Glucagonoma
Adenoma, Islet Cell
Erythema
Pancreatic Neoplasms
Tumors or cancer of the PANCREAS. Depending on the types of ISLET CELLS present in the tumors, various hormones can be secreted: GLUCAGON from PANCREATIC ALPHA CELLS; INSULIN from PANCREATIC BETA CELLS; and SOMATOSTATIN from the SOMATOSTATIN-SECRETING CELLS. Most are malignant except the insulin-producing tumors (INSULINOMA).
Glucagon
A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal GLUCAGON-LIKE PEPTIDES. Glucagon is secreted by PANCREATIC ALPHA CELLS and plays an important role in regulation of BLOOD GLUCOSE concentration, ketone metabolism, and several other biochemical and physiological processes. (From Gilman et al., Goodman and Gilman's The Pharmacological Basis of Therapeutics, 9th ed, p1511)
The hypothalamic satiety peptide CART is expressed in anorectic and non-anorectic pancreatic islet tumors and in the normal islet of Langerhans. (1/63)
The hypothalamic satiety peptide CART (cocaine and amphetamine regulated transcript) is expressed at high levels in anorectic rat glucagonomas but not in hypoglycemic insulinomas. However, a non-anorectic metastasis derived from the glucagonoma retained high CART expression levels and produced circulating CART levels comparable to that of the anorectic tumors. Moreover, distinct glucagonoma lines derived by stable HES-1 transfection of the insulinoma caused severe anorexia but retained low circulating levels of CART comparable to that of insulinoma bearing or control rats. Islet tumor associated anorexia and circulating CART levels are thus not correlated, and in line with this peripheral administration of CART (5-50 mg/kg) produced no effect on feeding behavior. In the rat two alternatively spliced forms of CART mRNA exist and quantitative PCR revealed expression of both forms in the hypothalamus, in the different islet tumors, and in the islets of Langerhans. Immunocytochemistry as well as in situ hybridization localized CART expression to the somatostatin producing islet D cell. A potential endocrine/paracrine role of islet CART remains to be clarified. (+info)Cloning and DNA-binding properties of the rat pancreatic beta-cell-specific factor Nkx6.1. (2/63)
The homeodomain (HD) protein Nkx6.1 is the most beta-cell-specific transcription factor known in the pancreas and its function is critical for the formation of the insulin-producing beta-cells. However, the target genes, DNA-binding site, and transcriptional properties of Nkx6.1 are unknown. Using in vitro binding site selection we have identified the DNA sequence of the Nkx6.1 binding site to be TTAATTG/A. A reporter plasmid containing four copies of this sequence is activated by an Nkx6.1HD/VP16 fusion construct. Full-length Nkx6.1 fails to activate this reporter plasmid in spite of robust interaction with the binding site in vitro. Stable expression of Nkx6.1 in the glucagon-producing alpha-cell-like MSL-G-AN cells induces expression of the endogenous insulin gene in a subset of the cell population. The expression of other known beta-cell-specific factors such as Pax4, Pax6, Pdx1, GLUT2 and GLP1-R is unchanged by the introduction of Nkx6.1. (+info)Beta-cell maturation leads to in vitro sensitivity to cytotoxins. (3/63)
Pancreatic beta-cells are more sensitive to several toxins (e.g., streptozotocin, alloxan, cytokines) than the other three endocrine cell types in the islets of Langerhans. Cytokine-induced free radicals in beta-cells may be involved in beta-cell-specific destruction in type 1 diabetes. To investigate if this sensitivity represents an acquired trait during beta-cell maturation, we used two in vitro cultured cell systems: 1) a pluripotent glucagon-positive pre-beta-cell phenotype (NHI-glu) that, after in vivo passage, matures into an insulin-producing beta-cell phenotype (NHI-ins) and 2) a glucagonoma cell-type (AN-glu) that, after stable transfection with pancreatic duodenal homeobox factor-1 (PDX-1), acquires the ability to produce insulin (AN-ins). After exposure to interleukin (IL)-1beta, both of the insulin-producing phenotypes were significantly more susceptible to toxic effects than their glucagon-producing counterparts. Nitric oxide (NO) production was induced in both NHI phenotypes, and inhibition with 0.5 mmol/l N(G)-monomethyl-L-arginine (NMMA) fully protected the cells. In addition, maturation into the NHI-ins phenotype was associated with an acquired dose-dependent sensitivity to the toxic effect of streptozotocin. Our results support the hypothesis that the exquisite sensitivity of beta-cells to IL-1beta and streptozotocin is an acquired trait during beta-cell maturation. These two cell systems will be useful tools for identification of molecular mechanisms involved in beta-cell maturation and sensitivity to toxins in relation to type 1 diabetes. (+info)Chromogranin A expression in hepatocellular carcinoma in a patient with germline MEN1 gene mutation. (4/63)
Hepatocellular carcinoma (HCC) was found in a patient with multiple endocrine neoplasia type 1 (MEN 1). The intriguing finding was that the HCC in the patient was positively stained for chromogranin A (CgA), a cellular marker for endocrine and neuroendocrine tumors. The patient had a pancreas endocrine tumor and type C hepatitis, that made pathological diagnosis of the origin of the tumor complicated. (+info)Mutation and expression analyses reveal differential subcellular compartmentalization of PTEN in endocrine pancreatic tumors compared to normal islet cells. (5/63)
The pathogenesis of sporadic endocrine pancreatic tumors (EPTs) is still primarily unknown. Comparative genomic hybridization studies revealed loss of 10q in a significant number (nine of 31) of EPTs. The tumor suppressor gene PTEN lies on 10q23, and so, is a candidate to play some role in EPT pathogenesis. Germline PTEN mutations are found in Cowden and Bannayan-Riley-Ruvalcaba syndromes, whereas somatic mutations and deletions are found in a variety of sporadic cancers. The mutation and expression status of PTEN in EPTs has not yet been examined. Mutation analysis of the entire coding region of PTEN including splice sites was performed in 33 tumors, revealing one tumor with somatic L182F (exon 6). Loss of heterozygosity of the 10q23 region was detected in eight of 15 informative malignant (53%) and in none of seven benign EPTs. PTEN expression was assessed in 24 available EPTs by immunohistochemistry using a monoclonal anti-PTEN antibody. Of these 24, 23 tumors showed strong immunoreactivity for PTEN. Only the EPTs with PTEN mutation lacked PTEN protein expression. Although normal islet cells always exhibited predominantly nuclear PTEN immunostaining, 19 of 23 EPTs had a predominantly cytoplasmic PTEN expression pattern. Exocrine pancreatic tissue was PTEN-negative throughout. PTEN mutation is a rare event in malignant EPTs and PTEN protein is expressed in most (23 of 24) EPTs. Thus, intragenic mutation or another means of physical loss of PTEN is rarely involved in the pathogenesis of EPTs. Instead, either an impaired transport system of PTEN to the nucleus or some other means of differential compartmentalization could account for impaired PTEN function. Loss of heterozygosity of the 10q23 region is a frequent event in malignant EPTs and might suggest several hypotheses: a different tumor suppressor gene in the vicinity of PTEN might be principally involved in EPT formation; alternatively, 10q loss, including PTEN, seems to be associated with malignant transformation, but the first step toward neoplasia might involve altered subcellular localization of PTEN. (+info)Putative tumor suppressor loci at 6q22 and 6q23-q24 are involved in the malignant progression of sporadic endocrine pancreatic tumors. (6/63)
Our previous comparative genomic hybridization study on sporadic endocrine pancreatic tumors (EPTs) revealed frequent losses on chromosomes 11q, 3p, and 6q. The aim of this study was to evaluate the importance of 6q losses in the oncogenesis of sporadic EPTs and to narrow down the smallest regions of allelic deletion. A multimodal approach combining polymerase chain reaction-based allelotyping, double-target fluorescence in situ hybridization, and comparative genomic hybridization was used in a collection of 109 sporadic EPTs from 93 patients. Nine polymorphic microsatellite markers (6q13 to 6q25-q27) were investigated, demonstrating a loss of heterozygosity (LOH) in 62.2% of the patients. A LOH was significantly more common in tumors >2 cm in diameter than below this threshold as well as in malignant than in benign tumors. We were able to narrow down the smallest regions of allelic deletion at 6q22.1 (D6S262) and 6q23-q24 (D6S310-UTRN) with LOH-frequencies of 50.0% and 41.2 to 56.3%, respectively. Several promising tumor suppressor candidates are located in these regions. Additional fluorescence in situ hybridization analysis on 46 EPTs using three locus-specific probes (6q21, 6q22, and 6q27) as well as a centromere 6-specific probe revealed complete loss of chromosome 6 especially in metastatic disease. We conclude that the two hot spots found on 6q may harbor putative tumor suppressor genes involved not only in the oncogenesis but maybe also in the malignant and metastatic progression of sporadic EPTs. (+info)Necrolytic migratory erythema associated with glucagonoma syndrome: a case report. (7/63)
Necrolytic migratory erythema is a rare skin condition that consists of migrating areas of erythema with blisters that heal with hyperpigmentation. It usually occurs in patients with an alpha islet cell tumor of the pancreas-or glucagonoma-and when associated with glucose intolerance, anemia, hyperglucagonemia, and weight loss defines the glucagonoma syndrome. We describe a 52-year-old female patient with necrolytic migratory erythema associated with glucagonoma syndrome who had metastatic disease at presentation and passed away one week after her admission. The autopsy showed a tumor in the body of the pancreas, which was diagnosed as a neuroendocrine tumor and confirmed by immunohistochemistry. The diagnosis of necrolytic migratory erythema is a matter of great importance, since it might be an auxiliary tool for the early detection of glucagonoma. (+info)Pancreatic vasopressin V1b receptors: characterization in In-R1-G9 cells and localization in human pancreas. (8/63)
Vasopressin (AVP) receptors present in In-R1-G9 cells, a hamster glucagon-secreting alpha-pancreatic cell line, were characterized using SSR-149415, a selective nonpeptide V1b receptor antagonist, and reference AVP compounds. Binding experiments, using [3H]AVP as a ligand, identified a single population of high-affinity binding sites. SSR-149415 competitively inhibited this binding and exhibited nanomolar and stereospecific affinity for these sites. The affinity of various AVP/oxytocin ligands confirmed a V1b binding profile. In functional studies, AVP was a potent stimulant in inducing intracellular Ca2+ increase, glucagon secretion, and cell proliferation. These effects were fully antagonized by SSR-149415 with a nanomolar potency, whereas its diasteroisomer as well as two selective V1a and V2 receptor antagonists were much less potent. Additionally, the order of potency of AVP agonists and antagonists was in agreement with V1b-mediated effects. By RT-PCR, we confirmed the presence of V1b receptor mRNA in both In-R1-G9 cells and in human pancreas. The distribution pattern of V1b receptors investigated in human pancreas by immunohistochemistry showed strong labeling in islets of Langerhans, and colocalization studies indicated that this receptor was expressed in alpha-glucagon, beta-insulin, and somatostatin pancreatic cells. Thus, in In-R1-G9 cells, AVP mediates intracellular Ca2+ increase, glucagon secretion, and cell proliferation by activating V1b receptors, and these effects are potently antagonized by SSR-149415. Moreover, the presence of V1b receptors also found in human Langerhans islets could suggest hormonal control of AVP in human pancreas. (+info)Glucagonoma is a rare pancreatic neuroendocrine tumor that produces and releases excessive amounts of glucagon hormone, leading to a characteristic clinical syndrome known as the Glucagonoma Syndrome, which includes symptoms such as necrolytic migratory erythema, diabetes mellitus, anemia, weight loss, and various gastrointestinal symptoms.
A glucagonoma is a rare type of neuroendocrine tumor that originates from the alpha cells of the pancreas, where the hormone glucagon is produced. This tumor can lead to an overproduction of glucagon, resulting in a characteristic syndrome known as the "glucagonoma syndrome."
The symptoms of glucagonoma syndrome may include:
1. A distinctive rash called necrolytic migratory erythema, which is characterized by red, swollen, and painful skin lesions that can affect various parts of the body.
2. Weight loss
3. Diabetes or high blood sugar levels (hyperglycemia)
4. Anemia
5. Deep vein thrombosis (blood clots in the deep veins)
6. Depression and confusion
7. A decreased appetite
8. Fatigue and weakness
9. Diarrhea or steatorrhea (fatty stools)
10. High levels of amino acids, fatty acids, and zinc in the blood.
Glucagonomas are typically slow-growing tumors, but they can metastasize (spread) to other organs such as the liver, lymph nodes, and bones. Treatment options for glucagonoma may include surgery to remove the tumor, chemotherapy, targeted therapy, or radiation therapy. Regular follow-up care is essential to monitor the tumor's progression and manage any associated symptoms.
An Adenoma, Islet Cell is a benign tumor originating from the islets of Langerhans within the pancreas, which can lead to hormonal imbalances depending on the specific cell type involved.
An islet cell adenoma is a rare, typically benign tumor that develops in the islets of Langerhans, which are clusters of hormone-producing cells in the pancreas. The islets of Langerhans contain several types of cells, including beta cells that produce insulin, alpha cells that produce glucagon, and delta cells that produce somatostatin.
Islet cell adenomas can cause various endocrine disorders depending on the type of hormone-producing cells involved. For example, if the tumor consists mainly of beta cells, it may secrete excessive amounts of insulin, leading to hypoglycemia (low blood sugar). Conversely, if the tumor is composed primarily of alpha cells, it may produce too much glucagon, resulting in hyperglycemia (high blood sugar) and a condition known as glucagonoma.
Islet cell adenomas are usually slow-growing and small but can become quite large in some cases. They are typically diagnosed through imaging tests such as CT scans or MRI, and hormone levels may be measured to determine the type of cells involved. Treatment options include surgical removal of the tumor, medication to manage hormonal imbalances, and, in rare cases, radiofrequency ablation or embolization.
'Erythema' is a clinical term that refers to the reddening of the skin or mucous membranes, often as a result of increased blood flow due to congestion in the capillaries, and can be an indicator of various underlying conditions such as inflammation, infection, or irritation.
Erythema is a term used in medicine to describe redness of the skin, which occurs as a result of increased blood flow in the superficial capillaries. This redness can be caused by various factors such as inflammation, infection, trauma, or exposure to heat, cold, or ultraviolet radiation. In some cases, erythema may also be accompanied by other symptoms such as swelling, warmth, pain, or itching. It is a common finding in many medical conditions and can vary in severity from mild to severe.
Pancreatic neoplasms are abnormal growths of tissue in the pancreas that can be benign or malignant, often characterized by progressive and invasive growth which can interfere with normal endocrine and exocrine functions, leading to serious health consequences if not detected and treated early.
Pancreatic neoplasms refer to abnormal growths in the pancreas that can be benign or malignant. The pancreas is a gland located behind the stomach that produces hormones and digestive enzymes. Pancreatic neoplasms can interfere with the normal functioning of the pancreas, leading to various health complications.
Benign pancreatic neoplasms are non-cancerous growths that do not spread to other parts of the body. They are usually removed through surgery to prevent any potential complications, such as blocking the bile duct or causing pain.
Malignant pancreatic neoplasms, also known as pancreatic cancer, are cancerous growths that can invade and destroy surrounding tissues and organs. They can also spread (metastasize) to other parts of the body, such as the liver, lungs, or bones. Pancreatic cancer is often aggressive and difficult to treat, with a poor prognosis.
There are several types of pancreatic neoplasms, including adenocarcinomas, neuroendocrine tumors, solid pseudopapillary neoplasms, and cystic neoplasms. The specific type of neoplasm is determined through various diagnostic tests, such as imaging studies, biopsies, and blood tests. Treatment options depend on the type, stage, and location of the neoplasm, as well as the patient's overall health and preferences.
Glucagon is a hormone, primarily produced by the alpha cells of the pancreatic islets, that functions to raise blood glucose levels by stimulating the conversion of glycogen to glucose in the liver and increasing gluconeogenesis.
Glucagon is a hormone produced by the alpha cells of the pancreas. Its main function is to regulate glucose levels in the blood by stimulating the liver to convert stored glycogen into glucose, which can then be released into the bloodstream. This process helps to raise blood sugar levels when they are too low, such as during hypoglycemia.
Glucagon is a 29-amino acid polypeptide that is derived from the preproglucagon protein. It works by binding to glucagon receptors on liver cells, which triggers a series of intracellular signaling events that lead to the activation of enzymes involved in glycogen breakdown.
In addition to its role in glucose regulation, glucagon has also been shown to have other physiological effects, such as promoting lipolysis (the breakdown of fat) and inhibiting gastric acid secretion. Glucagon is often used clinically in the treatment of hypoglycemia, as well as in diagnostic tests to assess pancreatic function.
Glucagonoma - Wikipedia
NME has occasionally been observed in people who do not have glucagonoma. People who develop glucagonoma from Mahvash disease ... "Glucagonoma and the glucagonoma syndrome". Oncology Letters. 15 (3): 2749-2755. doi:10.3892/ol.2017.7703. ISSN 1792-1074. PMC ... "Glucagonoma Syndrome", Endotext, MDText.com, Inc., PMID 25905270, retrieved 2019-09-24 "Glucagonoma: Practice Essentials, ... Glucagonoma is a very rare tumor of the pancreatic alpha cells that results in the overproduction of the hormone, glucagon. ...
Glucagonoma: Practice Essentials, Pathophysiology, Epidemiology
Malignant glucagonomas are islet cell pancreatic tumors that are discovered because of glucagonoma syndrome (in which the ... glucagonoma autonomously secretes glucagon), because of local mass effects, or incidentally. ... A glucagonoma is a rare neuroendocrine tumor with nearly exclusive pancreatic localization. ... Glucagonoma and the glucagonoma syndrome. Oncol Lett. 2018 Mar. 15 (3):2749-2755. [QxMD MEDLINE Link]. [Full Text]. ...
Glucagonoma: Background, Pathophysiology, Epidemiology
Malignant glucagonomas are islet cell pancreatic tumors that are discovered because of glucagonoma syndrome (in which the ... glucagonoma autonomously secretes glucagon), because of local mass effects, or incidentally. ... A glucagonoma is a rare neuroendocrine tumor with nearly exclusive pancreatic localization. ... Glucagonoma may also occur as a single microadenoma found incidentally at autopsy in elderly patients. Glucagonoma very rarely ...
Glucagonoma - Gastrointestinal Disorders - MSD Manual Professional Edition
Glucagonoma - Etiology, pathophysiology, symptoms, signs, diagnosis & prognosis from the MSD Manuals - Medical Professional ... Most patients with glucagonoma have glucagon levels > 1000 pg/mL (> 1000 ng/L; normal is < 200 pg/mL [< 200 ng/L]). However, ... Symptoms and Signs of Glucagonoma Because glucagonomas produce glucagon, which raises blood sugar, the symptoms are the same as ... A glucagonoma is a pancreatic alpha-cell tumor that secretes glucagon, causing hyperglycemia and a characteristic rash. ...
necrolytic migratory erythema (glucagonoma syndrome) histopathology - Loma Linda Dermatopathology
Understanding neuroendocrine medical terms
Pancreatic glucagonoma metastasising to the right ovary five years after initial surgery: a case report<...
Pancreatic glucagonoma metastasising to the right ovary five years after initial surgery : a case report. In: Journal of the ... Pancreatic glucagonoma metastasising to the right ovary five years after initial surgery: a case report. / Watt, David Graham; ... Watt, D. G., Pandanaboyana, S., Herrington, C. S., & Tait, I. (2013). Pancreatic glucagonoma metastasising to the right ovary ... Watt DG, Pandanaboyana S, Herrington CS, Tait I. Pancreatic glucagonoma metastasising to the right ovary five years after ...
Glucagonoma Syndrome: A Case Report
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Glucagonoma and the glucagonoma syndrome-cumulative experience with an elusive endocrine tumour. Clin Endocrinol. 2011;74(5): ... Glucagonoma Syndrome: A Case Report Authors. * Teh Roseleen Nadia Roslan Hospital Raja Perempuan Zainab II, Kota Bharu, ... The glucagonoma syndrome: A review of its features and discussion of new perspectives. Am J Med Sci. 2001;321(5):306-20. http ... Glucagonoma syndromes, necrolytic migratory erythema (NME), glucagon, somatostatin Abstract. A 58-year-old Malay female with ...
Hypercalcemia Appeared in a Patient with Glucagonoma Treated with Octreotide Acetate Long-acting Release
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Search Results: Breast, Rash or multiple lesions
Javier Herrera, MD | Edmonds, WA
![PRIME PubMed | [The endocrine pancreas. From the isolated islet to the artificial pancreas (authors transl)]](data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAABAAAAAQCAMAAAAoLQ9TAAAAjVBMVEVHcEyABSSMBCaMBCaMBCaeFTaoGDqjAyqAACGaBCqMBCaMBCbCAC+MAyWlASipFzvEAzKOABP9/PySASTAACaCAAObAAKdACDZvMGpAA/AnqTRcX+pV2WoZnGze4TSrrS+ABTSnKXbyMzk1tqYITiIJTi+jZXqvMPx6+2wDzandXyZT1qrK0XIU2fUi5eD4+TyAAAADHRSTlMARNP5/NP6+UjRVlXxulOyAAAAwUlEQVQYlTWPR5bCMBAFG3hgm7GE1MrJORHvf7yxx0ztqv6mGyA/X25fLuccIL8zVn5h7J5D8a9SbqmAbBf5DmGbsi1I+al9GoZ4axoKtGxCF7UL1naI8xqa2WvXdklwhX6xoGfv3kvSlOp2UbWFiDqgoNToulcCR5jwhdEZU/WVGvonB9EjflprH4pP3cgJUDek9FT81dYPRQiBjJUmVpOvRsVXP/2dbgznXJCNIxzo+pwRYnfyA3AoMkp3Ox2v8AuqgBQxU3E0TgAAAABJRU5ErkJggg==)
PRIME PubMed | [The endocrine pancreas. From the isolated islet to the "artificial pancreas" (author's transl)]
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Primary Reference: Fitzgerald, P.A. and Klonoff, D.C. Hypothalamic and Pituitary Hormones, in Basic and Clinical Pharmacology, (Katzung, B. G., ed) Appleton-Lange, 1998, pp 603-618.. Primary Reference: Biller, Beverly M. K. and Daniels, Gilbert, H. Neuroendocrine Regulation and Diseases of the Anterior Pituitary and Hypothalamus, In Harrisons Principles of Internal Medicine 14th edition, (Isselbacher, K.J., Braunwald, E., Wilson, J.D., Martin, J.B., Fauci, A.S. and Kasper, D.L., eds) McGraw-Hill, Inc (Health Professions Division), 1998, pp 1972-1998. ...
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Table of contents | Heart
SKIN SIGNS OF SYSTEMIC CANCERS | Fitzpatrick's Color Atlas and Synopsis of Clinical Dermatology, 8e | AccessMedicine | McGraw...InsulinomaSyndromeNecrolyticTumorsDiagnosis of glucagonomaTumorMalignantGlucagon receptorCysticSymptomsGlucoseMetastaticMetastasesErythemaDiabetesLesionsCasesDiseaseCellsPatientsGlucagon in the bNeuroendocrineSomatostatinomaInsulinAdenomaRecurrentSurgicalCurativePNETClinicopathologicManifestationsHyperglucagonemiaDiagnosisPeptideTumourDistalDisordersImagingDistinctiveCase reportTypeSurgeryPeople
Insulinoma4
- Glucagonoma accounts for 5% of all PNETs and is the fourth most frequent functioning PNET, following insulinoma, gastrinoma, and vipoma. (bvsalud.org)
- MATERIALS AND METHODS: A review of the literature was performed using the PubMed database and Cochrane library aiming to identify reported cases of concomitant pancreatic insulinoma and glucagonoma. (bvsalud.org)
- RESULTS: A total of 8 cases of concomitant pancreatic insulinoma and glucagonoma were identified, corresponding to the period 1992-2021. (bvsalud.org)
- CONCLUSION: Concomitant insulinoma and glucagonoma are rare and challenging. (bvsalud.org)
Syndrome12
- The presence of glucagonoma syndrome, the symptoms that accompany the pancreatic tumor, as well as elevated levels of glucagon in the blood, are what is used to diagnose glucagonoma. (wikipedia.org)
- When a person presents with a blood glucagon concentration greater than 500 mg/mL along with the glucagonoma syndrome, a diagnosis can be established. (wikipedia.org)
- Malignant glucagonomas are islet cell pancreatic tumors that are discovered because of glucagonoma syndrome (in which the glucagonoma autonomously secretes glucagon), because of local mass effects, or incidentally. (medscape.com)
- Glucagonomas that are not associated with glucagonoma syndrome are diagnosed in various ways. (medscape.com)
- The vast majority of glucagonomas are sporadic (80%) with the remainder associated with multiple endocrine neoplasia type 1 (MEN1) , an inherited tumor predisposition syndrome, or Mahvash disease, an extremely rare cause of familial pancreatic α-cell hyperplasia and glucagonoma due to inactivating mutations in the glucagon receptor ( GCGR ) gene. (medscape.com)
- Glucagonoma very rarely is part of multiple endocrine neoplasia (MEN) type 1 syndrome (also called Wermer syndrome), and in such cases, the glucagonoma appears as a single, biologically inactive lesion. (medscape.com)
- The glucagonoma syndrome: Clinical and pathologic features in 21 patients. (asean-endocrinejournal.org)
- Glucagonoma and the glucagonoma syndrome-cumulative experience with an elusive endocrine tumour. (asean-endocrinejournal.org)
- The glucagonoma syndrome: Clinical features, diagnosis, and treatment. (asean-endocrinejournal.org)
- Necrolytic migratory erythema: A cutaneous clue to glucagonoma syndrome. (asean-endocrinejournal.org)
- The rarity of pseudoglucagonoma syndrome, or necrolytic migratory erythema occurring in the absence of a glucagonoma, warranted the discussion of this case. (cdlib.org)
- Although only having diagnosed the glucagonoma syndrome (GS) once, I have always been fascinated by necrolytic migratory erythema (NME), and conditions that mimic NME, the so-called pseudoglucagonoma syndrome (PGS). (aad.org)
Necrolytic4
- Typically associated with a rash called necrolytic migratory erythema, weight loss, and mild diabetes mellitus, most people with glucagonoma contract it spontaneously. (wikipedia.org)
- Necrolytic migratory erythema associated with glucagonoma: A report of 2 cases. (asean-endocrinejournal.org)
- Necrolytic migratory erythema without glucagonoma associated with hepatitis B. Eur J Dermatol. (asean-endocrinejournal.org)
- Recurrent cutaneous manifestation of GLUCAGONOMA characterized by necrolytic polycyclic migratory lesions with scaling borders. (bvsalud.org)
Tumors3
- Glucagonoma accounts for approximately 1% of neuroendocrine tumors, although this may be an underestimate given that glucagonoma is associated with non-specific symptoms. (wikipedia.org)
- A glucagonoma is a rare neuroendocrine tumor that originates almost exclusively in the pancreas and probably accounts for 1% of all neuroendocrine tumors. (medscape.com)
- The correct recognition of NME is very important, because it may allow early detection either of glucagonoma or of extrapancreatic, glucagon-secreting tumors. (medscape.com)
Diagnosis of glucagonoma1
- It is important to note that not all cases of hyperglucagonemia will lead to a diagnosis of glucagonoma. (wikipedia.org)
Tumor5
- Glucagonoma is a very rare tumor of the pancreatic alpha cells that results in the overproduction of the hormone, glucagon. (wikipedia.org)
- A glucagonoma is a rare neuroendocrine tumor with nearly exclusive pancreatic localization. (medscape.com)
- A glucagonoma is a pancreatic alpha-cell tumor that secretes glucagon , causing hyperglycemia and a characteristic rash. (msdmanuals.com)
- A glucagonoma happens when a tumor arises among a group of glucagon-producing cells. (hopkinsmedicine.org)
- These findings suggest a rare glucagonoma-like NET appendiceal tumor that had metastasized to the surface of ovary and were unresponsive to CRC chemotherapy regimens. (edu.au)
Malignant2
- In 75-80% of cases, the glucagonoma starts in malignant form, and in 50% of these cases, metastasis exists at diagnosis. (medscape.com)
- Malignant glucagonoma of the pancreas diagnosed through anemia and diabetes mellitus. (asean-endocrinejournal.org)
Glucagon receptor1
- Additionally, those with Mahvash disease have an increased risk for glucagonoma, as the glucagon receptor gene (GCGR) is mutated. (wikipedia.org)
Cystic1
- Malign cystic glucagonoma presented with diabetic ketoacidosis: Case report with an update. (asean-endocrinejournal.org)
Symptoms2
- Glucagonoma-associated neuropsychiatric and affective symptoms: Diagnostic dilemmas raised by paraneoplastic phenomena. (asean-endocrinejournal.org)
- Glucagonoma leads to diabetes-like symptoms and other severe symptoms, including: high blood sugar. (shakuhachi.net)
Glucose1
- Weight loss, the most commonly associated effect with glucagonoma, results from the glucagon hormone, which prevents the uptake of glucose by somatic cells. (wikipedia.org)
Metastatic1
- In contrast localised glucagonoma without metastatic spread may have prolonged disease free survival with radical resectional surgery. (dundee.ac.uk)
Metastases1
- Cervical metastases of glucagonoma in a patient with multiple endocrine neoplasia type 1: Report of a case. (asean-endocrinejournal.org)
Erythema1
- Although classically associated with a pancreatic glucagonoma, this patient experienced this figurate erythema in the setting of fatty liver disease with no glucagonoma. (cdlib.org)
Diabetes1
- Diabetes is not present in all cases of glucagonoma, but does frequently result from the insulin and glucagon imbalance. (wikipedia.org)
Lesions1
- NME presents in about 70% of cases of glucagonoma, and is characterized by erythematous lesions over the distal extremities and the groin area. (wikipedia.org)
Cases3
- Fewer than 251 cases of glucagonoma have been described in the literature since their first description by Becker in 1942. (wikipedia.org)
- The causes of sporadic glucagonomoas remain unknown, and it is noteworthy that some cases of NME without glucagonoma have been reported. (medscape.com)
- However, it is noteworthy that some cases of NME without glucagonoma have been reported. (medscape.com)
Disease1
- People who develop glucagonoma from Mahvash disease also do not develop NME, implying that working glucagon receptors are needed in order for NME to be present in a person. (wikipedia.org)
Cells1
- Glucagonoma results from the overproduction of glucagon, a peptide hormone located in the pancreatic alpha cells. (wikipedia.org)
Patients1
- Glucagonoma may also occur as a single microadenoma found incidentally at autopsy in elderly patients. (medscape.com)
Glucagon in the b1
- The diagnosis of glucagonoma is made by identifying high levels of glucagon in the blood. (msdmanuals.com)
Neuroendocrine2
- Glucagonoma accounts for approximately 1% of neuroendocrine tumors, although this may be an underestimate given that glucagonoma is associated with non-specific symptoms. (wikipedia.org)
- Pancreatic glucagonoma is a well-known rare subtype of pancreatic neuroendocrine tumors (PNETs). (sgo-iasgo.com)
Somatostatinoma1
- Usually it involves the non-insulin-producing cell types, the pancreatic alpha cells and the pancreatic delta cells (somatostatin-secreting cells) in glucagonoma and somatostatinoma, respectively. (icd10data.com)
Insulin2
- Diabetes is not present in all cases of glucagonoma, but does frequently result from the insulin and glucagon imbalance. (wikipedia.org)
- Patients with glucagonoma may experience diabetes because of a lack of equilibrium between levels of insulin production and glucagon production. (diabetessa.org.za)
Adenoma1
- Glucagonoma syndrome with serous oligocystic adenoma: A rare case report. (medscape.com)
Recurrent1
- However, no treatment strategy has been established for recurrent pancreatic glucagonoma. (sgo-iasgo.com)
Surgical2
- Often, NME resolves rapidly with surgical resection of a glucagonoma or with a potent inhibitor of glucagon release and glucagon action, such as somatostatin. (medscape.com)
- The rarity of this disease has hindered the discovery of therapeutic options, including drugs or surgical treatments for the recurrence of pancreatic glucagonoma. (sgo-iasgo.com)
Curative3
- The only curative therapy for glucagonoma is surgery, and even this is not always successful. (wikipedia.org)
- This study reports a rare case of complete resection for multiple metachronous liver metastases from a pancreatic glucagonoma after curative surgery. (sgo-iasgo.com)
- Thus, we report a rare case of conversion surgery for multiple metachronous liver metastases from a pancreatic glucagonoma after curative surgery. (sgo-iasgo.com)
PNET1
- In most cases, symptomatic pancreatic glucagonoma, a subtype of PNET, is diagnosed based on the chief complaints of skin rash, necrotizing migratory erythema (NME), and weight loss. (sgo-iasgo.com)
Clinicopathologic1
- Glucagonoma syndrome is an uncommon clinicopathologic entity. (medscape.com)
Manifestations1
- Clinicians should be aware of the unusual initial manifestations of glucagonoma. (nih.gov)
Hyperglucagonemia2
- It is important to note that not all cases of hyperglucagonemia will lead to a diagnosis of glucagonoma. (wikipedia.org)
- However, publications have reported that neither glucagonoma nor hyperglucagonemia is necessary for NME. (medscape.com)
Diagnosis1
- When a person presents with a blood glucagon concentration greater than 500 mg/mL along with the glucagonoma syndrome, a diagnosis can be established. (wikipedia.org)
Peptide2
- Glucagonoma results from the overproduction of glucagon, a peptide hormone located in the pancreatic alpha cells. (wikipedia.org)
- Enhanced glucagon-like peptide-1 secretion in a patient with glucagonoma: implications for glucagon-like peptide-1 secretion from pancreatic a cells in vivo. (medscape.com)
Tumour1
- A glucagonoma is a very rare pancreatic islet cell tumour secreting excessive amounts of glucagon. (endocrinesurgeon.co.uk)
Distal2
- NME presents in about 70% of cases of glucagonoma, and is characterized by erythematous lesions over the distal extremities and the groin area. (wikipedia.org)
- We report the case of a patient who underwent distal pancreatectomy for primary pancreatic glucagonoma and developed multiple hepatic metastatic recurrences five years after the primary surgery. (sgo-iasgo.com)
Disorders1
- Fang S, Li S, Cai T. Glucagonoma syndrome: a case report with focus on skin disorders. (medscape.com)
Imaging1
- Imaging features of glucagonoma syndrome: A case report and review of the literature. (medscape.com)
Distinctive1
- Distinctive dermatosis of the glucagonoma syndrome. (nih.gov)
Case report1
- 10. Glucagonoma syndrome: case report and literature review. (nih.gov)
Type1
- Multiple endocrine neoplasia type 1 includes glucagonoma as a component. (medscape.com)
Surgery1
- 7. Metabolic studies and glucagon gel filtration pattern before and after surgery in a case of glucagonoma syndrome. (nih.gov)
People5
- NME has occasionally been observed in people who do not have glucagonoma. (wikipedia.org)
- People who develop glucagonoma from Mahvash disease also do not develop NME, implying that working glucagon receptors are needed in order for NME to be present in a person. (wikipedia.org)
- About 60% of people diagnosed with glucagonoma are women. (wikipedia.org)
- People who are diagnosed with sporadic glucagonoma often have an increased mortality rate compared to those with MEN1, as the latter group will go to the doctor for periodic visits. (wikipedia.org)
- The estimated prevalence of glucagonoma is 1 in 20, 000, 000 people. (endocrinesurgeon.co.uk)