MCID: GLC036
MIFTS: 46

Glucagonoma

Categories: Endocrine diseases, Gastrointestinal diseases, Rare diseases

Aliases & Classifications for Glucagonoma

MalaCards integrated aliases for Glucagonoma:

Name: Glucagonoma 20 58 54 71
Pancreatic Glucagonoma 20 17
Glucagonoma Syndrome 20 58

Characteristics:

Orphanet epidemiological data:

58
glucagonoma
Inheritance: Not applicable; Prevalence: <1/1000000 (Europe); Age of onset: Adult; Age of death: adult;

Classifications:

Orphanet: 58  
Rare gastroenterological diseases
Rare endocrine diseases


Summaries for Glucagonoma

GARD : 20 The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.Orpha Number: 97280DefinitionGlucagonoma is a rare, functioning type of pancreatic neuroendocrine tumor (PNET; see this term) that hypersecretes glucagon, leading to a syndrome comprised of necrolytic migratory erythema, diabetes mellitus, anemia, weight loss, mucosal abnormalities, thromboembolism, gastrointestinal and neuropsychiatric symptoms.EpidemiologyThe estimated incidence in the general population is 1/20, 000,000.Clinical descriptionGlucagonoma usually presents in the fifth decade of life with the initial symptom often being necrolytic migratory erythema. This skin condition is characterized by a red, blistering and migratory rash, associated with an intense pruritus and that is mainly localized to the lower extremities and the groin. Diabetes is present in most cases and requires insulin therapy. Weight loss, anemia, mucosal abnormalities (glossitis, cheilitis, stomatitis), gastrointestinal disturbances, thromboembolism and neuropsychiatric symptoms (depression) are other frequent manifestations. Most glucagonomas have already metastasized by the time that they are diagnosed, mainly to the liver. In some cases glucagonoma may be associated with multiple endocrine neoplasia type 1 (MEN1; see this term). Glucogonoma can be non-functioning in rare cases.EtiologyThe etiology is unknown. These tumors of 2-25 cm mainly occur in the tail of the pancreas. They synthesize and secrete glucagon, which is responsible for balancing the effects of insulin, and is therefore essential in regulating blood sugar levels.Diagnostic methodsDiagnosis is based on clinical findings and endocrine tests. Serum glucagon levels are markedly elevated (>500 pg/mL) and levels of more than 1000 pg/mL are considered diagnostic if the patient also displays features of glucagonoma syndrome. Other hormones such as insulin, somatostatin and vasoactive intestinal peptide may also be elevated. Levels of blood chromogranin A are increased. Localization of tumors is possible by computed tomography (CT) scan, octreotide scan, magnetic resonance imaging (MRI) and/or endoscopic ultrasound.Differential diagnosisDifferential diagnoses include familial hyperglucagonemia, autoimmune and hereditary chronic pancreatitis, Mahvash disease, acrodermatitis enteropathica (see these terms) and cirrhosis.Management and treatmentSomatostatin analogues (octreotide or lanreotide) are usually effective in leading to a remission of rash in most patients as well as improving the symptoms of weight loss, abdominal pain and diarrhea. Glycemia is managed by insulin injections or anti-diabetic drugs. Parenteral nutrition (with essential fatty acids, amino acids, vitamins and minerals) may also be necessary. Surgical resection is the only curative option in localized cases. Surgical debulking followed by chemotherapy may be an option in extensive tumors. Resection of the lymph nodes, spleen and parts of the liver containing metastasized glocagonoma may also be necessary. As thromboembolic complications can occur, blood thinners (antiplatelets and anticoagulants) may be recommended.PrognosisThe prognosis of glucagonoma is usually poor as most have metastasized by the time that they are discovered. Those associated with MEN1 usually have a fairer prognosis as they are diagnosed sooner.Visit the Orphanet disease page for more resources.

MalaCards based summary : Glucagonoma, also known as pancreatic glucagonoma, is related to insulinoma and hyperparathyroidism, and has symptoms including diarrhea An important gene associated with Glucagonoma is SST (Somatostatin), and among its related pathways/superpathways are Salivary secretion and Gastric acid production. The drugs Fentanyl and Levoleucovorin have been mentioned in the context of this disorder. Affiliated tissues include pancreas, liver and spleen, and related phenotypes are neoplasm of the pancreas and nausea and vomiting

Wikipedia : 74 Glucagonoma is a very rare tumor of the pancreatic alpha cells that results in the overproduction of the... more...

Related Diseases for Glucagonoma

Diseases related to Glucagonoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 210)
# Related Disease Score Top Affiliating Genes
1 insulinoma 29.9 SST SCT GAST CHGA
2 hyperparathyroidism 29.8 SCT GAST CHGA
3 primary hyperparathyroidism 29.8 GAST CHGA
4 diarrhea 29.8 SST SCT GAST
5 peptic ulcer disease 29.8 SST SCT GAST
6 zollinger-ellison syndrome 29.7 SST SCT GAST CHGA
7 angiodysplasia 29.7 SST SCT
8 cholelithiasis 29.7 SST GAST
9 short bowel syndrome 29.7 SST GAST
10 pituitary adenoma 29.7 SST CHGB CHGA
11 parathyroid adenoma 29.6 CHGB CHGA
12 secretory diarrhea 29.5 VIP SST
13 pituitary adenoma, prolactin-secreting 29.4 SST CHGB CHGA
14 paraganglioma 29.4 SST CHGB CHGA
15 islet cell tumor 29.3 VIP SST SCT GAST CHGA
16 somatostatinoma 29.2 VIP SST GAST CHGA
17 multiple endocrine neoplasia, type i 29.1 VIP SST SCT GAST CHGA
18 gastrinoma 29.0 VIP SST SCT GAST CHGA
19 duodenal gastrinoma 29.0 SST SCT GAST CHGA
20 gastritis 28.9 SST SCT GAST CHGA
21 neuroendocrine tumor 28.8 VIP SST SCT GAST CHGB CHGA
22 multiple endocrine neoplasia 28.8 VIP SST SCT GAST CHGB CHGA
23 pheochromocytoma 28.7 VIP SST CHGB CHGA
24 vipoma 28.7 VIP SST SCT GAST CHGA
25 appendiceal l-cell glucagon-like peptide producing tumor 11.0
26 exanthem 10.6
27 glossitis 10.5
28 stomatitis 10.4
29 glucose intolerance 10.3
30 cheilitis 10.3
31 hypoglycemia 10.2
32 dermatitis 10.2
33 ampulla of vater neoplasm 10.2 SST CHGA
34 carotid body cancer 10.2 SST CHGA
35 duodenal benign neoplasm 10.1 SST CHGA
36 laryngeal neuroendocrine tumor 10.1 SST CHGA
37 gastric hemangioma 10.1 SST CHGA
38 angular cheilitis 10.1
39 acrodermatitis 10.1
40 hyperglycemia 10.1
41 ectopic cushing syndrome 10.1 SST CHGA
42 carcinoid syndrome 10.1 SST CHGA
43 small intestine benign neoplasm 10.1 SST CHGA
44 pulmonary neuroendocrine tumor 10.1 SST CHGA
45 meckel diverticulum 10.1 SST CHGA
46 adrenal medulla cancer 10.1 SST CHGA
47 pancreatoblastoma 10.1 SST CHGA
48 small cell carcinoma 10.1 GAST CHGA
49 pituitary carcinoma 10.1 SST CHGA
50 postgastrectomy syndrome 10.1 SST GAST

Graphical network of the top 20 diseases related to Glucagonoma:



Diseases related to Glucagonoma

Symptoms & Phenotypes for Glucagonoma

Human phenotypes related to Glucagonoma:

58 31 (show all 43)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 neoplasm of the pancreas 58 31 hallmark (90%) Very frequent (99-80%) HP:0002894
2 nausea and vomiting 58 31 frequent (33%) Frequent (79-30%) HP:0002017
3 constipation 58 31 frequent (33%) Frequent (79-30%) HP:0002019
4 diabetes mellitus 58 31 frequent (33%) Frequent (79-30%) HP:0000819
5 hepatomegaly 58 31 frequent (33%) Frequent (79-30%) HP:0002240
6 anorexia 58 31 frequent (33%) Frequent (79-30%) HP:0002039
7 chronic fatigue 58 31 frequent (33%) Frequent (79-30%) HP:0012432
8 skin rash 58 31 frequent (33%) Frequent (79-30%) HP:0000988
9 weight loss 58 31 frequent (33%) Frequent (79-30%) HP:0001824
10 acanthocytosis 58 31 frequent (33%) Frequent (79-30%) HP:0001927
11 pruritus 58 31 frequent (33%) Frequent (79-30%) HP:0000989
12 abnormal blistering of the skin 58 31 frequent (33%) Frequent (79-30%) HP:0008066
13 glossitis 58 31 frequent (33%) Frequent (79-30%) HP:0000206
14 diarrhea 58 31 frequent (33%) Frequent (79-30%) HP:0002014
15 poor appetite 58 31 frequent (33%) Frequent (79-30%) HP:0004396
16 episodic abdominal pain 58 31 frequent (33%) Frequent (79-30%) HP:0002574
17 normochromic anemia 58 31 frequent (33%) Frequent (79-30%) HP:0001895
18 stomatitis 58 31 frequent (33%) Frequent (79-30%) HP:0010280
19 necrolytic migratory erythema 58 31 frequent (33%) Frequent (79-30%) HP:0031181
20 depressivity 58 31 occasional (7.5%) Occasional (29-5%) HP:0000716
21 ascites 58 31 occasional (7.5%) Occasional (29-5%) HP:0001541
22 gastrointestinal hemorrhage 58 31 occasional (7.5%) Occasional (29-5%) HP:0002239
23 intestinal obstruction 58 31 occasional (7.5%) Occasional (29-5%) HP:0005214
24 steatorrhea 58 31 occasional (7.5%) Occasional (29-5%) HP:0002570
25 intrahepatic cholestasis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001406
26 thromboembolism 58 31 occasional (7.5%) Occasional (29-5%) HP:0001907
27 extrahepatic cholestasis 58 31 occasional (7.5%) Occasional (29-5%) HP:0012334
28 intermittent jaundice 58 31 occasional (7.5%) Occasional (29-5%) HP:0001046
29 abnormal gastrointestinal motility 58 31 occasional (7.5%) Occasional (29-5%) HP:0030895
30 lack of bowel sounds 58 31 occasional (7.5%) Occasional (29-5%) HP:0030145
31 increased circulating gonadotropin level 58 31 very rare (1%) Very rare (<4-1%) HP:0000837
32 hypercalcemia 58 31 very rare (1%) Very rare (<4-1%) HP:0003072
33 abnormality of the thyroid gland 58 31 very rare (1%) Very rare (<4-1%) HP:0000820
34 growth hormone excess 58 31 very rare (1%) Very rare (<4-1%) HP:0000845
35 subcutaneous lipoma 58 31 very rare (1%) Very rare (<4-1%) HP:0001031
36 parathyroid adenoma 58 31 very rare (1%) Very rare (<4-1%) HP:0002897
37 adrenocortical adenoma 58 31 very rare (1%) Very rare (<4-1%) HP:0008256
38 pituitary adenoma 58 31 very rare (1%) Very rare (<4-1%) HP:0002893
39 primary hyperparathyroidism 58 31 very rare (1%) Very rare (<4-1%) HP:0008200
40 increased circulating prolactin concentration 58 31 very rare (1%) Very rare (<4-1%) HP:0000870
41 increased circulating cortisol level 31 very rare (1%) HP:0003118
42 hypercortisolism 58 Very rare (<4-1%)
43 abnormality of abdomen morphology 58 Occasional (29-5%)

UMLS symptoms related to Glucagonoma:


diarrhea

Drugs & Therapeutics for Glucagonoma

Drugs for Glucagonoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 80)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
2
Levoleucovorin Approved, Investigational Phase 2, Phase 3 68538-85-2 149436
3
leucovorin Approved Phase 2, Phase 3 58-05-9 6006
4
Fluorouracil Approved Phase 2, Phase 3 51-21-8 3385
5
Oxaliplatin Approved, Investigational Phase 2, Phase 3 61825-94-3 5310940 9887054 6857599 43805
6
Bevacizumab Approved, Investigational Phase 2, Phase 3 216974-75-3
7
Folic acid Approved, Nutraceutical, Vet_approved Phase 2, Phase 3 59-30-3 6037
8 Pancreatic Polypeptide Investigational Phase 2, Phase 3 59763-91-6
9 Cola Phase 3
10 Anesthetics Phase 3
11 Narcotics Phase 3
12 Analgesics, Opioid Phase 3
13 Analgesics Phase 3
14 Anesthetics, Intravenous Phase 3
15 Anesthetics, General Phase 3
16 Angiogenesis Inhibitors Phase 2, Phase 3
17 Micronutrients Phase 2, Phase 3
18 Antineoplastic Agents, Immunological Phase 2, Phase 3
19 Nutrients Phase 2, Phase 3
20 Protective Agents Phase 2, Phase 3
21 Immunosuppressive Agents Phase 2, Phase 3
22 Trace Elements Phase 2, Phase 3
23 Vitamin B9 Phase 2, Phase 3
24 Vitamin B Complex Phase 2, Phase 3
25 Vitamins Phase 2, Phase 3
26 Immunologic Factors Phase 2, Phase 3
27 Folate Phase 2, Phase 3
28 Antidotes Phase 2, Phase 3
29
Irinotecan Approved, Investigational Phase 2 97682-44-5, 100286-90-6 60838
30
Melphalan Approved Phase 2 148-82-3 4053 460612
31
Streptozocin Approved, Investigational Phase 2 18883-66-4 29327
32
Cisplatin Approved Phase 2 15663-27-1 84093 441203 2767
33
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
34
Gefitinib Approved, Investigational Phase 2 184475-35-2 123631
35
Clotrimazole Approved, Vet_approved Phase 2 23593-75-1 2812
36
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
37
Pancrelipase Approved, Investigational Phase 2 53608-75-6
38
Panobinostat Approved, Investigational Phase 2 404950-80-7 6918837
39
Gemcitabine Approved Phase 2 95058-81-4 60750
40
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
41
Romidepsin Approved, Investigational Phase 2 128517-07-7 5352062
42
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030
43
Everolimus Approved Phase 2 159351-69-6 6442177 70789204
44
Vitamin A Approved, Nutraceutical, Vet_approved Phase 2 68-26-8, 11103-57-4 445354
45
Imetelstat Investigational Phase 2 868169-64-6
46 Anti-Infective Agents Phase 2
47 Retinol palmitate Phase 2
48 retinol Phase 2
49
Motesanib diphosphate Phase 2 850649-62-6 11450633
50 topoisomerase I inhibitors Phase 2

Interventional clinical trials:

(show all 34)
# Name Status NCT ID Phase Drugs
1 A Randomized, Double-blind, Placebo-controlled Multi-center Study to Evaluate the Safety and Efficacy of Fentanyl Sublingual Spray (Fentanyl SL Spray) for the Treatment of Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
2 A Pilot Study of FOLFOX in Combination With Bevacizumab in Patients With Advanced Neuroendocrine Tumors Terminated NCT00227617 Phase 2, Phase 3 5-fluorouracil;leucovorin;oxaliplatin
3 Phase II Trial Of Thalidomide In Patients With Low Grade Neuroendocrine Tumors (Carcinoid and Islet Cell Cancers) Completed NCT00027638 Phase 2 thalidomide
4 A Phase II Clinical and Biologic Study of AMG 706 and Octreotide in Patients With Low-Grade Neuroendocrine Tumors Completed NCT00427349 Phase 2 AMG 706;octreotide
5 Study of First-Line Therapy Comprising Leucovorin Calcium, Fluorouracil, and Irinotecan (FOLFIRI) in Patients With Progressive Locally Advanced or Metastatic Duodenal-Pancreatic Endocrine Tumors Completed NCT00416767 Phase 2 fluorouracil;irinotecan hydrochloride;leucovorin calcium
6 A Phase II Study Of Hepatic Arterial Infusion Of Melphalan With Venous Filtration Via Peripheral Hepatic Perfusion (PHP) For Unresectable Primary And Metastatic Cancers Of The Liver Completed NCT00096083 Phase 2 isolated perfusion;melphalan
7 A Randomised Phase II Study Comparing Capecitabine Plus Streptozocin With or Without Cisplatin Chemotherapy as Treatment for Unresectable or Metastatic Neuroendocrine Tumors Completed NCT00602082 Phase 2 capecitabine;cisplatin;streptozocin
8 Phase II Study of PS-341 in Metastatic Neuroendocrine Tumors Completed NCT00017199 Phase 2 bortezomib
9 A Phase II Trial of Bay 43-9006 in Progressive Metastatic Neuroendocrine Tumors Completed NCT00131911 Phase 2 sorafenib tosylate
10 A Phase 2 Study of GW786034 (Pazopanib) in Advanced Low-Grade or Intermediate-Grade Neuroendocrine Carcinoma Completed NCT00454363 Phase 2 Pazopanib Hydrochloride
11 A Phase II Trial of ZD1839 (Iressa®) in Metastatic Neuroendocrine Tumors Completed NCT00075439 Phase 2 gefitinib
12 A Phase 2 Trial of Temsirolimus and Bevacizumab in Patients With Endometrial, Ovarian, Hepatocellular Carcinoma, Carcinoid or Islet Cell Cancer Completed NCT01010126 Phase 2 Temsirolimus
13 Phase II Study of Single Agent Regorafenib in Patients With Advanced/Metastatic Neuroendocrine Tumors Completed NCT02259725 Phase 2 regorafenib
14 A Randomized Phase II Study of Temozolomide or Temozolomide and Capecitabine in Patients With Advanced Pancreatic Neuroendocrine Tumors Active, not recruiting NCT01824875 Phase 2 temozolomide;capecitabine
15 A Phase 2 Study of Capecitabine, Temozolomide, and Bevacizumab for Metastatic or Unresectable Pancreatic Neuroendocrine Tumors Active, not recruiting NCT01525082 Phase 2 Capecitabine;Temozolomide
16 Clinical Trial of Sir-Spheres® in Patients With Symptomatic or Progressive Hepatic Metastases From Neuroendocrine Tumors Terminated NCT00466856 Phase 2 octreotide acetate
17 Phase II Study of Panobinostat (LBH589) Given in Combination With Bortezomib (Velcade) in Patients With Pancreatic Cancer Progressing on Gemcitabine Therapy Alone or Gemcitabine in Combination Terminated NCT01056601 Phase 2 Bortezomib;Panobinostat
18 Phase II Study of Depsipeptide in Metastatic Neuroendocrine Tumors Terminated NCT00084461 Phase 2 romidepsin
19 A Randomized, Double-Blinded, Placebo-Controlled Phase II Study of Adjuvant Everolimus Following the Resection of Metastatic Pancreatic Neuroendocrine Tumors to the Liver Terminated NCT02031536 Phase 2 everolimus
20 Phase II Evaluation of Real-Time, Pharmacokinetically Guided Everolimus in Patients With Hormone Receptor Positive Breast Cancer, Pancreatic Neuroendocrine Tumors (PNET), and Renal Cell Carcinoma Terminated NCT02273752 Phase 2 Everolimus
21 Phase II Study of Vatalanib and Octreotide in Patients With Progressive Low-Grade Neuroendocrine Tumors Withdrawn NCT00227773 Phase 2 octreotide acetate;vatalanib
22 Phase II Study of Dovitinib (TKI-258) in Progressive, Well-Differentiated Pancreatic Neuroendocrine Tumors With and Without Prior VEGF-Inhibitor Therapy Withdrawn NCT02108782 Phase 2 dovitinib lactate
23 A Phase I, Open-Label, Maximum Tolerated Single-Cycle and Four-Cycle Dose-Finding Study to Evaluation the Safety and Tolerability of 90Y-SMT 487 Administered by Intravenous Infusion to Subjects With Refractory Somatostatin-Receptor Positive Tumors Completed NCT00006368 Phase 1
24 A Phase I Study of Intraperitoneal Oxaliplatin Alone and in Combination With Intraperitoneal Floxuridine and Leucovorin in Patients With Advanced Metastatic Cancer Confined to the Peritoneal Cavity Completed NCT00005860 Phase 1 floxuridine;leucovorin calcium;oxaliplatin
25 A Phase I, Pharmacokinetic, and Biologic Correlative Study of R115777 (NSC 702818) and Herceptin in Patients With Advanced Cancer Completed NCT00005842 Phase 1 tipifarnib
26 A Phase I Trial of Herceptin and Interleukin-12 Completed NCT00004074 Phase 1
27 Phase I Study of Anti-IGF-1R Monoclonal Antibody, IMC-A12, and mTOR Inhibitor, Everolimus, in Advanced Low to Intermediate Grade Neuroendocrine Carcinoma Completed NCT01204476 Phase 1 Everolimus;Octreotide Acetate
28 A Phase I Trial of the mTOR Inhibitor RAD001 in Combination With VEGF Receptor Tyrosine Kinase Inhibitor PTK787/ZK 222584 in Patients With Advanced Solid Tumors Completed NCT00655655 Phase 1 everolimus;vatalanib
29 Phase I Feasibility Trial To Study The Safety Of Sealing Resected Pancreatic Surfaces After Partial Distal Pancreatectomy Using Laser Tissue Welding Recruiting NCT03147768 Phase 1
30 A Phase I Study of [111In-DTPA-D-Phe]-Octreotide in Patients With Refractory Malignancies Expressing Somatostatin Receptors Terminated NCT00002947 Phase 1
31 A Phase 1 Study of Veliparib (ABT-888) in Combination With Capecitabine and Temozolomide in Advanced Well-Differentiated Neuroendocrine Tumors Withdrawn NCT02831179 Phase 1 Capecitabine;Temozolomide;Veliparib
32 Clinical and Psychometric Validation of a Disease-Specific Questionnaire Module in Assessing the Quality of Life of Patients With G.I.-Related Neuroendocrine Tumours Unknown status NCT00454376
33 Evaluation of the Safety and Sensitivity of 68Ga-DOTATOC PET/CT for Imaging NET Patients Recruiting NCT03583528
34 Treatment of Patients With Hepatic Neuroendocrine Metastases Using Drug-Eluting Bead Embolization Terminated NCT00730483 PVA microporous hydrospheres/doxorubicin hydrochloride

Search NIH Clinical Center for Glucagonoma

Genetic Tests for Glucagonoma

Anatomical Context for Glucagonoma

MalaCards organs/tissues related to Glucagonoma:

40
Pancreas, Liver, Spleen, Pancreatic Islet, Pituitary, Thyroid, Endothelial

Publications for Glucagonoma

Articles related to Glucagonoma:

(show top 50) (show all 798)
# Title Authors PMID Year
1
Duodeno-pancreatic neuroendocrine tumours. 61 54
19708940 2010
2
[Glucagonoma without glucagonoma syndrome]. 54 61
20499510 2010
3
Glucagonoma diagnosed by arterial stimulation and venous sampling (ASVS). 61 54
19525592 2009
4
Relative contribution of PDX-1, MafA and E47/beta2 to the regulation of the human insulin promoter. 54 61
15862113 2005
5
[Diabetes mellitus and pancreatic tumor]. 54 61
14718149 2004
6
Ghrelin expression in islet cell tumors: augmented expression of ghrelin in a case of glucagonoma with multiple endocrine neoplasm type I. 61 54
12414844 2002
7
Surgical experience with functioning pancreatic neuroendocrine tumors. 54 61
12206598 2002
8
Somatostatin analogues in the treatment of endocrine tumors of the gastrointestinal tract. 61 54
12472080 2002
9
Identification of somatic mutations of the MEN1 gene in sporadic endocrine tumours. 54 61
10993646 2000
10
Beta-cell maturation leads to in vitro sensitivity to cytotoxins. 54 61
10580420 1999
11
In vivo and in vitro effects of somatostatin and insulin on glucagon release in a human glucagonoma. 54 61
9231061 1997
12
[Glucagonoma: case report and literature review]. 61 54
9201330 1996
13
The glucagonoma syndrome. Clinical and pathologic features in 21 patients. 54 61
8606627 1996
14
Calcium reverses octreotide inhibition of insulin and glucagon levels in patients with insulinoma and glucagonoma. 61 54
8813473 1996
15
Case report: diabetic ketoacidosis in a patient with glucagonoma. 61 54
7771503 1995
16
Functional characterization of somatostatin receptors expressed on hamster glucagonoma cells. 54 61
7840180 1995
17
Glucagon-like peptide-1 (GLP-1) molecular forms in human pancreatic endocrine tumors resemble those in intestine rather than pancreas. 61 54
7835211 1994
18
Expression, intracellular localization, and gene transcription regulation of the secretory protein 7B2 in endocrine pancreatic cell lines and human insulinomas. 61 54
7517367 1994
19
Identification of somatostatin receptor subtypes and an implication for the efficacy of somatostatin analogue SMS 201-995 in treatment of human endocrine tumors. 54 61
8132773 1994
20
Expression of chromogranin A and B and secretoneurin immunoreactivity in neoplastic and nonneoplastic pancreatic alpha cells. 61 54
7952497 1994
21
The long-acting somatostatin analogue octreotide alleviates symptoms by reducing posttranslational conversion of prepro-glucagon to glucagon in a patient with malignant glucagonoma, but does not prevent tumor growth. 61 54
8186658 1994
22
The dissociation of tumor-induced weight loss from hypoglycemia in a transplantable pluripotent rat islet tumor results in the segregation of stable alpha- and beta-cell tumor phenotypes. 61 54
8404649 1993
23
Identification of a transcriptional enhancer important for enteroendocrine and pancreatic islet cell-specific expression of the secretin gene. 54 61
1378528 1992
24
Serum neuron-specific enolase in diagnosis and follow-up of gastrointestinal neuroendocrine tumors. 54 61
1290031 1992
25
Chromogranin-B, a putative precursor of eight novel rat glucagonoma peptides through processing at mono-, di-, or tribasic residues. 61 54
1954895 1991
26
Morphological changes in the human endocrine pancreas induced by chronic excess of endogenous glucagon. 54 61
1679271 1991
27
Future medical prospects for Sandostatin. 61 54
1980778 1990
28
Future medical prospects for Sandostatin. 61 54
2205787 1990
29
Somatostatin analogue in treatment of coexisting glucagonoma and pancreatic pseudocyst: dissociation of responses. 61 54
2168587 1990
30
Somatostatin analogue and tissue cultures in the study of a human malignant glucagonoma. 61 54
2164620 1990
31
Necrolytic migratory erythema: an important sign of glucagonoma. 61
32527759 2021
32
Glucagonoma syndrome with atypical necrolytic migratory erythema. 61
31368453 2021
33
Pancreatic neuroendocrine neoplasms: Clinicopathological features and pathological staging. 61
33305819 2020
34
Glucagonoma-related necrolytic migratory erythema. 61
31515063 2020
35
Pseudoglucagonoma syndrome: Description of an 'Idiopatic' case. 61
32383170 2020
36
Concomitant Insulinoma and Glucagonoma in Solitary Cystic Pancreatic Lesion in a Young Male Patient. 61
33003101 2020
37
Glucagonoma: From skin lesions to the neuroendocrine component (Review). 61
32905095 2020
38
Benign-Appearing Glucagonoma Undergoing Malignant Transformation after a Prolonged Period. 61
33082756 2020
39
The RNA-binding protein, HuD regulates proglucagon biosynthesis in pancreatic α cells. 61
32828297 2020
40
A retrospective comparison of robotic versus laparoscopic distal resection and enucleation for potentially benign pancreatic neoplasms. 61
32016613 2020
41
Necrolytic Migratory Erythema Associated with a Glucagonoma. 61
32757527 2020
42
The magnesium transporter NIPAL1 is a pancreatic islet-expressed protein that conditionally impacts insulin secretion. 61
32439805 2020
43
Occult insulinoma, glucagonoma and pancreatic endocrine pseudotumour in a patient with multiple endocrine neoplasia type 1. 61
31889623 2020
44
Neuropsychiatric symptoms, skin disease, and weight loss: necrolytic migratory erythema and a glucagonoma. 61
32199485 2020
45
Glucagonoma with Paraneoplasic Dermatitis: Diagnosis and Management. 61
31152349 2020
46
Case of autoimmune progesterone dermatitis presenting as necrotic migratory erythema successfully controlled by danazol. 61
31829458 2020
47
Clinical Characteristics and Management of Functional Pancreatic Neuroendocrine Neoplasms: A Single Institution 20-Year Experience with 286 Patients. 61
33204258 2020
48
MANIFESTATIONS OF GLUCAGONOMA SYNDROME. 61
32524009 2020
49
Molecular imaging of a glucagonoma with 18F-FDG PET/CT and 68Ga-DOTATATE PET/CT imaging: A case report and review of the literature. 61
31737140 2020
50
Diarrhea: a missed D in the 4D glucagonoma syndrome. 61
31807436 2019

Variations for Glucagonoma

Expression for Glucagonoma

Search GEO for disease gene expression data for Glucagonoma.

Pathways for Glucagonoma

Pathways related to Glucagonoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.56 SST SCT GAST
2 9.64 VIP SCT GAST

GO Terms for Glucagonoma

Cellular components related to Glucagonoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.35 SST SCT GAST CHGB CHGA
2 secretory granule GO:0030141 9.16 CHGB CHGA
3 extracellular region GO:0005576 9.1 VIP SST SCT GAST CHGB CHGA

Biological processes related to Glucagonoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 G protein-coupled receptor signaling pathway GO:0007186 8.92 VIP SST SCT GAST

Molecular functions related to Glucagonoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 hormone activity GO:0005179 9.02 VIP SST SCT GAST CHGB

Sources for Glucagonoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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