MCID: VPM001
MIFTS: 46

Vipoma malady

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

Aliases & Classifications for Vipoma

Aliases & Descriptions for Vipoma:

Name: Vipoma 12 50 56 52 42 14 69
Pancreatic Cholera 56 69
Pancreatic Vipoma 50 69
Malignant Vasoactive Intestinal Peptide-Secreting Tumor 12
Watery Diarrhea-Hypokalemia-Achlorhydria Syndrome 56
Vasoactive Intestinal Peptide-Secreting Tumor 12
Vasoactive Intestinal Peptide-Producing Tumor 50
Vasoactive Intestinal Peptide Tumor 50
Diarrheogenic Islet Cell Tumor 56
Verner-Morrison Syndrome 56
Vip- Secreting Tumor 12
Vip-Secreting Tumor 56
Vipoma, Malignant 12
Malignant Vipoma 69
Wdha Syndrome 56

Characteristics:

Orphanet epidemiological data:

56
vipoma
Inheritance: Not applicable; Prevalence: <1/1000000 (Europe); Age of onset: All ages; Age of death: any age;

Classifications:



External Ids:

Disease Ontology 12 DOID:5574
MeSH 42 D003969
NCIt 47 C26749
SNOMED-CT 64 253005002 447643008
Orphanet 56 ORPHA97282
ICD10 via Orphanet 34 E16.8
MESH via Orphanet 43 D003969
UMLS via Orphanet 70 C0011993
UMLS 69 C0011993

Summaries for Vipoma

NIH Rare Diseases : 50 vipoma is a rare cancer that develops within the pancreas. this tumor causes pancreatic cells to produce high levels of a hormone called vasoactive intestinal peptide (vip). the signs and symptoms of a vipoma include abdominal pain, flushing or redness of the face, nausea, watery diarrhea, weight loss, dehydration, and low blood potassium (hypokalemia). vipomas are usually diagnosed in adults around age 50. the cause of vipoma is unknown. treatment may include intravenous (iv) fluids to correct dehydration, medications such as octreotide to help control diarrhea, and surgery to remove the tumor. last updated: 3/8/2010

MalaCards based summary : Vipoma, also known as pancreatic cholera, is related to pancreatic vasoactive intestinal peptide producing tumor and pancreatic cholera, and has symptoms including nausea and vomiting, generalized muscle weakness and diabetes mellitus. 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, liver and thyroid.

Wikipedia : 71 A VIPoma (also known as Verner–Morrison syndrome, after the physicians who first described it) is a... more...

Related Diseases for Vipoma

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

(show top 50) (show all 102)
id Related Disease Score Top Affiliating Genes
1 pancreatic vasoactive intestinal peptide producing tumor 11.2
2 pancreatic cholera 10.8
3 pancreatitis 10.3
4 inflammatory diarrhea 10.2 SST VIP
5 drug psychosis 10.2 SST VIP
6 placenta accreta 10.2 CALCA VIP
7 cockayne syndrome type i 10.2 CALCA VIP
8 pemphigus foliaceus 10.2 CALCA SST
9 nutritional optic neuropathy 10.2 GAST SST
10 cough variant asthma 10.2 CALCA VIP
11 cherubism 10.2 GAST SST
12 brain ependymoma 10.1 CALCA SST
13 myocardial stunning 10.1 GAST SST
14 lung occult small cell carcinoma 10.1 CALCA SST
15 bile acid synthesis defect, congenital, 4 10.1 SST VIP
16 alpha-heavy chain disease 10.1 CALCA GAST
17 color blindness 10.1 GAST SST
18 oculocutaneous albinism 10.1 CHGA SST
19 axial osteomalacia 10.1 CALCA SST
20 breast secretory carcinoma 10.1 CHGA SST
21 childhood intracortical osteosarcoma 10.1 GAST SST
22 autoimmune pancreatitis 10.1 CHGA GAST
23 bizarre leiomyoma 10.1 CALCA GAST
24 mitochondrial complex ii deficiency 10.1 CHGA SST
25 adult ependymoblastoma 10.1 CALCA CHGA
26 perineural angioma 10.1 CALCA CHGA
27 gastric duplication cysts 10.1 CHGA GAST
28 granulocytopenia 10.1 CHGA GAST
29 parietal lobe neoplasm 10.1 CALCA CHGA
30 non-syndromic x-linked intellectual disability 10.1 CHGA SST
31 retinal vascular occlusion 10.1 GAST SST
32 acinar cell cystadenocarcinoma 10.1 CHGA GAST
33 testicular trophoblastic tumor 10.1 CALCA CHGA
34 urinary tract papillary transitional cell benign neoplasm 10.0 CALCA CHGA
35 aneurysm 10.0 CALCA CHGA
36 cowpox 10.0 CHGA GAST
37 band keratopathy 10.0 SST VIP
38 parathyroid carcinoma 10.0 CALCA CHGA
39 peripheral degeneration of cornea 10.0 NTS SST
40 childhood teratocarcinoma of the testis 10.0 CALCA CHGA
41 meige syndrome 10.0 CHGA SST
42 wells-jankovic syndrome 10.0 CALCA SST VIP
43 rickets 10.0 NTS SST
44 brown-vialetto-van laere syndrome 10.0 CALCA CHGA
45 diarrhea 10.0
46 anisometropia 10.0 CHGA SST
47 gmppa-cdg 10.0 CHGA SST VIP
48 bone dysplasia azouz type 10.0 CHGA SST VIP
49 myoepithelial carcinoma 10.0 CHGA SST VIP
50 hepatitis 9.9

Graphical network of the top 20 diseases related to Vipoma:



Diseases related to Vipoma

Symptoms & Phenotypes for Vipoma

Human phenotypes related to Vipoma:

56 32 (show all 42)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 nausea and vomiting 56 32 Frequent (79-30%) HP:0002017
2 generalized muscle weakness 56 32 Frequent (79-30%) HP:0003324
3 diabetes mellitus 56 32 Frequent (79-30%) HP:0000819
4 hepatomegaly 56 32 Frequent (79-30%) HP:0002240
5 malabsorption 56 32 Frequent (79-30%) HP:0002024
6 dehydration 56 32 Frequent (79-30%) HP:0001944
7 hypokalemia 56 32 Very frequent (99-80%) HP:0002900
8 respiratory insufficiency due to muscle weakness 56 32 Very rare (<4-1%) HP:0002747
9 ascites 56 32 Occasional (29-5%) HP:0001541
10 weight loss 56 32 Frequent (79-30%) HP:0001824
11 increased circulating gonadotropin level 56 32 Very rare (<4-1%) HP:0000837
12 anorexia 56 32 Frequent (79-30%) HP:0002039
13 chronic fatigue 56 32 Frequent (79-30%) HP:0012432
14 muscle cramps 56 32 Frequent (79-30%) HP:0003394
15 extrahepatic cholestasis 56 32 Occasional (29-5%) HP:0012334
16 parathyroid adenoma 56 32 Very rare (<4-1%) HP:0002897
17 hypercalcemia 56 32 Frequent (79-30%) HP:0003072
18 growth hormone excess 56 32 Very rare (<4-1%) HP:0000845
19 subcutaneous lipoma 56 32 Very rare (<4-1%) HP:0001031
20 episodic abdominal pain 56 32 Frequent (79-30%) HP:0002574
21 pituitary adenoma 56 32 Very rare (<4-1%) HP:0002893
22 adrenocortical adenoma 56 32 Very rare (<4-1%) HP:0008256
23 erythema 56 32 Frequent (79-30%) HP:0010783
24 neoplasm of the pancreas 56 32 Very frequent (99-80%) HP:0002894
25 follicular thyroid carcinoma 56 32 Very rare (<4-1%) HP:0006731
26 neoplasm of the liver 56 32 Very rare (<4-1%) HP:0002896
27 intrahepatic cholestasis 56 32 Occasional (29-5%) HP:0001406
28 prolactin excess 56 32 Very rare (<4-1%) HP:0000870
29 ganglioneuroma 56 32 Very rare (<4-1%) HP:0003005
30 poor appetite 56 32 Frequent (79-30%) HP:0004396
31 elevated calcitonin 56 32 Very rare (<4-1%) HP:0003528
32 primary hyperparathyroidism 56 32 Very rare (<4-1%) HP:0008200
33 intermittent jaundice 56 32 Occasional (29-5%) HP:0001046
34 secretory diarrhea 56 32 Very frequent (99-80%) HP:0005208
35 normochromic anemia 56 32 Frequent (79-30%) HP:0001895
36 abnormal gastrointestinal motility 56 32 Occasional (29-5%) HP:0030895
37 benign gastrointestinal tract tumors 56 32 Very rare (<4-1%) HP:0006719
38 hypercortisolism 56 Very rare (<4-1%)
39 hematochezia 56 Excluded (0%)
40 abnormality of the thyroid gland 56 Very rare (<4-1%)
41 increased circulating cortisol level 32 HP:0003118
42 abnormality of the abdomen 56 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 56)
id 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
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
5
Gefitinib Approved, Investigational Phase 2 184475-35-2 123631
6
Bevacizumab Approved, Investigational Phase 2 216974-75-3
7
Everolimus Approved Phase 2 159351-69-6 6442177
8
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
9
Octreotide Approved, Investigational Phase 2 83150-76-9 383414 6400441
10
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
11
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
12
Niacin Approved, Investigational, Nutraceutical Phase 2 59-67-6 938
13
Nicotinamide Approved, Nutraceutical Phase 2 98-92-0 936
14
Epothilone B Experimental, Investigational Phase 2 152044-54-7
15 Antimitotic Agents Phase 2,Phase 1
16 Epothilones Phase 2
17 Alkylating Agents Phase 2,Phase 1
18 Antimetabolites Phase 2,Phase 1
19 Antimetabolites, Antineoplastic Phase 2,Phase 1
20 Antineoplastic Agents, Alkylating Phase 2,Phase 1
21 Micronutrients Phase 2
22 Nicotinic Acids Phase 2
23 Protein Kinase Inhibitors Phase 2
24 Trace Elements Phase 2
25 Vitamin B Complex Phase 2
26 Vitamins Phase 2
27 Angiogenesis Inhibitors Phase 2,Phase 1
28 Angiogenesis Modulating Agents Phase 2,Phase 1
29 Anti-Bacterial Agents Phase 2
30 Antibiotics, Antitubercular Phase 2
31 Antibodies Phase 2
32 Antibodies, Monoclonal Phase 2
33 Antifungal Agents Phase 2
34 Anti-Infective Agents Phase 2
35 Antineoplastic Agents, Hormonal Phase 2
36 Endothelial Growth Factors Phase 2
37 Gastrointestinal Agents Phase 2
38 Immunoglobulin G Phase 2
39 Immunoglobulins Phase 2
40 Immunosuppressive Agents Phase 2
41 Mitogens Phase 2
42 Folate Nutraceutical Phase 2
43 Vitamin B3 Nutraceutical Phase 2
44 Vitamin B9 Nutraceutical Phase 2
45
Carboplatin Approved Phase 1 41575-94-4 10339178 498142 38904
46
Paclitaxel Approved, Vet_approved Phase 1 33069-62-4 36314
47
Trastuzumab Approved, Investigational Phase 1 180288-69-1 9903
48
Pancrelipase Approved Phase 1 53608-75-6
49
Veliparib Investigational Phase 1 912444-00-9 11960529
50 Adjuvants, Immunologic Phase 1

Interventional clinical trials:


id Name Status NCT ID Phase
1 EPO906 in Carcinoid and Other Neuroendocrine Tumors Completed NCT00050349 Phase 2
2 Capecitabine and Temozolomide for Neuroendocrine Cancers Completed NCT00869050 Phase 2
3 Sorafenib Tosylate in Treating Patients With Progressive Metastatic Neuroendocrine Tumors Completed NCT00131911 Phase 2
4 Gefitinib in Treating Patients With Progressive Metastatic Neuroendocrine Tumors Completed NCT00075439 Phase 2
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
6 Interleukin-12 and Trastuzumab in Treating Patients With Cancer That Has High Levels of HER2/Neu Completed NCT00004074 Phase 1
7 Laser Tissue Welding - Distal Pancreatectomy Sealing Study Recruiting NCT03147768 Phase 1
8 Veliparib, Capecitabine, and Temozolomide in Patients With Advanced, Metastatic, and Recurrent Neuroendocrine Tumor Not yet recruiting NCT02831179 Phase 1
9 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:

39
Pancreas, Liver, Thyroid, Pituitary, Lung, Colon

Publications for Vipoma

Articles related to Vipoma:

(show top 50) (show all 102)
id Title Authors Year
1
Percutaneous Irreversible Electroporation for Pancreatic VIPoma: A Case Report. ( 27977628 )
2017
2
Emergency therapy for liver metastases from advanced VIPoma: surgery or transarterial chemoembolization? ( 27583030 )
2016
3
When Symptomatic Treatment Becomes Antitumor Treatment for Vipoma: Opportunity for Frail Elderly Adults. ( 26889855 )
2016
4
Ventricular fibrillation resulting from electrolyte imbalance reveals vipoma in MEN1 syndrome. ( 27071757 )
2016
5
VIPoma with multiple endocrine neoplasia type 1 identified as an atypical gene mutation. ( 26564120 )
2015
6
Metastatic VIPoma presenting as an ovarian mass. ( 26657531 )
2015
7
Pancreatic VIPoma visualized by 68Ga DOTA-NOC PET-CT. ( 26235641 )
2015
8
Sporadic pancreatic vasoactive intestinal peptide-producing tumor (VIPoma) in a 47-year-old male. ( 24785507 )
2014
9
Sunitinib Achieved Fast and Sustained Control of VIPoma Symptoms. ( 25305306 )
2014
10
Contrast enhanced multi-detector CT and MR findings of a well-differentiated pancreatic vipoma. ( 25349667 )
2014
11
Extra-pancreatic vipoma. ( 25184777 )
2014
12
VIPoma in a 37-year-old man. ( 23993192 )
2013
13
A rare cause of diarrhea: pancreatic VIPoma. ( 24008482 )
2013
14
Sunitinib for the treatment of metastatic paraganglioma and vasoactive intestinal polypeptide-producing tumor (VIPoma). ( 23407483 )
2013
15
Ischemic stroke as a presenting feature of VIPoma due to MEN 1 syndrome. ( 24251163 )
2013
16
Education and imaging. Hepatobiliary and pancreatic: pancreatic VIPomas associated with multiple endocrine neoplasia type I. ( 22353352 )
2012
17
VIPoma that arose from the rectum in a 65-year-old male patient. ( 22350188 )
2012
18
Diagnosis and treatment of VIPoma: a case report and literature review in China. ( 22695090 )
2012
19
MSH2 and CXCR4 involvement in malignant VIPoma. ( 23231927 )
2012
20
Benign pancreatic vipoma. ( 21526882 )
2011
21
VIPoma Crisis: Immediate and life saving reduction of massive stool volumes on starting treatment with octreotide. ( 21509215 )
2011
22
Is radiofrequency ablation justified for liver metastatic VIPoma patient undergoing Whipple procedure? ( 20819560 )
2010
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
Hypokalemic rhabdomyolysis due to watery diarrhea, hypokalemia, achlorhydria (WDHA) syndrome caused by vipoma. ( 19488018 )
2009
26
VIPoma with expression of both VIP and VPAC1 receptors in a patient with WDHA syndrome. ( 19184565 )
2009
27
Nesidioblastosis as a cause of focal pancreatic 111In-pentetreotide uptake in a patient with putative VIPoma: another differential diagnosis. ( 19387771 )
2009
28
Multi-visceral resection of pancreatic VIPoma in a patient with sinistral portal hypertension. ( 18662399 )
2008
29
[Diagnostic procedures and more particularly, place of scintigraphy in neuroendocrine tumors, example of vipoma in MEN 1]. ( 17292846 )
2007
30
Radiofrequency ablation has a valuable therapeutic role in metastatic VIPoma. ( 16354964 )
2006
31
Diagnosis and treatment of VIPoma in a female patient. ( 16357627 )
2006
32
Calcitonin-secreting VIPoma. ( 16416161 )
2005
33
VIPomas: an update in diagnosis and management in a series of 11 patients. ( 16001675 )
2005
34
VIPoma. ( 15590967 )
2004
35
Diagnosis and treatment of VIPoma in China: (case report and 31 cases review) diagnosis and treatment of VIPoma. ( 14707737 )
2004
36
VIPoma: a rare cause of a pancreatic mass. ( 15162993 )
2004
37
[Jejunal vipoma]. ( 15646540 )
2004
38
Vasoactive intestinal polypeptide-secreting tumor (VIPoma) with liver metastases: dramatic and durable symptomatic benefit from hepatic artery embolization, a case report. ( 12482130 )
2002
39
Dynamic gadolinium-enhanced MR imaging of pancreatic VIPoma in a patient with Verner-Morrison syndrome. ( 11702127 )
2001
40
Vipoma: effective treatment with octreotide in the oldest old. ( 11347804 )
2001
41
VIPoma of pancreas in a child. ( 11059195 )
2000
42
Vasoactive Intestinal Peptide Tumor (VIPoma) ( 25905195 )
2000
43
Metastatic jejunal VIPoma: beneficial effect of combination therapy with interferon-alpha and 5-fluorouracil. ( 10638600 )
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
Location of a VIPoma by iodine-123-vasoactive intestinal peptide scintigraphy. ( 9744346 )
1998
46
The cost-effectiveness of octreotide acetate in the treatment of carcinoid syndrome and VIPoma. ( 9780538 )
1998
47
Vipoma/diarrheogenic syndrome: a statistical evaluation of 241 reported cases. ( 10089056 )
1998
48
Metastatic pancreatic VIPoma: deteriorating clinical course and successful treatment by liver transplantation. ( 9577908 )
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:

id Super pathways Score Top Affiliating Genes
1 9.47 GAST VIP

GO Terms for Vipoma

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

id Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.26 CALCA CHGA GAST SST
2 transport vesicle GO:0030133 9.16 CHGA NTS
3 extracellular region GO:0005576 9.1 CALCA CHGA GAST NTS SST VIP

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

id Name GO ID Score Top Affiliating Genes
1 regulation of blood pressure GO:0008217 8.96 CALCA CHGA
2 response to heat GO:0009408 8.62 CALCA SST

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

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

Sources for Vipoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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