MCID: ISL001
MIFTS: 60

Islet Cell Tumor malady

Endocrine diseases, Cancer diseases categories

Summaries for Islet Cell Tumor

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MalaCards: Islet Cell Tumor, also known as benign islet cell tumor, is related to pancreatitis and hypoglycemia. An important gene associated with Islet Cell Tumor is CHGA (chromogranin A (parathyroid secretory protein 1)), and among its related pathways are Calcitonin-like ligand receptors and Inhibition of TSC complex formation by PKB. The compounds estrogen and forskolin have been mentioned in the context of this disorder. Affiliated tissues include pancreas, pancreatic islet and liver, and related mouse phenotypes are adipose tissue and tumorigenesis.

Aliases & Classifications for Islet Cell Tumor

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10DISEASES, 8Disease Ontology, 60UMLS, 44Novoseek, 56SNOMED-CT, 39NCIt, 34MeSH
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer diseases
Anatomical: Endocrine diseases


Aliases & Descriptions:

islet cell tumor 8 60
benign islet cell tumor 8 60
pancreatic endocrine carcinoma 10
pancreatic endocrine neoplasm 8
tumor of endocrine pancreas 8
islet cell tumour, benign 8
benign islet cell tumour 8
carcinoma islet cell 44
islet cell neoplasm 8
islet cell tumour 8


Related Diseases for Islet Cell Tumor

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17GeneCards, 18GeneDecks
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Diseases related to Islet Cell Tumor via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 220)
idRelated DiseaseScoreTop Affiliating Genes
1pancreatitis31.7INS, SST
2hypoglycemia31.4INS, IGF2, SST, IAPP, CHGA, GHRH
3diarrhea30.9INS, SST, CHGA, GAST, CALCA, VIP
4pancreatic islet cell tumors30.8PTHLH, RET, PCSK1, SST, IAPP, CHGA
5hyperinsulinism30.7INS, IGF2, SST
6zollinger-ellison syndrome30.6SST, CHGA, GHRH, MEN1, GAST
7hypercalcemia30.5PTHLH, IAPP, CHGA, MEN1, GAST, CALCA
8cushing's syndrome30.5RET, SST, CHGA, SYP, GHRH, MEN1
9acromegaly30.5INS, IGF2, SST, GHRH, GHRHR, MEN1
10pheochromocytoma30.5RET, PCSK1, SST, NTS, IAPP, CHGA
11thyroiditis30.5CALCA
12duodenitis30.5GAST
13peptic ulcer30.5SST, GAST
14von hippel-lindau disease30.5TSC2, RET, MEN1
15hypokalemia30.5INS, VIP
16somatostatinoma30.5SST, CHGA, GHRH, GAST, CALCA, VIP
17insulinoma30.4INS, PCSK1, IGF2, SST, NTS, IAPP
18adenoma30.4TSC1, INS, RET, PCSK1, POU1F1, IGF2
19gastric ulcer30.3GAST
20hypertension30.3INS, CALCA
21duodenal ulcer30.3INS, SST, GAST
22thyroid cancer30.3RET, SST, CHGA, CALCA
23neurofibromatosis30.1TSC2, TSC1, RET, SST, MEN1
24cholelithiasis30.1SST
25sarcoma30.1IGF2
26acute pancreatitis30.1SST, IAPP
27diabetic ketoacidosis30.1INS
28pancreatic cholera30.1SST, VIP
29leydig cell tumor30.1PTHLH
30acinar cell carcinoma30.1CHGA, GAST
31tuberous sclerosis30.1TSC2, TSC1, SYP
32hepatocellular carcinoma30.1INS, IGF2, MEN1, VIP
33diabetes mellitus30.1INS, SST, IAPP, CALCA, GCGR
34cholera30.1INS, NTS, CALCA, VIP
35hyperparathyroidism30.1PTHLH, RET, CHGA, MEN1, GAST, CALCA
36neuroendocrine carcinoma30.1SST, CHGA, SYP, MEN1, GAST, CALCA
37hyperglycemia30.1INS, SST, IAPP, GHRH, GCGR
38gastrinoma30.1SST, IAPP, CHGA, SYP, MEN1, GAST
39vipoma30.1SST, NTS, CHGA, GHRH, MEN1, GAST
40type 2 diabetes mellitus30.1INS, PCSK1, NTS, IAPP, GCGR
41obesity30.1INS, PCSK1, NTS, IAPP, GHRH, GCGR
42neuroendocrine tumor30.1RET, SST, NTS, CHGA, SYP, MEN1
43parathyroid carcinoma30.1RET, MEN1
44adenocarcinoma30.1TSC2, TSC1, PTHLH, POU1F1, SST, NTS
45giant cell tumor10.5
46pheochromocytoma-islet cell tumor syndrome10.5
47syndromic diarrhea10.4
48amyloid tumor10.3
49hepatitis10.3
50portal hypertension10.2

Graphical network of the top 20 diseases related to Islet Cell Tumor:



Diseases related to islet cell tumor

Clinical Features for Islet Cell Tumor

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Drugs & Therapeutics for Islet Cell Tumor

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Islet Cell Tumor

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Anatomical Context for Islet Cell Tumor

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32MalaCards
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MalaCards organs/tissues related to Islet Cell Tumor:

32
Pancreas, Pancreatic islet, Liver, Pituitary, Thyroid, Breast, B cells, Bone, Spinal cord, Ovary, Testes

Animal Models for Islet Cell Tumor or affiliated genes

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36MGI
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Publications for Islet Cell Tumor

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50PubMed
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Articles related to Islet Cell Tumor:

(show top 50)    (show all 358)
idTitleAuthorsYear
1
Management of Non-Islet-Cell Tumor Hypoglycemia: A Clinical Review. (24423303)
2013
2
Non-islet cell tumor hypoglycemia associated with uterine leiomyomata. (21550955)
2011
3
Insulinoma with six islet cell tumors associated with severe hypoglycemia. (19525595)
2009
4
Homozygous P86S mutation of the human glucagon receptor is associated with hyperglucagonemia, alpha cell hyperplasia, and islet cell tumor. (19657311)
2009
5
Non-islet-cell tumor hypoglycemia and lactic acidosis in a child with congenital HIV and Burkitt's lymphoma. (18825882)
2008
6
Clinical features of insulin-like growth factor-II producing non-islet-cell tumor hypoglycemia. (16860583)
2006
7
Zollinger-Ellison phenotype in the absence of hypergastrinemia and islet-cell tumor. (15879632)
2005
8
Surgical treatment of nonfunctioning islet cell tumor: report of 41 cases. (15313691)
2004
9
Non-islet origin of pancreatic islet cell tumors. (15070966)
2004
10
The diagnosis of fasting hypoglycemia due to an islet-cell tumor obscured by a highly specific insulin assay. (12679423)
2003
11
Pancreatic islet cell tumor in a domestic ferret. (14615962)
2003
12
High frequency loss of heterozygosity in von Hippel-Lindau (VHL)-associated and sporadic pancreatic islet cell tumors: evidence for a stepwise mechanism for malignant conversion in VHL tumorigenesis. (11929809)
2002
13
Insulin and somatostatin releasing islet cell tumor caused hypoglycemia. (11334393)
2001
14
Microcytic adenoma coexistent with low-grade malignant islet cell tumor of the pancreas. (11319320)
2001
15
Malignant islet cell tumor projecting into the main pancreatic duct. (11180883)
2000
16
Serum high molecular weight form of insulin-like growth factor II from patients with non-islet cell tumor hypoglycemia is O-glycosylated. (9709962)
1998
17
Multihormone-producing islet cell tumor of the pancreas associated with somatostatin-immunoreactive amyloid: immunohistochemical and immunoelectron microscopic studies. (9500779)
1998
18
Activity of cytosolic and mitochondrial enzymes participating in nutrient catabolism of normal and tumoral islet cells. (7767786)
1995
19
ACTH-secreting islet cell tumor: appearances on dynamic gadolinium-enhanced MRI. (7674861)
1995
20
Giant nonfunctioning islet cell tumor requiring pancreaticoduodenectomy and complete liver revascularization. (8101891)
1993
21
Functional islet cell tumor in six ferrets. (8382672)
1993
22
Durable hepatic tumor regression after arterial chemoembolization-infusion in patients with islet cell carcinoma of the pancreas metastatic to the liver. (8391377)
1993
23
Abnormal facilitative glucose transporter gene expression in human islet cell tumors. (8421107)
1993
24
7B2, a possible marker for nonfunctioning pancreatic islet cell tumor. (1662184)
1991
25
Islet amyloid polypeptide and calcitonin gene-related peptide immunoreactivity in amyloid and tumor cells of canine pancreatic endocrine tumors. (1972303)
1990
26
Watery diarrhea syndrome caused by multihormonal malignant pancreatic islet cell tumor secreting somatostatin, vasoactive intestinal peptide, serotonin, and prostaglandin E--a clinicopathological, biochemical, immunohistochemical, and ultrastructural study. (2883647)
1986
27
Acromegaly due to secretion of growth hormone by an ectopic pancreatic islet-cell tumor. (2981107)
1985
28
Crystals and alpha-1-antitrypsin-reactive globoid inclusions in an islet cell tumor of the pancreas. (3001984)
1985
29
Insulin, glucagon, and somatostatin secretion by cultured rat islet cell tumor and its clones. (6141566)
1984
30
Pancreatic islet cell tumor associated with Cushing's syndrome. (6097131)
1984
31
Immunocytochemical demonstration of insulin in spontaneous pancreatic islet cell tumors of Fischer rats. (6308877)
1983
32
Commercial immunoperoxidase kits in the study of 13 pancreatic islet-cell tumors. (6126116)
1982
33
Insulin, glucagon, and somatostatin receptors on cultured cells and clones from rat islet cell tumor. (6295859)
1982
34
Coexistence of bilateral pheochromocytoma and pancreatic islet cell tumor: report of a case and review of the literature. (215301)
1978
35
Pheochromocytoma with prominent calcification and associated pancreatic islet cell tumor. (199750)
1977
36
Glucagon-producing islet cell tumor of the pancreas. (197840)
1977
37
Hydroxyindole-producing tumors of the pancreas. Carcinoid-islet cell tumor and oat cell carcinoma. (4340726)
1972
38
Islet cell tumor with hypoglycemia. (4323689)
1971
39
Arteriographic demonstration of a giant islet cell tumor of the pancreas. (5099980)
1971
40
Cholera-like syndrome and pancreatic islet cell tumors. (4316335)
1970
41
Hyperinsulinism. Surgical results and management of occult functioning islet cell tumor: review of 154 cases. (4296712)
1968
42
Islet cell tumor in the neonate. Studies in carbohydrate metabolism and therapeutic response. (4296173)
1968
43
PEPTIC ULCER WITH ISLET CELL TUMOR: A REAPPRAISAL. (14215844)
1964
44
DIARRHEA ASSOCIATED WITH PANCREATIC ISLET-CELL TUMORS. (14116905)
1964
45
PANCREATIC ISLET CELL TUMOR IN A DOG. (14238882)
1964
46
Physiological effects of extracts of a diarrhea-producing nonbeta islet cell tumor of the pancreas. (14471107)
1962
47
Gastric hypersecretion, peptic ulceration and islet-cell tumor of the pancreas (the Zollinger-Ellison syndrome); report of a case and review of the literature. (13483869)
1957
48
Benign cystic islet-cell tumor of the head of the pancreas; report of a case probably of delta-cell origin. (14387377)
1955
49
Islet cell tumor of the pancreas with surgical cure. (13065977)
1953
50
Islet-cell tumor of the pancreas with hyperinsulinism; a report of six cases. (21001775)
1946

Genetic Variations for Islet Cell Tumor

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Expression for genes affiliated with Islet Cell Tumor

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Islet Cell Tumor

Search GEO for disease gene expression data for Islet Cell Tumor.

Pathways for genes affiliated with Islet Cell Tumor

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53Reactome, 12EMD Millipore, 51QIAGEN, 29KEGG
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Pathways related to Islet Cell Tumor according to GeneCards/GeneDecks:

(show all 11)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.2CALCA, IAPP
210.1TSC1, TSC2
310.1CHGA, PCSK1, IAPP
4
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10.0CALCA, IAPP, INS
510.0TSC1, TSC2, IGF2
6
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10.0TSC2, TSC1, IGF2
7
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9.8TSC2, TSC1, IGF2, INS
8
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9.7GHRH, TSC1, INS, IGF2, TSC2
9
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9.7GHRHR, VIP, GCGR, IAPP, CALCA, GHRH
10
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9.3GHRH, SST, IAPP, CALCA, NTS, PTHLH
11
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8.2INS, TSC2, TSC1, PTHLH, IGF2, SST

Compounds for genes affiliated with Islet Cell Tumor

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44Novoseek, 49PharmGKB, 11DrugBank, 28IUPHAR, 24HMDB, 59Tocris Bioscience, 2BitterDB
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Compounds related to Islet Cell Tumor according to GeneCards/GeneDecks:

(show top 50)    (show all 151)
idCompoundScoreTop Affiliating Genes
1estrogen4410.7PCSK1
2forskolin44 49 1112.4GHRHR, CALCA, GHRH, POU1F1
3111in-pentetreotide4410.2CALCA, SYP, CHGA, SST
4atropine44 28 1112.2CALCA, GAST, GHRH, NTS
5glutamate4410.1GAST, GHRHR, GHRH, NTS, POU1F1
6i-app4410.1CALCA, GAST, IAPP, PCSK1, INS
7mibg4410.1VIP, CALCA, CHGA, SST
8dopamine44 28 11 2413.1VIP, GAST, GHRH, CHGA, IAPP, NTS
9bromocriptine28 44 1112.0VIP, GHRH, SST, IGF2, POU1F1
10gnrh4410.0VIP, GAST, GHRH, CHGA, NTS, PCSK1
11clonidine44 28 1112.0CALCA, GHRH, CHGA, NTS, SST
12propranolol44 28 49 11 2414.0VIP, CALCA, GAST, GHRH, CHGA, SST
13streptozotocin4410.0SYP, CHGA, IAPP, SST, INS
14calcium gluconate4410.0INS, GAST, CALCA
15dbc-amp449.9VIP, GHRH, SYP, NTS, IGF2, PTHLH
16oxyntomodulin44 5910.9PCSK1, SST, NTS, CHGA, GAST, GCGR
17catecholamine449.9VIP, SYP, CHGA, IAPP, NTS, RET
18nifedipine44 49 28 1112.8VIP, GAST, GHRH, CHGA, NTS, SST
195-hydroxytryptamine449.8VIP, CALCA, GAST, SYP, CHGA, NTS
20hydrocortisone44 2 59 1112.8CALCA, GAST, SST, IGF2, INS, PTHLH
21dexamethasone44 49 28 1112.8INS, POU1F1, IGF2, NTS, IAPP, GHRH
22isoproterenol44 1110.8VIP, CALCA, GAST, CHGA, IAPP, NTS
23ibmx44 28 5911.7VIP, CALCA, IAPP, SST, IGF2, PCSK1
24norepinephrine44 11 2411.7PTHLH, NTS, CHGA, SYP, GHRH, GAST
25c-peptide449.7INS, PCSK1, IGF2, SST, NTS, IAPP
26vitamin d449.6CALCA, GAST, MEN1, GHRH, CHGA, IGF2
27ribonucleic acid449.6GHRHR, GHRH, CHGA, IGF2, POU1F1, PCSK1
285-hydroxyindoleacetic acid44 2410.6VIP, GAST, CHGA, SST
29lactate449.6VIP, CALCA, CHGA, IAPP, SST, IGF2
30acth449.6POU1F1, IGF2, SST, NTS, CHGA, SYP
31acetylcholine44 49 28 11 2413.6VIP, CALCA, GAST, SYP, CHGA, IAPP
32thyroxine44 2410.5VIP, CALCA, GAST, GHRH, NTS, SST
33epinephrine44 11 2411.5VIP, GCGR, CALCA, GAST, CHGA, IAPP
34creatinine449.5GAST, SYP, CHGA, IAPP, IGF2, INS
35thymidine44 2410.5VIP, GAST, MEN1, IAPP, NTS, IGF2
36octreotide44 59 28 1112.5GHRH, GAST, CALCA, VIP, CHGA, NTS
37paraffin449.5SYP, CHGA, NTS, IGF2, RET, PTHLH
38histamine44 28 2411.4VIP, GCGR, CALCA, GAST, SYP, CHGA
39glycogen44 2410.4GCGR, SYP, CHGA, IAPP, IGF2, INS
40serine449.4VIP, MEN1, GHRH, SYP, IAPP, NTS
41glucose449.3TSC2, TSC1, PTHLH, PCSK1, IGF2, NTS
42cyclic amp44 2410.1VIP, GCGR, CALCA, PTHLH, RET, POU1F1
43vegf449.0SYP, MEN1, GAST, CALCA, VIP, CHGA
44alanine449.0VIP, GAST, NTS, IGF2, POU1F1, RET
45adenylate448.9CALCA, GCGR, VIP, PTHLH, PCSK1, IGF2
46arginine448.9TSC2, VIP, GHRHR, GHRH, PTHLH, INS
47lipid448.8GHRH, SYP, CHGA, IAPP, NTS, PCSK1
48testosterone44 59 11 2411.8TSC2, PTHLH, VIP, CALCA, INS, RET
49tyrosine448.7VIP, GAST, MEN1, SYP, CHGA, NTS
50calcium44 49 11 2411.6GHRHR, MEN1, GAST, CALCA, GCGR, VIP

GO Terms for genes affiliated with Islet Cell Tumor

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16Gene Ontology
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Cellular components related to Islet Cell Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1secretory granule lumenGO:03477410.1PCSK1, INS
2TSC1-TSC2 complexGO:0335969.8TSC1, TSC2
3neuronal cell bodyGO:0430259.7CALCA, IAPP, SST, TSC2, VIP
4extracellular spaceGO:0056159.3CALCA, GHRH, IAPP, SST, IGF2, PCSK1
5extracellular regionGO:0055769.0PTHLH, VIP, CALCA, GAST, GHRH, CHGA

Biological processes related to Islet Cell Tumor according to GeneCards/GeneDecks:

(show all 20)
idNameGO IDScoreTop Affiliating Genes
1somatotropin secreting cell developmentGO:06013310.5POU1F1, GHRHR
2growth hormone secretionGO:03025210.4GHRHR, GHRH
3positive regulation of growth hormone secretionGO:06012410.4GHRH, GHRHR
4positive regulation of insulin receptor signaling pathwayGO:04662810.3INS, IGF2
5positive regulation of multicellular organism growthGO:04001810.3GHRH, GHRHR, POU1F1
6positive regulation of vasodilationGO:04590910.3VIP, CALCA, INS
7negative regulation of bone resorptionGO:04577910.3CALCA, IAPP
8regulation of blood pressureGO:00821710.3GCGR, CALCA, CHGA
9negative regulation of cell sizeGO:04579210.2TSC1, TSC2
10positive regulation of cAMP biosynthetic processGO:03081910.2GHRH, CALCA, GHRHR, PTHLH
11determination of adult lifespanGO:00834010.2GHRHR, POU1F1
12MAPK cascadeGO:00016510.2INS, MEN1, RET
13positive regulation of glycogen biosynthetic processGO:04572510.1INS, IGF2
14positive regulation of insulin-like growth factor receptor signaling pathwayGO:04356810.1GHRH, GHRHR
15adenylate cyclase-activating G-protein coupled receptor signaling pathwayGO:00718910.1GHRH, GHRHR, GCGR, PTHLH, CALCA
16insulin receptor signaling pathwayGO:00828610.0TSC1, INS, IGF2, TSC2
17negative regulation of cell proliferationGO:0082859.9SST, MEN1, PTHLH, POU1F1, TSC2, TSC1
18cell-cell signalingGO:0072679.8PCSK1, PTHLH, SST, GHRH, CALCA, INS
19positive regulation of cell proliferationGO:0082849.8GHRHR, GHRH, IGF2, POU1F1, INS, PTHLH
20G-protein coupled receptor signaling pathwayGO:0071869.6VIP, INS, SST, GAST

Molecular functions related to Islet Cell Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1insulin-like growth factor receptor bindingGO:00515910.1IGF2, INS
2hormone activityGO:0051799.0VIP, CALCA, GAST, IAPP, SST, IGF2

Products for genes affiliated with Islet Cell Tumor

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Sources for Islet Cell Tumor

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet