الوصول الحر: https://doaj.org/article/8069c723bfad4a558e8ada4b81602cb7Test

المؤلفون: Alessandra Elvevi, Pietro Invernizzi, Maurizio Quatrini, Sara Massironi, Federica Cavalcoli

المساهمون: Massironi, S, Cavalcoli, F, Elvevi, A, Quatrini, M, Invernizzi, P

المصدر: Endocrine. 75:942-948

مصطلحات موضوعية: medicine.medical_specialty, Endocrinology, Diabetes and Metabolism, Neuroendocrine tumors, Gastroenterology, Somatostatin analog, Zollinger-Ellison Syndrome, Endocrinology, Neuroendocrine tumor, Internal medicine, medicine, Humans, Pathological, Retrospective Studies, Gastrin, Gastrinoma, Somatostatin receptor, business.industry, Neuroendocrine neoplasia, medicine.disease, Zollinger-Ellison syndrome, Pancreatic Neoplasms, Somatostatin, Tumor progression, Zollinger Ellison Syndrome, business

الوصف: Purpose Zollinger Ellison syndrome (ZES) is a rare syndrome caused by gastrin hypersecretion from a gastrinoma. Gastrinoma treatment has two goals: the control of acid hypersecretion and the control of tumor growth. While therapy for the syndrome is univocally based on proton pump inhibitors, the one for disease control is still debated. We here aimed at evaluating the role of somatostatin analogs (SSAs) in the control of tumor progression in a series of ZES patients. Methods A retrospective analysis of a prospectively collected database of ZES patients, followed and managed from 1990 to 2019, was performed. The patients' clinical, pathological, treatment, and follow-up data were analyzed. Data regarding SSAs therapy start, dosage, duration, and side effects were collected. Results 33 patients with ZES were diagnosed. Fourteen patients (42%) had a grade 1 (G1) neuroendocrine neoplasm (NEN), five had G2 (15%), none had G3. Fifteen patients (45%) had metastatic disease. Overall, 12 (36%) underwent SSAs therapy. The median treatment duration was 36 months. Eight patients (67%) had a sustained response to SSAs, four (33%) showed an early progression, with a significant difference in terms of PFS between the patients with early and late progression (84 vs 2 months, p = 0.004). No differences in terms of OS and PFS were observed between the treated and non-treated patients, despite the proportion of metastatic patients was greater in the SSAs-treated group (75% vs 29% in the non-treated group, p = 0.01). Conclusion Present data support the use of SSAs in ZES, considering that gastrinoma is mainly a well-differentiated low-grade tumor (G1 or G2), with a high expression of somatostatin receptors.

وصف الملف: STAMPA

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::562cc12736ce594a52334fa5844b4bdeTest
https://doi.org/10.1007/s12020-021-02915-7Test

المؤلفون: Zaher Toumi, Katrina Clair Cockburn, Alison Mackie, Peter Julyan

المصدر: Journal of Gastrointestinal Surgery

مصطلحات موضوعية: Radioisotope, medicine.medical_specialty, English language, Review Article, Stomach Neoplasms, Intestinal Neoplasms, medicine, Humans, Gastrointestinal tract, Gastrinoma, Diagnostic techniques, Lesion detection, business.industry, Gastroenterology, Radioguided Surgery, medicine.disease, Pancreatic Neoplasms, Neuroendocrine Tumors, medicine.anatomical_structure, Systematic review, Surgery, Computer-Assisted, Neuroendocrine tumours/surgery, Radiopharmaceutical, Surgery, Radiology, Pancreas, business

الوصف: Background Radioguided surgery (RGS) for gastroenteropancreatic neuroendocrine tumours (GEP-NETs) has been suggested as a way to improve intraoperative lesion detection. This systematic literature review of reports of the use of RGS for GEP-NETs was performed to determine if there is a benefit. Methods A literature search was conducted using Google Scholar and PubMed, and snowballing from any relevant literature. Full-text studies were included if they were published in the English language and reported outcomes of RGS on human subjects with GEP-NETs. Qualitative data synthesis was performed. Results Twenty-six papers including a total of 209 patients were included. The tracers used were predominantly indium-111 pentetreotide, gallium-68 DOTA-peptides, and technetium-99m EDDA/HYNIC-peptides. Heterogeneous protocols make comparisons difficult, but most papers reported a benefit from the use of RGS in tumours in the gastrointestinal tract; utility in localisation of pancreatic tumours was less clear. Time between tracer administration and operation varied: from 16 h to 8 days with indium-111, 0–24 h with technetium-99m, and 19–193 min with gallium-68. Eight teams reported the thresholding technique used for discrimination—four used a ratio, four statistical methods, and one looked at the sensitivity and specificity of different cut-offs. Six teams performed follow-up of 24 patients (three pancreas, eight gastrinoma, 13 gastrointestinal tract) for between 3 months and 3 years. Two patients relapsed (one pancreas, one gastrinoma) between 6 and 12 months post-surgery. Conclusions RGS appears to aid in localisation of gastrointestinal NETs, but the benefit is more equivocal in pancreatic NETs. Further work into outcomes is warranted.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::10da2094e4c4383e5a66b5ec7161c9b3Test
http://europepmc.org/articles/PMC8654712Test

المؤلفون: Mesut Sezikli, Hüseyin Köseoğlu, Tolga Duzenli

المصدر: World Journal of Clinical Cases

مصطلحات موضوعية: Gastrinoma, medicine.medical_specialty, Proliferation index, business.industry, Atrophic gastritis, Gastric Neuroendocrine Carcinoma, Gastric neuroendocrine tumors, Gastric Neuroendocrine Neoplasm, General Medicine, Review, Neuroendocrine tumors, medicine.disease, Gastroenterology, Carcinoid, Hypergastrinemia, Gastric neuroendocrine carcinoma, Gastric neuroendocrine neoplasm, Somatostatin receptor imaging, Internal medicine, Medicine, Enterochromaffin-like cell, business, Grading (tumors)

الوصف: Gastric neuroendocrine neoplasms (g-NENs) or neuroendocrine tumors are generally slow-growing tumors with increasing incidence. They arise from enterochromaffin like cells and are divided into four types according to clinical characteristic features. Type 1 and 2 are gastrin dependent, whereas type 3 and 4 are sporadic. The reason for hypergastrinemia is atrophic gastritis in type 1, and gastrin releasing tumor (gastrinoma) in type 2 g-NEN. The diagnosis of g-NENs needs histopathological investigation taken by upper gastrointestinal endoscopy. g-NENs are positively stained with chomogranin A and synaptophysin. Grading is made with mitotic index and ki-67 proliferation index on histopathological analysis. It is crucial to discriminate between types of g-NENs, because the management, treatment and prognosis differ significantly between subtypes. Treatment options for g-NENs include endoscopic resection, surgical resection with or without antrectomy, medical treatment with somatostatin analogues, netazepide or chemotherapy regimens. Follow-up without excision is another option in appropriate cases. The prognosis of type 1 and 2 g-NENs are good, whereas the prognosis of type 3 and 4 g-NENs are close to the prognosis of gastric adenocancer.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::356314840081918cadfa40db9e49c5f6Test
http://europepmc.org/articles/PMC8462212Test

المؤلفون: Vincenzo Mazzaferro, Pietro Invernizzi, Alessandra Elvevi, Jorgelina Coppa, Roberta Elisa Rossi, Davide Citterio, Sara Massironi

المساهمون: Rossi, R, Elvevi, A, Citterio, D, Coppa, J, Invernizzi, P, Mazzaferro, V, Massironi, S

المصدر: World Journal of Gastroenterology

مصطلحات موضوعية: Endoscopic ultrasound, medicine.medical_specialty, Pancreatic neuroendocrine neoplasm, Peptic, Disease, Review, Gastroenterology, Zollinger-Ellison Syndrome, Internal medicine, Diagnosis, medicine, Multiple Endocrine Neoplasia Type 1, Humans, Multiple endocrine neoplasia, Duodenal neuroendocrine neoplasm, Retrospective Studies, Gastrinoma, medicine.diagnostic_test, business.industry, General Medicine, medicine.disease, Primary tumor, Zollinger-Ellison syndrome, digestive system diseases, Neuroendocrine neoplasm, Pancreatic Neoplasms, Neuroendocrine neoplasms, Therapy, Differential diagnosis, business, Diagnosi

الوصف: Zollinger-Ellison syndrome (ZES) associated with pancreatic or duodenal gastrinoma is characterized by gastric acid hypersecretion, which typically leads to gastroesophageal reflux disease, recurrent peptic ulcers, and chronic diarrhea. As symptoms of ZES are nonspecific and overlap with other gastrointestinal disorders, the diagnosis is often delayed with an average time between the onset of symptoms and final diagnosis longer than 5 years. The critical step for the diagnosis of ZES is represented by the initial clinical suspicion. Hypergastrinemia is the hallmark of ZES; however, hypergastrinemia might recognize several causes, which should be ruled out in order to make a final diagnosis. Gastrin levels > 1000 pg/mL and a gastric pH below 2 are considered to be diagnostic for gastrinoma; some specific tests, including esophageal pH-recording and secretin test, might be useful in selected cases, although they are not widely available. Endoscopic ultrasound is very useful for the diagnosis and the local staging of the primary tumor in patients with ZES, particularly in the setting of multiple endocrine neoplasia type 1. Some controversies about the management of these tumors also exist. For the localized stage, the combination of proton pump inhibitory therapy, which usually resolves symptoms, and surgery, whenever feasible, with curative intent represents the hallmark of gastrinoma treatment. The high expression of somatostatin receptors in gastrinomas makes them highly responsive to somatostatin analogs, supporting their use as anti-proliferative agents in patients not amenable to surgical cure. Other medical options for advanced disease are super-imposable to other neuroendocrine neoplasms, and studies specifically focused on gastrinomas only are scant and often limited to case reports or small retrospective series. The multidisciplinary approach remains the cornerstone for the proper management of this composite disease. Herein, we reviewed available literature about gastrinoma-associated ZES with a specific focus on differential diagnosis, providing potential diagnostic and therapeutic algorithms.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8de7f799050828bdfd1b132adaafbe14Test
http://europepmc.org/articles/PMC8475006Test

المؤلفون: Georgiana Emmanuela Gîlcă-Blanariu, Smaranda Diaconescu, Silvia Poamaneagra, Otilia Marginean, Gabriela Stefanescu, Gabriela Paduraru

المصدر: World Journal of Gastroenterology

مصطلحات موضوعية: Adult, Oncology, medicine.medical_specialty, Pancreatoblastoma, Vasoactive intestinal peptide-secreting tumor, Review, Disease, Gene mutation, Renal cell carcinoma, Pancreatic cancer, Internal medicine, Humans, Medicine, Child, Pancreas, Insulinoma, Gastrinoma, Pancreatic metastasis, business.industry, Gastroenterology, General Medicine, medicine.disease, Childhood, Pancreatic Neoplasms, Survival Rate, Transition, Screening, business

الوصف: The presence of pancreatic cancer during childhood is extremely rare, and physicians may be tempted to overlook this diagnosis based on age criteria. However, there are primary malignant pancreatic tumors encountered in pediatric patients, such as pancreatoblastoma, and tumors considered benign in general but may present a malignant potential, such as the solid pseudo-papillary tumor, insulinoma, gastrinoma, and vasoactive intestinal peptide secreting tumor. Their early diagnosis and management are of paramount importance since the survival rates tend to differ for various types of these conditions. Many pediatric cancers may present pancreatic metastases, such as renal cell carcinoma, which may evolve with pancreatic metastatic disease even after two or more decades. Several childhood diseases may create a predisposition for the development of pancreatic cancer during adulthood; hence, there is a need for extensive screening strategies and complex programs to facilitate the transition from pediatric to adult healthcare. Nevertheless, genetic studies highlight the fact the specific gene mutations and family aggregations may be correlated with a special predisposition towards pancreatic cancer. This review aims to report the main pancreatic cancers diagnosed during childhood, the most important childhood diseases predisposing to the development of pancreatic malignancies, and the gene mutations associates with pancreatic malignant tumors.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::33c52e9086d4e07e3bce5d930224b4afTest
https://doi.org/10.3748/wjg.v27.i32.5322Test

المؤلفون: Shaowei Xie, Ling Qiu, Guohua Yang, Songlin Yu, Xinqi Cheng, Yuanyuan Zhang, Danchen Wang, Qian Cheng, Yutong Zou, Xiaoli Ma, Jialei Yu, Fang Zhang, Dandan Sun

المصدر: Clinical Chemistry. 67:1220-1229

مصطلحات موضوعية: Percentile, Gastrinoma, Chromatography, medicine.diagnostic_test, Chemistry, Biochemistry (medical), Clinical Biochemistry, Radioimmunoassay, Mass spectrometry, medicine.disease, Pancreatic Neoplasms, Tandem Mass Spectrometry, Liquid chromatography–mass spectrometry, Immunoassay, Gastrins, medicine, Humans, Biomarker (medicine), Chromatography, Liquid, Gastrin

الوصف: Background Two major forms of gastrin, gastrin-17 (G17) and gastrin-34 (G34), exist in blood. However, conventional immunoassay methods can only quantify total gastrin or G17 alone. Here, we aimed to establish a liquid chromatography–tandem mass spectrometry (LC–MS/MS) method to quantify G17 and G34 simultaneously. Methods Serum samples were prepared by anion-exchange solid-phase extraction. The analytical performance of the LC–MS/MS method was validated and the method was compared to chemiluminescence immunoassay (CLIA) and radioimmunoassay (RIA). The G17 and G34 concentrations in 245 serum samples from healthy controls, individuals with gastrinoma, and individuals with other diseases were analyzed. Results The total runtime of the LC–MS/MS method was 6 min. No substantial matrix effect was observed with internal standard correction. The intraassay coefficients of variation (CVs) for G17 and G34 were 4.0%–14.2% and 4.4%–10.4%, respectively, and total CVs were 5.2%–14.1% and 4.6%–12.4%, respectively. The correlation coefficient between LC–MS/MS and CLIA was 0.87, and between LC–MS/MS and RIA was 0.84. The G17+G34 concentrations for 87.5% of individuals with gastrinoma were higher than the 95th percentile of healthy controls (18.1 pg/mL), whereas the concentrations for individuals with other diseases and gastrinoma overlapped. Based on the Youden indices calculated for G17+G34, G34, and G17, the most specific biomarker was G17 (96.9% clinical specificity at 209.8 pg/mL) for gastrinoma. Conclusions This method should aid in the diagnosis of diseases associated with increased gastrin concentrations.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d2da8ad8ca8f8eaac170e0f89ed99ae9Test
https://doi.org/10.1093/clinchem/hvab097Test

المؤلفون: Shota Kuriyama, Masayuki Kitano, Nobuyuki Higashino, Naoki Kawamura, Akira Ikoma, Nobuyuki Kawai, Hirotatsu Sato, Yuya Funayama, Tetsuo Sonomura, Takao Koyama, Ryota Tanaka, Atsufumi Kamisako, Kodai Fukuda, Daisaku Ito

المصدر: Quant Imaging Med Surg

مصطلحات موضوعية: medicine.medical_specialty, Gastrinoma, business.industry, chemistry.chemical_element, Calcium, Splenic artery, medicine.disease, Gastroenterology, Gastroduodenal artery, medicine.anatomical_structure, chemistry, Internal medicine, medicine.artery, medicine, Duodenum, Radiology, Nuclear Medicine and imaging, Superior mesenteric artery, Pancreas, business, Letter to the Editor, Gastrin

الوصف: Pancreatic gastrinoma is a rare pancreatic neuroendocrine tumor with an incidence of about 0.5–21.5 cases per million people worldwide. The most common sites are the duodenum (75%), pancreas (20%), and other organs (5%). Gastrinomas are malignant in about 60–90% of cases (1-5). Malignant gastrinomas originating beneath the duodenal mucosa often metastasize to the liver, even if the tumor diameter is ≤1 cm. Therefore, in patients with gastrinoma and Zollinger–Ellison syndrome, it is important to determine the number and site(s) of gastrinoma(s), as well as the presence or absence of liver metastases. However, the detection sensitivity of computed tomography (CT) and magnetic resonance imaging is about 10–50%, that of angiography is 20–50%, and that of somatostatin receptor scintigraphy is 30–70%. Tumor size is strongly correlated with the detection sensitivity of any examination modality, and tumors of

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::496152ff1c776dbba3844c2a84909ba0Test
https://doi.org/10.21037/qims-20-1224Test

المؤلفون: Struthers, Jason D., Robl, Nick, Wong, Valerie M., Kiupel, Matti

المساهمون: SAGE Journals

المصدر: Animal, Dairy, and Veterinary Science Student Research

مصطلحات موضوعية: Canis lupus baileyi, dogs, gastrin, gastrinoma, Mexican gray wolf, neuroendocrine tumor, ulcer, Zollinger–Ellison syndrome, Animal Sciences

الوصف: Gastrinoma, an infrequent diagnosis in middle-aged dogs, occurs with nonspecific gastrointestinal morbidity. Laboratory tests can yield a presumptive diagnosis, but definitive diagnosis depends on histopathology and immunohistochemistry. We describe a malignant pancreatic gastrinoma with lymph node metastases and corresponding Zollinger–Ellison syndrome in a Mexican gray wolf (Canis lupus baileyi) and review this endocrine neoplasm in domestic dogs. A 12-y-old, captive, male Mexican gray wolf developed inappetence and weight loss. Abdominal ultrasonography revealed a thickened duodenum and peritoneal effusion. Two duodenal perforations were noted on exploratory celiotomy and were repaired. Persisting clinical signs led to a second celiotomy that revealed a mesenteric mass, which was diagnosed histologically as a neuroendocrine carcinoma. During the following 16 mo, the wolf received a combination of H2-receptor antagonists, proton-pump inhibitors, gastroprotectants, and anti-emetics, but had recurrent episodes of anorexia, nausea, acid reflux, and remained underweight. Worsening clinical signs and weakness prompted euthanasia. The antemortem serum gastrin concentration of 414 ng/L (reference interval: 10–40 ng/L) corroborated hypergastrinemia. Autopsy revealed a mass expanding the right pancreatic limb; 3 parapancreatic mesenteric masses; duodenal ulcers; focal duodenal perforation with septic fibrinosuppurative peritonitis; chronic-active ulcerative esophagitis; and poor body condition. The pancreatic mass was diagnosed histologically as a neuroendocrine carcinoma and the parapancreatic masses as lymph node metastases. Immunohistochemistry of the pancreatic mass was positive for gastrin and negative for glucagon, insulin, pancreatic polypeptide, serotonin, somatostatin, and vasoactive intestinal peptide.

وصف الملف: application/pdf

العلاقة: https://digitalcommons.usu.edu/advs_stures/10Test; https://digitalcommons.usu.edu/context/advs_stures/article/1009/viewcontent/1040638718779638.pdfTest

الإتاحة: https://doi.org/10.1177/1040638718779638Test
https://digitalcommons.usu.edu/advs_stures/10Test
https://digitalcommons.usu.edu/context/advs_stures/article/1009/viewcontent/1040638718779638.pdfTest

المؤلفون: Guarnotta, Valentina, Martini, Chiara, Davì, Maria Vittoria, Pizza, Genoveffa, Colao, Annamaria, Faggiano, Antongiulio

المساهمون: Guarnotta, Valentina, Martini, Chiara, Davì, Maria Vittoria, Pizza, Genoveffa, Colao, Annamaria, Faggiano, Antongiulio

مصطلحات موضوعية: Gastrinoma, neuroendocrine tumour, somatostatin, somatostatin analogue, endocrinology, diabetes and metabolism

الوصف: Purpose Analyze the role of somatostatin analogues (SSAs) in the treatment of sporadic and MEN1-related gastrinomas, trying to define whether recent trials have changed the landscape of gastrinoma therapy. Methods We evaluate the rationale of SSA use in the treatment of gastrinomas, summarize the current literature concerning the effect of SSAs on the control of Zollinger- Ellison syndrome (ZES) and gastrinomas tumor progression and discuss their role in the most recent guidelines. Results The medical treatment of gastrinoma and related ZES is aimed at controlling acid hypersecretion and tumor progression, in inoperable patients. The use of proton pump inhibitors (PPIs) to control the syndrome is a cornerstone in the ZES therapy. SSAs are not usually indicated for antisecretory purpose, because PPIs are considered the treatment of choice, due to their long lasting high efficacy and oral availability. The antiproliferative effect of SSAs has been established by two placebo-controlled trials that have clearly demonstrated a significant increase in progression free survival in patients affected by non-functioning well-differentiated advanced neuroendocrine tumors (NETs). The recent ENETS guidelines recommend the use of SSAs in advanced well differentiated NETs as antiproliferative agents. Conclusions The high sstr-expression in gastrinomas make them highly responsive to SSAs and support the use of such drugs to counteract the tumour growth in patients not amenable to surgical cure. Unfortunately, limited data, mainly case reports or small series, support the use of SSAs in advanced gastrinomas, therefore, it is difficult to quantify their ability to control tumour growth and disease progression

العلاقة: info:eu-repo/semantics/altIdentifier/wos/WOS:000427297400003; volume:60; issue:1; firstpage:15; lastpage:27; numberofpages:13; journal:ENDOCRINE; http://hdl.handle.net/11573/1298799Test; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85030836561

الإتاحة: https://doi.org/10.1007/s12020-017-1420-4Test
http://hdl.handle.net/11573/1298799Test