MCID: DDN005
MIFTS: 28

Duodenal Somatostatinoma

Categories: Cancer diseases, Gastrointestinal diseases, Neuronal diseases

Aliases & Classifications for Duodenal Somatostatinoma

MalaCards integrated aliases for Duodenal Somatostatinoma:

Name: Duodenal Somatostatinoma 12 15
Duodenal Somatostatin-Producing Neuroendocrine Tumor 70
Duodenal Delta Cell Somatostatin Producing Tumor 12

Classifications:



External Ids:

Disease Ontology 12 DOID:7479
NCIt 50 C27407
UMLS 70 C1333320

Summaries for Duodenal Somatostatinoma

MalaCards based summary : Duodenal Somatostatinoma, also known as duodenal somatostatin-producing neuroendocrine tumor, is related to cholelithiasis and neurofibromatosis, type i, and has symptoms including diarrhea An important gene associated with Duodenal Somatostatinoma is SST (Somatostatin), and among its related pathways/superpathways are FOXA2 and FOXA3 transcription factor networks and HIF-2-alpha transcription factor network. Affiliated tissues include pituitary, thyroid and pancreas, and related phenotypes are digestive/alimentary and liver/biliary system

Related Diseases for Duodenal Somatostatinoma

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

(show top 50) (show all 128)
# Related Disease Score Top Affiliating Genes
1 cholelithiasis 30.4 SST INS GAST
2 neurofibromatosis, type i 30.2 SST NF1 MEN1
3 tuberous sclerosis 30.0 SST NF1 INS
4 pancreatic somatostatinoma 30.0 SST PPY NF1
5 gastric ulcer 29.9 SST GAST
6 gastrointestinal stromal tumor 29.9 SST NF1 MEN1 EPAS1
7 neuroendocrine tumor 29.8 SST MEN1 GAST
8 carcinoid tumors, intestinal 29.8 SST MEN1 GAST
9 neuroendocrine carcinoma 29.8 SST MEN1 GAST
10 somatostatinoma 29.8 SST PPY NF1 MEN1 INS GAST
11 multiple endocrine neoplasia 29.5 SST NF1 MEN1 GAST
12 paraganglioma 29.0 SST NF1 MEN1 EPAS1 EGLN1
13 neurofibromatosis 10.5
14 acidophil adenoma 10.3 SST INS
15 transient refractive change 10.3 SST INS
16 chronic mountain sickness 10.2 SST EPAS1
17 esophageal varix 10.2 SST INS
18 adrenal medulla cancer 10.2 SST NF1
19 jejunal somatostatinoma 10.2 SST PPY
20 autonomic nervous system benign neoplasm 10.2 SST NF1
21 peripheral nervous system benign neoplasm 10.2 SST NF1
22 hepatic vascular disease 10.2 SST INS
23 rectum neuroendocrine neoplasm 10.2 SST GAST
24 appendix lymphoma 10.2 SST GAST
25 glucagonoma 10.2 SST GAST
26 gastric antral vascular ectasia 10.2 SST GAST
27 small intestine neuroendocrine neoplasm 10.2 SST MEN1
28 colon neuroendocrine neoplasm 10.2 SST GAST
29 carcinoid syndrome 10.2 SST MEN1
30 diamond-blackfan anemia 5 10.2 SST PPY
31 small intestine benign neoplasm 10.1 SST MEN1
32 hormone producing pituitary cancer 10.1 SST MEN1
33 pulmonary neuroendocrine tumor 10.1 SST MEN1
34 duodenogastric reflux 10.1 SST GAST
35 skin lipoma 10.1 NF1 MEN1
36 duodenitis 10.1 INS GAST
37 pituitary adenoma 1, multiple types 10.1 SST MEN1
38 von hippel-lindau syndrome 10.1
39 obstructive jaundice 10.1
40 pituitary carcinoma 10.1 SST MEN1
41 vein disease 10.1 SST INS
42 pituitary tumors 10.1 SST MEN1
43 autonomic nervous system disease 10.1 PPY INS
44 hypertrophic pyloric stenosis 10.1 SST GAST
45 pyloric stenosis 10.1 SST GAST
46 pernicious anemia 10.1 SST GAST
47 endometrial small cell carcinoma 10.1 PPY GAST
48 multiple endocrine neoplasia, type iib 10.1 NF1 MEN1
49 functioning pituitary adenoma 10.1 SST MEN1
50 acth-secreting pituitary adenoma 10.1 SST MEN1

Graphical network of the top 20 diseases related to Duodenal Somatostatinoma:



Diseases related to Duodenal Somatostatinoma

Symptoms & Phenotypes for Duodenal Somatostatinoma

UMLS symptoms related to Duodenal Somatostatinoma:


diarrhea

MGI Mouse Phenotypes related to Duodenal Somatostatinoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 digestive/alimentary MP:0005381 9.55 GAST INS MEN1 NF1 SST
2 liver/biliary system MP:0005370 9.35 EGLN1 EPAS1 INS MEN1 NF1
3 muscle MP:0005369 9.02 EGLN1 EPAS1 INS MEN1 NF1

Drugs & Therapeutics for Duodenal Somatostatinoma

Search Clinical Trials , NIH Clinical Center for Duodenal Somatostatinoma

Genetic Tests for Duodenal Somatostatinoma

Anatomical Context for Duodenal Somatostatinoma

MalaCards organs/tissues related to Duodenal Somatostatinoma:

40
Pituitary, Thyroid, Pancreas, Small Intestine, Retina, Cortex, Colon

Publications for Duodenal Somatostatinoma

Articles related to Duodenal Somatostatinoma:

(show top 50) (show all 51)
# Title Authors PMID Year
1
Somatostatinoma: Beyond neurofibromatosis type 1 (Review). 61
32905002 2020
2
Clinical manifestations of Pacak-Zhuang syndrome in a male pediatric patient. 61
31876082 2020
3
Vascular Changes in the Retina and Choroid of Patients With EPAS1 Gain-of-Function Mutation Syndrome. 61
31876943 2020
4
Duodenal somatostatinoma presenting as obstructive jaundice with the coexistence of a gastrointestinal stromal tumour in neurofibromatosis type 1: a case with review of the literature. 61
30635305 2019
5
Functional Imaging Signature of Patients Presenting with Polycythemia/Paraganglioma Syndromes. 61
28336782 2017
6
Education and imaging. Gastrointestinal: neurofibromatosis type 1, duodenal somatostatinoma and gastrointestinal stromal tumors; a triad worth remembering. 61
24646427 2014
7
Patient with adult-onset type II citrullinemia beginning 2 years after operation for duodenal malignant somatostatinoma: Indication for liver transplantation. 61
23617280 2013
8
Duodenal somatostatinoma: a case report and review of the literature. 61
23618063 2013
9
Endoscopic submucosal dissection of solitary duodenal somatostatinoma (with video). 61
22018555 2012
10
Duodenal somatostatinoma: a case report and review. 61
21437171 2011
11
Somatostatinoma: collision with neurofibroma and ultrastructural features. 61
16874660 2006
12
Duodenal somatostatinoma and gastrointestinal stromal tumor associated with neurofibromatosis type 1: diagnosis with PET/CT. 61
16861519 2006
13
Duodenal somatostatinoma associated with diabetic ketoacidosis presumably caused by somatostatin-28 hypersecretion. 61
16105971 2005
14
Duodenal somatostatinoma: clinical and immunohistochemical patterns--difficult differential diagnosis in regard to gangliocytic paraganglioma: report of a case. 61
15851380 2005
15
Large duodenal somatostatinoma in the third portion associated with severe glucose intolerance. 61
15468970 2004
16
Von Recklinghausen's neurofibromatosis associated with duodenal somatostatinoma. A case report and review of the literature. 61
15258554 2004
17
Duodenal somatostatinoma associated with Von Recklinghausen's disease. 61
15619018 2004
18
Malignant duodenal somatostatinoma presenting in association with von Recklinghausen disease. 61
14700294 2003
19
Duodenal somatostatinoma: a rare cause of gastrointestinal bleeding. 61
12897887 2003
20
Duodenal somatostatinoma presenting with complete somatostatinoma syndrome. 61
11606861 2001
21
Duodenal somatostatinoma of the ampulla of vater diagnosed by endoscopic fine needle aspiration biopsy: a case report. 61
11480730 2001
22
[Duodenal somatostatinoma. Anatomic/clinical study of 12 operated cases]. 61
11340706 2001
23
Duodenal somatostatinoma and erythrocytosis in a patient with von Hippel-Lindau disease type 2A. 61
11201368 2001
24
Longevity possible in metastatic duodenal somatostatinoma. 61
11107073 2000
25
Duodenal somatostatinoma: a case report and review of 31 cases with special reference to the relationship between tumor size and metastasis. 61
10792774 2000
26
Heterotopic pancreas, periampullary somatostatinoma and type I neurofibromatosis: a pathogenetic proposal. 61
10399162 1999
27
Duodenal somatostatinoma presenting as upper gastrointestinal bleeding. 61
10235228 1999
28
Carcinoid somatostatinoma of the duodenum. 61
9870739 1998
29
[Duodenal somatostatinomas associated with von Recklinghausen disease]. 61
9888169 1998
30
Thirty-Year Survival with an Untreated Malignant Duodenal Somatostatinoma. 61
12114786 1998
31
Small duodenal somatostatinoma with high plasma somatostatin level. 61
9177540 1997
32
[Pancreatic and duodenal somatostatinoma. Two clinico-pathologic entities]. 61
9172623 1996
33
[Duodenal somatostatinoma associated with von Recklinghausen's neurofibromatosis]. 61
8975174 1996
34
Von Recklinghausen's disease associated with duodenal somatostatinoma: contrast of duodenal versus pancreatic somatostatinomas. 61
7745981 1995
35
Duodenal malignant somatostatinoma. 61
18612476 1995
36
Exocrine pancreatic insufficiency and pancreatic fibrosis due to duodenal somatostatinoma in a patient with neurofibromatosis. 61
1348357 1992
37
von Recklinghausen's disease associated with somatostatin-rich duodenal carcinoid (somatostatinoma), medullary thyroid carcinoma and diffuse adrenal medullary hyperplasia. 61
1686137 1991
38
Duodenal somatostatinoma with congenital pseudoarthrosis. 61
2033227 1991
39
Duodenal somatostatinoma with psammoma bodies. 61
2565711 1989
40
Duodenal carcinoid (somatostatinoma) combined with von Recklinghausen's disease. A case report and review of the literature. 61
2541598 1989
41
A polypoid duodenal somatostatinoma. A case report. 61
2830659 1988
42
Synchronous duodenal neuroendocrine tumours in von Recklinghausen's disease--a case report of co-existing gangliocytic paraganglioma and somatostatin-rich glandular carcinoid. 61
2894342 1987
43
Duodenal somatostatinoma with psammoma bodies: an immunohistochemical and ultrastructural study. 61
2867674 1986
44
Somatostatin-containing D cells exhibit immunoreactivity for rat somatostatin cryptic peptide in six mammalian species. An electron-microscopical study. 61
2877739 1986
45
[A case of duodenal somatostatinoma and a review of the literature]. 61
2867238 1985
46
Immunohistochemical detection of chromogranin and neuron-specific enolase in pancreatic endocrine neoplasms. 61
6205601 1984
47
Duodenal somatostatinoma. 61
6318875 1984
48
A case of duodenal somatostatinoma: diagnostic usefulness of calcium--pentagastrin test. 61
6137434 1983
49
Ultrastructural characteristics of duodenal somatostatinomas. I. Somatostatinoma cells and Schwann-like cells. 61
6306985 1983
50
Duodenal somatostatinoma. Immunohistopathology and review of literature. 61
6301208 1983

Variations for Duodenal Somatostatinoma

Expression for Duodenal Somatostatinoma

Search GEO for disease gene expression data for Duodenal Somatostatinoma.

Pathways for Duodenal Somatostatinoma

Pathways related to Duodenal Somatostatinoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 10.6 NF1 INS
2 10.15 EPAS1 EGLN1

GO Terms for Duodenal Somatostatinoma

Biological processes related to Duodenal Somatostatinoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cognition GO:0050890 9.16 NF1 INS
2 regulation of angiogenesis GO:0045765 8.96 NF1 EGLN1
3 response to hypoxia GO:0001666 8.8 NF1 EPAS1 EGLN1

Molecular functions related to Duodenal Somatostatinoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 hormone activity GO:0005179 8.92 SST PPY INS GAST

Sources for Duodenal Somatostatinoma

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 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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