MCID: VPM001
MIFTS: 51

Vipoma

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

Aliases & Classifications for Vipoma

MalaCards integrated aliases for Vipoma:

Name: Vipoma 12 77 54 60 56 45 15 74
Pancreatic Vipoma 54 17 74
Diarrheogenic Islet Cell Tumor 54 60
Vip-Secreting Tumor 54 60
Pancreatic Cholera 60 74
Malignant Vasoactive Intestinal Peptide-Secreting Tumor 12
Watery Diarrhea-Hypokalemia-Achlorhydria Syndrome 60
Vasoactive Intestinal Peptide-Secreting Tumor 12
Vasoactive Intestinal Peptide-Producing Tumor 54
Vasoactive Intestinal Peptide Tumor 54
Verner-Morrison Syndrome 60
Vip- Secreting Tumor 12
Vipoma, Malignant 12
Malignant Vipoma 74
Wdha Syndrome 60

Characteristics:

Orphanet epidemiological data:

60
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 : 54 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 pancreatic vipoma, 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 are Signaling by GPCR and Gastric acid production. The drugs Capecitabine and Temozolomide have been mentioned in the context of this disorder. Affiliated tissues include pancreas, small intestine and testes, and related phenotypes are hypokalemia and neoplasm of the pancreas

Wikipedia : 77 A VIPoma or vipoma (/vɪˈpoʊmə/) 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 33.5 SST VIP
2 pancreatic cholera 31.7 GAST SCT SST VIP
3 malignant pheochromocytoma 30.8 CHGA SST
4 pheochromocytoma 30.2 CHGA SST VIP
5 paraganglioma 29.9 CHGA SST
6 insulinoma 29.8 CHGA SCT SST
7 glucagonoma 29.7 CHGA SCT SST VIP
8 carcinoid syndrome 29.7 CHGA NTS SST
9 multiple endocrine neoplasia, type i 29.3 CHGA GAST SCT SST VIP
10 small intestinal vasoactive intestinal peptide producing tumor 12.7
11 pancreatic vasoactive intestinal peptide producing tumor 11.5
12 pancreatic neuroendocrine tumor 11.1
13 cholera 10.6
14 large cell carcinoma 10.4
15 smith-magenis syndrome 10.4
16 diarrhea 10.4
17 cryptosporidiosis 10.2 SST VIP
18 pylorospasm 10.2 GAST SST
19 postcholecystectomy syndrome 10.2 GAST SST
20 retinitis pigmentosa 40 10.2 GAST SST
21 pernicious anemia 10.2 GAST SST
22 autonomic nervous system benign neoplasm 10.2 CHGA VIP
23 gastrointestinal neuroendocrine tumor 10.2 CHGA SST
24 pituitary carcinoma 10.2 CHGA SST
25 hyperprolactinemia 10.1 SST VIP
26 angiodysplasia 10.1 SCT SST
27 ileum cancer 10.1 GAST SST
28 gastritis 10.1 CHGA GAST
29 carcinoid tumors, intestinal 10.1 CHGA SST
30 diffuse pulmonary fibrosis 10.1 CHGA VIP
31 autoimmune atrophic gastritis 10.1 CHGA GAST
32 goblet cell carcinoid 10.1 CHGA GAST
33 pancreatic cystadenoma 10.1 CHGA SST
34 ganglioneuroma 10.1 CHGA VIP
35 multiple endocrine neoplasia 10.1
36 steatorrhea 10.1 SCT SST
37 diphyllobothriasis 10.1 GAST SCT
38 non-functioning pancreatic endocrine tumor 10.1 CHGA GAST
39 endocrine organ benign neoplasm 10.1 CHGA SST
40 small intestine cancer 10.1 SCT SST
41 peptic ulcer disease 10.1 GAST SCT
42 irritable bowel syndrome 10.0 CHGA VIP
43 cell type benign neoplasm 10.0 CHGA SST
44 autonomic nervous system neoplasm 10.0 NTS VIP
45 pertussis 10.0 SST VIP
46 peripheral nervous system neoplasm 10.0 NTS VIP
47 short bowel syndrome 10.0 NTS SST
48 stomach disease 10.0 GAST SST
49 merkel cell carcinoma 10.0 CHGA SST
50 neurofibromatosis, type i 10.0

Graphical network of the top 20 diseases related to Vipoma:



Diseases related to Vipoma

Symptoms & Phenotypes for Vipoma

Human phenotypes related to Vipoma:

60 33 (show all 43)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hypokalemia 60 33 hallmark (90%) Very frequent (99-80%) HP:0002900
2 neoplasm of the pancreas 60 33 hallmark (90%) Very frequent (99-80%) HP:0002894
3 secretory diarrhea 60 33 hallmark (90%) Very frequent (99-80%) HP:0005208
4 diabetes mellitus 60 33 frequent (33%) Frequent (79-30%) HP:0000819
5 nausea and vomiting 60 33 frequent (33%) Frequent (79-30%) HP:0002017
6 hepatomegaly 60 33 frequent (33%) Frequent (79-30%) HP:0002240
7 malabsorption 60 33 frequent (33%) Frequent (79-30%) HP:0002024
8 dehydration 60 33 frequent (33%) Frequent (79-30%) HP:0001944
9 generalized muscle weakness 60 33 frequent (33%) Frequent (79-30%) HP:0003324
10 weight loss 60 33 frequent (33%) Frequent (79-30%) HP:0001824
11 anorexia 60 33 frequent (33%) Frequent (79-30%) HP:0002039
12 chronic fatigue 60 33 frequent (33%) Frequent (79-30%) HP:0012432
13 hypercalcemia 60 33 frequent (33%) Frequent (79-30%) HP:0003072
14 episodic abdominal pain 60 33 frequent (33%) Frequent (79-30%) HP:0002574
15 erythema 60 33 frequent (33%) Frequent (79-30%) HP:0010783
16 poor appetite 60 33 frequent (33%) Frequent (79-30%) HP:0004396
17 normochromic anemia 60 33 frequent (33%) Frequent (79-30%) HP:0001895
18 muscle spasm 33 frequent (33%) HP:0003394
19 ascites 60 33 occasional (7.5%) Occasional (29-5%) HP:0001541
20 extrahepatic cholestasis 60 33 occasional (7.5%) Occasional (29-5%) HP:0012334
21 intrahepatic cholestasis 60 33 occasional (7.5%) Occasional (29-5%) HP:0001406
22 intermittent jaundice 60 33 occasional (7.5%) Occasional (29-5%) HP:0001046
23 abnormal gastrointestinal motility 60 33 occasional (7.5%) Occasional (29-5%) HP:0030895
24 respiratory insufficiency due to muscle weakness 60 33 very rare (1%) Very rare (<4-1%) HP:0002747
25 increased circulating gonadotropin level 60 33 very rare (1%) Very rare (<4-1%) HP:0000837
26 parathyroid adenoma 60 33 very rare (1%) Very rare (<4-1%) HP:0002897
27 growth hormone excess 60 33 very rare (1%) Very rare (<4-1%) HP:0000845
28 subcutaneous lipoma 60 33 very rare (1%) Very rare (<4-1%) HP:0001031
29 pituitary adenoma 60 33 very rare (1%) Very rare (<4-1%) HP:0002893
30 adrenocortical adenoma 60 33 very rare (1%) Very rare (<4-1%) HP:0008256
31 follicular thyroid carcinoma 60 33 very rare (1%) Very rare (<4-1%) HP:0006731
32 ganglioneuroma 60 33 very rare (1%) Very rare (<4-1%) HP:0003005
33 neoplasm of the liver 60 33 very rare (1%) Very rare (<4-1%) HP:0002896
34 prolactin excess 60 33 very rare (1%) Very rare (<4-1%) HP:0000870
35 elevated calcitonin 60 33 very rare (1%) Very rare (<4-1%) HP:0003528
36 primary hyperparathyroidism 60 33 very rare (1%) Very rare (<4-1%) HP:0008200
37 benign gastrointestinal tract tumors 60 33 very rare (1%) Very rare (<4-1%) HP:0006719
38 increased circulating cortisol level 33 very rare (1%) HP:0003118
39 muscle cramps 60 Frequent (79-30%)
40 hypercortisolism 60 Very rare (<4-1%)
41 hematochezia 60 Excluded (0%)
42 abnormality of the thyroid gland 60 Very rare (<4-1%)
43 abnormality of the abdomen 60 Occasional (29-5%)

UMLS symptoms related to Vipoma:


diarrhea

MGI Mouse Phenotypes related to Vipoma:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 no phenotypic analysis MP:0003012 8.92 CHGA NTS SST SSTR3

Drugs & Therapeutics for Vipoma

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

(show top 50) (show all 52)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Capecitabine Approved, Investigational Phase 2,Phase 1 154361-50-9 60953
2
Temozolomide Approved, Investigational Phase 2,Phase 1 85622-93-1 5394
3
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
4
Gefitinib Approved, Investigational Phase 2 184475-35-2 123631
5
Octreotide Approved, Investigational Phase 2 83150-76-9 6400441 383414
6
Bevacizumab Approved, Investigational Phase 2 216974-75-3
7
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
8
Sirolimus Approved, Investigational Phase 2 53123-88-9 46835353 5284616 6436030
9
Everolimus Approved Phase 2 159351-69-6 70789204 6442177
10
Pasireotide Approved Phase 2 396091-73-9 9941444
11
Somatostatin Approved, Investigational Phase 2 38916-34-6, 51110-01-1 53481605
12 Alkylating Agents Phase 2,Phase 1
13 Antimetabolites Phase 2,Phase 1
14 Antimetabolites, Antineoplastic Phase 2,Phase 1
15 Antineoplastic Agents, Alkylating Phase 2,Phase 1
16 Epothilones Phase 2
17 Antimitotic Agents Phase 2,Phase 1
18 Epothilone B Phase 2
19 Protein Kinase Inhibitors Phase 2
20 Angiogenesis Modulating Agents Phase 2,Phase 1
21 Antineoplastic Agents, Immunological Phase 2,Phase 1
22 Angiogenesis Inhibitors Phase 2,Phase 1
23 Immunologic Factors Phase 2,Phase 1
24 Gastrointestinal Agents Phase 2
25 Antineoplastic Agents, Hormonal Phase 2
26 Immunoglobulin G Phase 2
27 Antifungal Agents Phase 2
28 Endothelial Growth Factors Phase 2
29 Anti-Infective Agents Phase 2
30 Anti-Bacterial Agents Phase 2
31 Antibodies, Monoclonal Phase 2
32 Immunoglobulins Phase 2
33 Mitogens Phase 2
34 Immunosuppressive Agents Phase 2
35 Antibiotics, Antitubercular Phase 2
36 Antibodies Phase 2
37 Hormone Antagonists Phase 2
38 Hormones Phase 2
39 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2
40
Carboplatin Approved Phase 1 41575-94-4 10339178 38904 498142
41
Paclitaxel Approved, Vet_approved Phase 1 33069-62-4 36314
42
Trastuzumab Approved, Investigational Phase 1 180288-69-1 9903
43
Pancrelipase Approved, Investigational Phase 1 53608-75-6
44 Interleukin-12 Phase 1
45 Albumin-Bound Paclitaxel Phase 1
46 Antineoplastic Agents, Phytogenic Phase 1
47 Adjuvants, Immunologic Phase 1
48 Hemostatics Phase 1
49 pancreatin Phase 1
50 Radiopharmaceuticals

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:

42
Pancreas, Small Intestine, Testes, Pituitary, Thyroid, Endothelial, Colon

Publications for Vipoma

Articles related to Vipoma:

(show top 50) (show all 102)
# 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
When Symptomatic Treatment Becomes Antitumor Treatment for Vipoma: Opportunity for Frail Elderly Adults. ( 26889855 )
2016
5
Ventricular fibrillation resulting from electrolyte imbalance reveals vipoma in MEN1 syndrome. ( 27071757 )
2016
6
Emergency therapy for liver metastases from advanced VIPoma: surgery or transarterial chemoembolization? ( 27583030 )
2016
7
VIPoma with multiple endocrine neoplasia type 1 identified as an atypical gene mutation. ( 26564120 )
2015
8
Sunitinib achieved fast and sustained control of VIPoma symptoms. ( 25305306 )
2015
9
Pancreatic VIPoma visualized by 68Ga DOTA-NOC PET-CT. ( 26235641 )
2015
10
Metastatic VIPoma presenting as an ovarian mass. ( 26657531 )
2015
11
Sporadic pancreatic vasoactive intestinal peptide-producing tumor (VIPoma) in a 47-year-old male. ( 24785507 )
2014
12
Extra-pancreatic vipoma. ( 25184777 )
2014
13
Contrast enhanced multi-detector CT and MR findings of a well-differentiated pancreatic vipoma. ( 25349667 )
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
Diagnosis and treatment of VIPoma: a case report and literature review in China. ( 22695090 )
2012
20
MSH2 and CXCR4 involvement in malignant VIPoma. ( 23231927 )
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
Amelioration of symptoms and reduction of VIP levels after hepatic artery chemoembolization in a patient with sandostatin resistant VIPoma. ( 20351979 )
2010
24
VIPoma syndrome: challenges in management. ( 20730389 )
2010
25
Is radiofrequency ablation justified for liver metastatic VIPoma patient undergoing Whipple procedure? ( 20819560 )
2010
26
Hypokalemic rhabdomyolysis due to watery diarrhea, hypokalemia, achlorhydria (WDHA) syndrome caused by vipoma. ( 19488018 )
2009
27
VIPoma with expression of both VIP and VPAC1 receptors in a patient with WDHA syndrome. ( 19184565 )
2009
28
Nesidioblastosis as a cause of focal pancreatic 111In-pentetreotide uptake in a patient with putative VIPoma: another differential diagnosis. ( 19387771 )
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
Radiofrequency ablation has a valuable therapeutic role in metastatic VIPoma. ( 16354964 )
2006
32
Diagnosis and treatment of VIPoma in a female patient. ( 16357627 )
2006
33
Calcitonin-secreting VIPoma. ( 16416161 )
2005
34
Diagnosis and treatment of VIPoma in China: (case report and 31 cases review) diagnosis and treatment of VIPoma. ( 14707737 )
2004
35
VIPoma: a rare cause of a pancreatic mass. ( 15162993 )
2004
36
Images in clinical medicine. VIPoma. ( 15317894 )
2004
37
VIPoma. ( 15590967 )
2004
38
[Jejunal vipoma]. ( 15646540 )
2004
39
Vasoactive intestinal polypeptide-secreting tumor (VIPoma) with liver metastases: dramatic and durable symptomatic benefit from hepatic artery embolization, a case report. ( 12482130 )
2002
40
Vipoma: effective treatment with octreotide in the oldest old. ( 11347804 )
2001
41
Dynamic gadolinium-enhanced MR imaging of pancreatic VIPoma in a patient with Verner-Morrison syndrome. ( 11702127 )
2001
42
Metastatic jejunal VIPoma: beneficial effect of combination therapy with interferon-alpha and 5-fluorouracil. ( 10638600 )
2000
43
VIPoma of pancreas in a child. ( 11059195 )
2000
44
Long-term survival after diagnosis of hepatic metastatic VIPoma: report of two cases with disparate courses and review of therapeutic options. ( 10389687 )
1999
45
The cost-effectiveness of octreotide acetate in the treatment of carcinoid syndrome and VIPoma. ( 9780538 )
1998
46
Metastatic pancreatic VIPoma: deteriorating clinical course and successful treatment by liver transplantation. ( 9577908 )
1998
47
Location of a VIPoma by iodine-123-vasoactive intestinal peptide scintigraphy. ( 9744346 )
1998
48
Vipoma/diarrheogenic syndrome: a statistical evaluation of 241 reported cases. ( 10089056 )
1998
49
MR imaging of metastatic pancreatic VIPoma. ( 9408142 )
1997
50
Pathophysiology and management of VIPoma: a case study. ( 8829164 )
1996

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
Show member pathways
13.13 GAST NTS SCT SST SSTR3 VIP
2 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 region GO:0005576 9.1 CHGA GAST NTS SCT SST VIP
2 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 9.43 GAST NTS SCT SST SSTR3 VIP
2 hormone-mediated apoptotic signaling pathway GO:0008628 9.16 SST SSTR3
3 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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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