MCID: VPM001
MIFTS: 48

Vipoma

Categories: Cancer diseases, Endocrine diseases, Gastrointestinal diseases, Rare diseases

Aliases & Classifications for Vipoma

MalaCards integrated aliases for Vipoma:

Name: Vipoma 12 76 53 59 55 44 15 73
Diarrheogenic Islet Cell Tumor 53 59
Vip-Secreting Tumor 53 59
Pancreatic Cholera 59 73
Pancreatic Vipoma 53 73
Malignant Vasoactive Intestinal Peptide-Secreting Tumor 12
Watery Diarrhea-Hypokalemia-Achlorhydria Syndrome 59
Vasoactive Intestinal Peptide-Secreting Tumor 12
Vasoactive Intestinal Peptide-Producing Tumor 53
Vasoactive Intestinal Peptide Tumor 53
Verner-Morrison Syndrome 59
Vip- Secreting Tumor 12
Vipoma, Malignant 12
Malignant Vipoma 73
Wdha Syndrome 59

Characteristics:

Orphanet epidemiological data:

59
vipoma
Inheritance: Not applicable; Prevalence: <1/1000000 (Europe); Age of onset: All ages; Age of death: any age;

Classifications:



Summaries for Vipoma

NIH Rare Diseases : 53 A VIPoma is a rare cancer caused by a type of pancreatic neuroendocrine tumor, which is tumor that arises from cells that produce hormones - allowing the tumor itself to produce hormones. A VIPoma secretes vasoactive intestinal peptide (VIP), a hormone that stimulates the secretion (and inhibits the absorption) of sodium, chloride, potassium and water within the small intestine. Excessive secretion can increase bowel motility and lead to signs and symptoms such as abdominal pain and cramping, severe watery diarrhea, dehydration, flushing of the face, muscle cramps due to low potassium levels (hypokalemia), and weight loss. When a person has a VIPoma and these associated symptoms, it is referred to as VIPoma syndrome, also called WDHA syndrome or Verner-Morrison syndrome. The majority of people with a VIPoma have VIPoma syndrome. VIPomas are usually diagnosed in middle-aged adults or in young children. Tests used to diagnose a VIPoma may include blood tests (including VIP level), imaging studies such as a CT scan or MRI, and examination of a stool sample. Unfortunately, even though these tumors are slow-growing, the majority of VIPomas are metastatic (have spread to other parts of the body) at the time of diagnosis. The underlying cause of VIPomas is not yet known. Management typically first involves treating dehydration with fluids and correcting the imbalance of hormones and other substances (metabolic abnormalities) in the body. Medications such as octreotide and lanreotide can usually stop the diarrhea and inhibit the secretion of VIP. Surgery typically follows in order to remove the tumor. When there is no evidence of metastasis, complete removal of the tumor is the only possible cure. The majority of metastatic cases will not be cured by surgery, but symptoms may be managed and prolonged survival is often possible due to the slow-growing nature of these tumors. Targeted therapy or chemotherapy may also be used for people with progressive, advanced disease. The median survival of people with VIPomas is 96 months, but long-term survival mainly depends on the tumor grade, staging, and whether all or most of the tumor can be removed.

MalaCards based summary : Vipoma, also known as diarrheogenic islet cell tumor, is related to wdha syndrome and pancreatic cholera, and has symptoms including diarrhea An important gene associated with Vipoma is VIP (Vasoactive Intestinal Peptide), and among its related pathways/superpathways is Gastric acid production. The drugs Capecitabine and Dacarbazine have been mentioned in the context of this disorder. Affiliated tissues include pancreas, testes and small intestine, and related phenotypes are diabetes mellitus and nausea and vomiting

Wikipedia : 76 A VIPoma or vipoma (/vɪ�?po�?m�?/) is a rare endocrine tumor that overproduces vasoactive intestinal... more...

Related Diseases for Vipoma

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

(show top 50) (show all 96)
# Related Disease Score Top Affiliating Genes
1 wdha syndrome 33.3 SST VIP
2 pancreatic cholera 31.7 GAST SCT SST VIP
3 malignant pheochromocytoma 30.7 CHGA SST
4 diarrhea 30.1 GAST SST
5 pheochromocytoma 30.1 CHGA SST VIP
6 adenoma of the pancreas 30.0 CHGA SST
7 paraganglioma 29.8 CHGA SST
8 insulinoma 29.8 CHGA SCT SST
9 glucagonoma 29.7 CHGA SCT SST VIP
10 carcinoid syndrome 29.5 CHGA NTS SST
11 multiple endocrine neoplasia, type i 29.4 CHGA GAST SCT SST VIP
12 small intestinal vasoactive intestinal peptide producing tumor 12.7
13 pancreatic vasoactive intestinal peptide producing tumor 11.4
14 pancreatic neuroendocrine tumor 11.1
15 cholera 10.6
16 smith-magenis syndrome 10.4
17 large cell carcinoma 10.4
18 cryptosporidiosis 10.1 SST VIP
19 pylorospasm 10.1 GAST SST
20 postcholecystectomy syndrome 10.1 GAST SST
21 angiodysplasia 10.1 SCT SST
22 multiple endocrine neoplasia 10.1
23 retinitis pigmentosa 40 10.1 GAST SST
24 pernicious anemia 10.1 GAST SST
25 hyperprolactinemia 10.1 SST VIP
26 duodenal disease 10.1 SCT SST
27 autonomic nervous system benign neoplasm 10.1 CHGA VIP
28 steatorrhea 10.1 SCT SST
29 ileum cancer 10.1 GAST SST
30 diphyllobothriasis 10.0 GAST SCT
31 gastrointestinal neuroendocrine tumor 10.0 CHGA SST
32 small intestine cancer 10.0 SCT SST
33 pituitary carcinoma 10.0 CHGA SST
34 diffuse pulmonary fibrosis 10.0 CHGA VIP
35 carcinoid tumors, intestinal 10.0 CHGA SST
36 gastritis 10.0 CHGA GAST
37 pertussis 10.0 SST VIP
38 ganglioneuroma 10.0 CHGA VIP
39 autoimmune atrophic gastritis 10.0 CHGA GAST
40 pancreatic cystadenoma 10.0 CHGA SST
41 goblet cell carcinoid 10.0 CHGA GAST
42 peptic ulcer disease 10.0 GAST SCT
43 endocrine organ benign neoplasm 10.0 CHGA SST
44 non-functioning pancreatic endocrine tumor 10.0 CHGA GAST
45 stomach disease 10.0 GAST SST
46 irritable bowel syndrome 10.0 CHGA VIP
47 cell type benign neoplasm 10.0 CHGA SST
48 merkel cell carcinoma 10.0 CHGA SST
49 neurofibromatosis, type i 10.0
50 acute pancreatitis 10.0 SCT SST

Graphical network of the top 20 diseases related to Vipoma:



Diseases related to Vipoma

Symptoms & Phenotypes for Vipoma

Human phenotypes related to Vipoma:

59 32 (show all 42)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 diabetes mellitus 59 32 frequent (33%) Frequent (79-30%) HP:0000819
2 nausea and vomiting 59 32 frequent (33%) Frequent (79-30%) HP:0002017
3 hepatomegaly 59 32 frequent (33%) Frequent (79-30%) HP:0002240
4 malabsorption 59 32 frequent (33%) Frequent (79-30%) HP:0002024
5 dehydration 59 32 frequent (33%) Frequent (79-30%) HP:0001944
6 hypokalemia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002900
7 respiratory insufficiency due to muscle weakness 59 32 very rare (1%) Very rare (<4-1%) HP:0002747
8 generalized muscle weakness 59 32 frequent (33%) Frequent (79-30%) HP:0003324
9 ascites 59 32 occasional (7.5%) Occasional (29-5%) HP:0001541
10 weight loss 59 32 frequent (33%) Frequent (79-30%) HP:0001824
11 increased circulating gonadotropin level 59 32 very rare (1%) Very rare (<4-1%) HP:0000837
12 anorexia 59 32 frequent (33%) Frequent (79-30%) HP:0002039
13 chronic fatigue 59 32 frequent (33%) Frequent (79-30%) HP:0012432
14 muscle cramps 59 32 frequent (33%) Frequent (79-30%) HP:0003394
15 extrahepatic cholestasis 59 32 occasional (7.5%) Occasional (29-5%) HP:0012334
16 parathyroid adenoma 59 32 very rare (1%) Very rare (<4-1%) HP:0002897
17 hypercalcemia 59 32 frequent (33%) Frequent (79-30%) HP:0003072
18 growth hormone excess 59 32 very rare (1%) Very rare (<4-1%) HP:0000845
19 subcutaneous lipoma 59 32 very rare (1%) Very rare (<4-1%) HP:0001031
20 episodic abdominal pain 59 32 frequent (33%) Frequent (79-30%) HP:0002574
21 pituitary adenoma 59 32 very rare (1%) Very rare (<4-1%) HP:0002893
22 adrenocortical adenoma 59 32 very rare (1%) Very rare (<4-1%) HP:0008256
23 erythema 59 32 frequent (33%) Frequent (79-30%) HP:0010783
24 neoplasm of the pancreas 59 32 hallmark (90%) Very frequent (99-80%) HP:0002894
25 follicular thyroid carcinoma 59 32 very rare (1%) Very rare (<4-1%) HP:0006731
26 neoplasm of the liver 59 32 very rare (1%) Very rare (<4-1%) HP:0002896
27 intrahepatic cholestasis 59 32 occasional (7.5%) Occasional (29-5%) HP:0001406
28 prolactin excess 59 32 very rare (1%) Very rare (<4-1%) HP:0000870
29 ganglioneuroma 59 32 very rare (1%) Very rare (<4-1%) HP:0003005
30 poor appetite 59 32 frequent (33%) Frequent (79-30%) HP:0004396
31 elevated calcitonin 59 32 very rare (1%) Very rare (<4-1%) HP:0003528
32 primary hyperparathyroidism 59 32 very rare (1%) Very rare (<4-1%) HP:0008200
33 normochromic anemia 59 32 frequent (33%) Frequent (79-30%) HP:0001895
34 intermittent jaundice 59 32 occasional (7.5%) Occasional (29-5%) HP:0001046
35 secretory diarrhea 59 32 hallmark (90%) Very frequent (99-80%) HP:0005208
36 abnormal gastrointestinal motility 59 32 occasional (7.5%) Occasional (29-5%) HP:0030895
37 benign gastrointestinal tract tumors 59 32 very rare (1%) Very rare (<4-1%) HP:0006719
38 hypercortisolism 59 Very rare (<4-1%)
39 hematochezia 59 Excluded (0%)
40 abnormality of the thyroid gland 59 Very rare (<4-1%)
41 increased circulating cortisol level 32 very rare (1%) HP:0003118
42 abnormality of the abdomen 59 Occasional (29-5%)

UMLS symptoms related to Vipoma:


diarrhea

Drugs & Therapeutics for Vipoma

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

(show top 50) (show all 63)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Capecitabine Approved, Investigational Phase 2,Phase 1 154361-50-9 60953
2
Dacarbazine Approved, Investigational Phase 2,Phase 1 4342-03-4 5351166
3
Temozolomide Approved, Investigational Phase 2,Phase 1 85622-93-1 5394
4
Nicotinamide Approved, Investigational Phase 2 98-92-0 936
5
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
6
Gefitinib Approved, Investigational Phase 2 184475-35-2 123631
7
Octreotide Approved, Investigational Phase 2 83150-76-9 383414 6400441
8
Sirolimus Approved, Investigational Phase 2 53123-88-9 46835353 6436030 5284616
9
Everolimus Approved Phase 2 159351-69-6 6442177
10
Bevacizumab Approved, Investigational Phase 2 216974-75-3
11
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
12
Pasireotide Approved Phase 2 396091-73-9 9941444
13
Somatostatin Approved, Investigational Phase 2 51110-01-1, 38916-34-6 53481605
14
Niacin Approved, Investigational, Nutraceutical Phase 2 59-67-6 938
15
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
16 Alkylating Agents Phase 2,Phase 1
17 Antimetabolites Phase 2,Phase 1
18 Antimetabolites, Antineoplastic Phase 2,Phase 1
19 Antineoplastic Agents, Alkylating Phase 2,Phase 1
20 Epothilones Phase 2
21 Antimitotic Agents Phase 2,Phase 1
22 Epothilone B Phase 2
23 Vitamin B Complex Phase 2
24 Nicotinic Acids Phase 2
25 Vitamin B9 Phase 2
26 Vitamins Phase 2
27 Trace Elements Phase 2
28 Protein Kinase Inhibitors Phase 2
29 Vitamin B3 Phase 2
30 Folate Phase 2
31 Micronutrients Phase 2
32 Immunologic Factors Phase 2,Phase 1
33 Angiogenesis Modulating Agents Phase 2,Phase 1
34 Angiogenesis Inhibitors Phase 2,Phase 1
35 Antineoplastic Agents, Hormonal Phase 2
36 Gastrointestinal Agents Phase 2
37 Antibodies Phase 2
38 Immunoglobulins Phase 2
39 Endothelial Growth Factors Phase 2
40 Antifungal Agents Phase 2
41 Anti-Bacterial Agents Phase 2
42 Immunoglobulin G Phase 2
43 Antibodies, Monoclonal Phase 2
44 Immunosuppressive Agents Phase 2
45 Antibiotics, Antitubercular Phase 2
46 Mitogens Phase 2
47 Anti-Infective Agents Phase 2
48 Hormone Antagonists Phase 2
49 Hormones Phase 2
50 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2

Interventional clinical trials:

(show all 11)
# Name Status NCT ID Phase Drugs
1 Capecitabine and Temozolomide for Neuroendocrine Cancers Completed NCT00869050 Phase 2 Capecitabine;Temozolomide
2 EPO906 in Carcinoid and Other Neuroendocrine Tumors Completed NCT00050349 Phase 2 EPO906 epothilone B
3 Sorafenib Tosylate in Treating Patients With Progressive Metastatic Neuroendocrine Tumors Completed NCT00131911 Phase 2 sorafenib tosylate
4 Gefitinib in Treating Patients With Progressive Metastatic Neuroendocrine Tumors Completed NCT00075439 Phase 2 gefitinib
5 Everolimus and Octreotide Acetate With or Without Bevacizumab in Treating Patients With Locally Advanced or Metastatic Pancreatic Neuroendocrine Tumors That Cannot Be Removed by Surgery Active, not recruiting NCT01229943 Phase 2 Everolimus;Octreotide Acetate
6 Study of Pasireotide in Patients With Rare Tumors of Neuroendocrine Origin Terminated NCT00958841 Phase 2 pasireotide LAR
7 Interleukin-12 and Trastuzumab in Treating Patients With Cancer That Has High Levels of HER2/Neu Completed NCT00004074 Phase 1
8 Laser Tissue Welding - Distal Pancreatectomy Sealing Study Recruiting NCT03147768 Phase 1
9 Veliparib, Capecitabine, and Temozolomide in Patients With Advanced, Metastatic, and Recurrent Neuroendocrine Tumor Withdrawn NCT02831179 Phase 1 Capecitabine;Temozolomide;Veliparib
10 DOTATOC PET/CT for Imaging NET Patients Recruiting NCT03583528
11 Gastroenteral-Pancreatic Neuroendocrine Tumors in Taiwan Enrolling by invitation NCT02102893

Search NIH Clinical Center for Vipoma

Cochrane evidence based reviews: vipoma

Genetic Tests for Vipoma

Anatomical Context for Vipoma

MalaCards organs/tissues related to Vipoma:

41
Pancreas, Testes, Small Intestine, Liver, Thyroid, Pituitary, Lung

Publications for Vipoma

Articles related to Vipoma:

(show top 50) (show all 104)
# Title Authors Year
1
Paediatric VIPoma: A Jamboree of Electrolytes. ( 29744252 )
2018
2
Perioperative Hypotensive Crisis in an Adolescent with a Pancreatic VIPoma and MEN1-Gene Variant. ( 30326482 )
2018
3
Percutaneous Irreversible Electroporation for Pancreatic VIPoma: A Case Report. ( 27977628 )
2017
4
Ventricular fibrillation resulting from electrolyte imbalance reveals vipoma in MEN1 syndrome. ( 27071757 )
2016
5
Emergency therapy for liver metastases from advanced VIPoma: surgery or transarterial chemoembolization? ( 27583030 )
2016
6
When Symptomatic Treatment Becomes Antitumor Treatment for Vipoma: Opportunity for Frail Elderly Adults. ( 26889855 )
2016
7
Metastatic VIPoma presenting as an ovarian mass. ( 26657531 )
2015
8
VIPoma with multiple endocrine neoplasia type 1 identified as an atypical gene mutation. ( 26564120 )
2015
9
Pancreatic VIPoma visualized by 68Ga DOTA-NOC PET-CT. ( 26235641 )
2015
10
Sunitinib Achieved Fast and Sustained Control of VIPoma Symptoms. ( 25305306 )
2014
11
Sporadic pancreatic vasoactive intestinal peptide-producing tumor (VIPoma) in a 47-year-old male. ( 24785507 )
2014
12
Contrast enhanced multi-detector CT and MR findings of a well-differentiated pancreatic vipoma. ( 25349667 )
2014
13
Extra-pancreatic vipoma. ( 25184777 )
2014
14
Sunitinib for the treatment of metastatic paraganglioma and vasoactive intestinal polypeptide-producing tumor (VIPoma). ( 23407483 )
2013
15
VIPoma in a 37-year-old man. ( 23993192 )
2013
16
A rare cause of diarrhea: pancreatic VIPoma. ( 24008482 )
2013
17
Ischemic stroke as a presenting feature of VIPoma due to MEN 1 syndrome. ( 24251163 )
2013
18
VIPoma that arose from the rectum in a 65-year-old male patient. ( 22350188 )
2012
19
MSH2 and CXCR4 involvement in malignant VIPoma. ( 23231927 )
2012
20
Diagnosis and treatment of VIPoma: a case report and literature review in China. ( 22695090 )
2012
21
VIPoma Crisis: Immediate and life saving reduction of massive stool volumes on starting treatment with octreotide. ( 21509215 )
2011
22
Benign pancreatic vipoma. ( 21526882 )
2011
23
VIPoma syndrome: challenges in management. ( 20730389 )
2010
24
Is radiofrequency ablation justified for liver metastatic VIPoma patient undergoing Whipple procedure? ( 20819560 )
2010
25
Amelioration of symptoms and reduction of VIP levels after hepatic artery chemoembolization in a patient with sandostatin resistant VIPoma. ( 20351979 )
2010
26
Hypokalemic rhabdomyolysis due to watery diarrhea, hypokalemia, achlorhydria (WDHA) syndrome caused by vipoma. ( 19488018 )
2009
27
Nesidioblastosis as a cause of focal pancreatic 111In-pentetreotide uptake in a patient with putative VIPoma: another differential diagnosis. ( 19387771 )
2009
28
VIPoma with expression of both VIP and VPAC1 receptors in a patient with WDHA syndrome. ( 19184565 )
2009
29
Multi-visceral resection of pancreatic VIPoma in a patient with sinistral portal hypertension. ( 18662399 )
2008
30
[Diagnostic procedures and more particularly, place of scintigraphy in neuroendocrine tumors, example of vipoma in MEN 1]. ( 17292846 )
2007
31
Diagnosis and treatment of VIPoma in a female patient. ( 16357627 )
2006
32
Radiofrequency ablation has a valuable therapeutic role in metastatic VIPoma. ( 16354964 )
2006
33
Calcitonin-secreting VIPoma. ( 16416161 )
2005
34
VIPoma: a rare cause of a pancreatic mass. ( 15162993 )
2004
35
[Jejunal vipoma]. ( 15646540 )
2004
36
VIPoma. ( 15590967 )
2004
37
[The VIP-secreting tumor as a differential diagnosis of protracted diarrhea in pediatrics]. ( 15455292 )
2004
38
Diagnosis and treatment of VIPoma in China: (case report and 31 cases review) diagnosis and treatment of VIPoma. ( 14707737 )
2004
39
Images in clinical medicine. VIPoma. ( 15317894 )
2004
40
Vasoactive intestinal polypeptide-secreting tumor (VIPoma) with liver metastases: dramatic and durable symptomatic benefit from hepatic artery embolization, a case report. ( 12482130 )
2002
41
Vipoma: effective treatment with octreotide in the oldest old. ( 11347804 )
2001
42
Dynamic gadolinium-enhanced MR imaging of pancreatic VIPoma in a patient with Verner-Morrison syndrome. ( 11702127 )
2001
43
Vasoactive Intestinal Peptide Tumor (VIPoma) ( 25905195 )
2000
44
Metastatic jejunal VIPoma: beneficial effect of combination therapy with interferon-alpha and 5-fluorouracil. ( 10638600 )
2000
45
VIPoma of pancreas in a child. ( 11059195 )
2000
46
Long-term survival after diagnosis of hepatic metastatic VIPoma: report of two cases with disparate courses and review of therapeutic options. ( 10389687 )
1999
47
Metastatic pancreatic VIPoma: deteriorating clinical course and successful treatment by liver transplantation. ( 9577908 )
1998
48
Location of a VIPoma by iodine-123-vasoactive intestinal peptide scintigraphy. ( 9744346 )
1998
49
The cost-effectiveness of octreotide acetate in the treatment of carcinoid syndrome and VIPoma. ( 9780538 )
1998
50
Vipoma/diarrheogenic syndrome: a statistical evaluation of 241 reported cases. ( 10089056 )
1998

Variations for Vipoma

Expression for Vipoma

Search GEO for disease gene expression data for Vipoma.

Pathways for Vipoma

Pathways related to Vipoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 9.64 GAST SCT VIP

GO Terms for Vipoma

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

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.46 CHGA GAST SCT SST
2 extracellular region GO:0005576 9.1 CHGA GAST NTS SCT SST VIP
3 transport vesicle GO:0030133 8.96 CHGA NTS

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

# Name GO ID Score Top Affiliating Genes
1 G protein-coupled receptor signaling pathway GO:0007186 9.35 GAST NTS SCT SST VIP
2 regulation of signaling receptor activity GO:0010469 9.02 GAST NTS SCT SST VIP

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

# Name GO ID Score Top Affiliating Genes
1 neuropeptide hormone activity GO:0005184 8.96 NTS VIP
2 hormone activity GO:0005179 8.92 GAST SCT SST VIP

Sources for Vipoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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