Vipoma malady

NIH Rare Diseases: 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.³⁰

MalaCards: Vipoma, also known as vasoactive intestinal peptide-secreting tumor (disorder), is related to wdha syndrome and parathyroid adenoma. An important gene associated with Vipoma is VIP (vasoactive intestinal peptide), and among its related pathways are Class A/1 (Rhodopsin-like receptors) and Class B/2 (Secretin family receptors). The compounds c-peptide and helospectin have been mentioned in the context of this disorder. Affiliated tissues include liver, pancreas and pancreatic islet, and related mouse phenotypes are endocrine/exocrine gland and growth/size.

Wikipedia: A VIPoma (also known as Verner Morrison syndrome, after the physicians who first described it) is a rare...⁴⁴ more...

Aliases & Descriptions:

vipoma 6 30 8 32 vasoactive intestinal peptide-secreting tumor (disorder) 6 malignant vasoactive intestinal peptide-secreting tumor 6 vasoactive intestinal peptide-producing tumor 30 vasoactive intestinal peptide (vip) tumor 30

vip- secreting tumor 6 vip producing tumor 43 pancreatic vipoma 30 vipoma, malignant 6 malignant vipoma 43

Diseases related to vipoma by text searches and GeneDecks gene sharing:

(show top 50)    (show all 222)

id	Related Disease	Score	Top Affiliating Genes
1	wdha syndrome	28.0	VIP, CALCA, SST
2	parathyroid adenoma	26.6	VIP, CHGA, MEN1, CALCA
3	diarrhea	26.1	VIP, SCT, CHGA, CALCA, SST, NTS
4	pancreatitis	25.0	VIP, SCT, CHGA, MEN1, CALCA, GH1
5	neuroendocrine tumor	24.3	VIP, SCT, CHGA, MEN1, CALCA, GH1
6	adenocarcinoma	22.5	VIP, SCT, CHGA, MEN1, CALCA, GH1
7	adenoma	20.9	VIP, SCT, CHGA, MEN1, CALCA, GH1
8	intestinal neuroendocrine tumor	13.4	CHGA, SST
9	angiodysplasia	13.4	SCT, SST
10	pancreatic islet cell tumors	13.4	SST, GAST
11	choledochal cyst	13.3	SCT, SST
12	gastrointestinal neuroendocrine tumor	13.3	CHGA, SST
13	duodenal ulcer	13.3	SCT, GAST
14	pituitary-dependent cushing's disease	13.3	GH1, SST
15	cushing's syndrome	13.3	GH1, SST
16	fetal adenoma	13.2	GH1, GHRH
17	peutz-jeghers syndrome	13.2	SMAD4, GAST
18	mitochondrial encephalomyopathy lactic acidosis and stroke-like episodes	13.2	GH1, GHRH
19	middle ear cancer	13.2	CHGA, PPY
20	megacolon	13.2	VIP, GAST
21	dumping syndrome	13.2	SCT, SST, NTS
22	pancreatoblastoma	13.2	CHGA, SMAD4
23	ganglioneuroblastoma	13.2	VIP, CHGA, SST
24	vasomotor rhinitis	13.2	VIP, CALCA
25	pancreatic ductal carcinoma	13.2	CHGA, SMAD4
26	ganglioneuroma	13.2	VIP, CHGA, SST
27	pituitary carcinoma	13.2	CHGA, GHRH, SST
28	growth disorders	13.1	GH1, GHRH
29	duodenogastric reflux	13.1	SCT, NTS, GAST
30	megaloblastic anemia	13.1	CHGA, GAST
31	merkel cell carcinoma	13.1	CHGA, SST
32	gastroesophageal reflux disease	13.1	SCT, NTS, GAST
33	irritable bowel syndrome	13.1	VIP, CHGA, SST
34	adrenal gland hyperfunction	13.1	GH1, GHRH, SST
35	adrenocortical carcinoma	13.1	VIP, CHGA, SMAD4
36	acinar cell carcinoma	13.1	CHGA, SMAD4
37	hypocalciuric hypercalcemia	13.0	CALCA, GAST
38	hypopituitarism	13.0	GH1, GHRH, SST
39	idiopathic juvenile osteoporosis	13.0	CALCA, GH1
40	eating disorder	13.0	GH1, SST, NTS
41	obstructive jaundice	13.0	SCT, GH1, NTS
42	addison's disease	13.0	CHGA, SST, GAST
43	cystinosis	13.0	GH1, SST, GAST
44	ovarian mucinous neoplasm	13.0	CHGA, SST, SMAD4
45	hypoadrenalism	13.0	CALCA, GH1
46	constipation	12.9	VIP, CHGA, SST, NTS
47	esophageal atresia	12.9	VIP, CHGA, CALCA
48	apudoma	12.9	CALCA, SST, GAST
49	orthostatic hypotension	12.9	CHGA, CALCA, SST
50	teratoma	12.9	GH1, SST, NTS

Approved drugs:

Drug clinical trials:

MalaCards organs/tissues related to vipoma:

²²

MGI Mouse Phenotypes related to vipoma:

²⁵

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	endocrine/exocrine gland phenotype	MP:0005379	9.3	GHRH, MEN1
2	growth/size phenotype	MP:0005378	7.3	PPY, NTS, SST, GHRH, GH1, MEN1
3	nervous system phenotype	MP:0003631	6.9	SMAD4, NTS, SST, GHRH, GH1, CALCA
4	homeostasis/metabolism phenotype	MP:0005376	6.3	VIP, GAST, SMAD4, NTS, SST, GHRH

Articles related to vipoma:

(show all 22)

id	Title	Authors	Year	Affiliating Genes
1	VIPoma with expression of both VIP and VPAC1 receptors in a patient with WDHA syndrome. (19184565)	Nakayama S.... Hanafusa T.	2009	VIP
2	Diagnostic procedures and more particularly, place of scintigraphy in neuroendocrine tumors, example of vipoma in MEN 1 (17292846)	Rigabert J.... De Clermont H.	2007	CALCA
3	VIPomas: an update in diagnosis and management in a series of 11 patients. (16001675)	Nikou G.C.... Polyzos A.	2005	SST, VIP
4	Jejunal vipoma (15646540)	LecorguillAc M.... Ruszniewski P.	2004	SST
5	Metastatic jejunal VIPoma: beneficial effect of combination therapy with interferon-alpha and 5-fluorouracil. (10638600)	Cellier C.... Landi B.	2000	SST
6	Vipoma/diarrheogenic syndrome: a statistical evaluati on of 241 reported cases. (10089056)	Soga J.... Yakuwa Y.	1998	SST
7	Location of a VIPoma by iodine-123-vasoactive intestinal peptide scintigraphy. (9744346)	Virgolini I.... Niederle B.	1998	SSTR3, VIP
8	Massive amyloid deposition in pancreatic vipoma: a ca se report. (9007963)	Crowley P.F.... Rode J.	1996	CALCA
9	Metastasizing pancreatic vipoma. Its diagnosis and therapy with the somatostatin analog octreotide (7588006)	Scheffold N.... Cyran J.	1995	SST, VIP
10	In vitro release of vasoactive intestinal polypeptide and pancreatic polypeptide from human VIPoma cells and its inhibition by somatostatin analogue (SMS 201-995). (8197564)	Yasunami Y.... Ikeda S.	1994	SST
11	Liver VIPoma: report of two cases and literature review. (7950821)	Lundstedt C.... Amin T.	1994	SST
12	Stimulation of vasoactive intestinal peptide and neurotensin secretion by pentagastrin in a patient with VIPoma syndrome. (8128360)	Brunt L.M.... Wells S.A.	1994	VIP, NTS
13	Tc-99m sestamibi imaging of a pancreatic VIPoma and parathyroid adenoma in a patient with multiple type I endocrine neoplasia. (7914844)	Cesani F.... Villanueva-Meyer J.	1994	VIP
14	Calcitonin, as SMS 201-995, ameliorates the VIPoma syndrome. (8383153)	Antonelli A.... Baschieri L.	1993	VIP
15	VIPoma: surgical treatment (1314346)	Procacciante F.... Romeo F.	1992	SST
16	Long-term therapy of a metastasizing pancreatic vipoma using the somatostatin derivative octreotide (1326786)	FlA1ckiger A.... Schlup P.	1992	SST
17	Giant metastatic VIPoma in the liver. (1655553)	Dohmen K.... Hachimine K.	1991	VIP
18	Vipoma in an adolescent: treatment with a delayed-action somatostatin analog, octreotide or SMS 201-995, and surgical removal (2176428)	Brichard B.... Cornu G.	1990	SST, VIP
19	Vipoma of the pancreas. Apropos of a case and review of the literature (2167711)	Riestra S.... Rabal A.	1990	VIP
20	The somatostatin analog SMS 201-995 in long-term tre atment of vipoma (2177234)	Christensen C.	1990	SST
21	Treatment of metastatic Vipoma by liver transplantation. (10150088)	Bramley P.N.... Giles G.R.	1990	VIP
22	Diarrhoea in vipoma patients associated with cosecretion of a second active peptide (peptide histidine isoleucine) explained by single coding gene. (6139527)	Bloom S.R.... Polak J.M.	1983	VIP

Genes related to vipoma according to GeneCards:

(show all 14)

id	Symbol	Description	Score	GeneCards Section Context
1	VIP	vasoactive intestinal peptide	11.0	Publications
2	SST	somatostatin	11.0	Publications
3	NTS	neurotensin	11.0	Publications
4	GHRH	growth hormone releasing hormone	11.0
5	SCT	secretin	11.0
6	GAST	gastrin	11.0
7	CHGA	chromogranin A (parathyroid secretory protein 1)	11.0
8	SMAD4	SMAD family member 4	11.0
9	GH1	growth hormone 1	11.0
10	PPY	pancreatic polypeptide	11.0
11	SSTR3	somatostatin receptor 3	11.0	Publications
12	SMS	spermine synthase	11.0
13	MEN1	multiple endocrine neoplasia I	11.0
14	CALCA	calcitonin-related polypeptide alpha	11.0	Publications

Pathways related to vipoma according to GeneDecks:

id	Pathway	Score	Top Affiliating Genes
1	Class A/1 (Rhodopsin-like receptors)38	9.2	SST, SSTR3, PPY
2	Class B/2 (Secretin family receptors)38	9.1	SCT, CALCA, GHRH

Compounds related to vipoma according to GeneDecks:

(show top 50)    (show all 129)

id	Compound	Score	Top Affiliating Genes
1	c-peptide32	10.6	SCT
2	helospectin32	10.4	SCT
3	carbachol32 9 9	12.0	SCT, SST, GAST
4	minigastrin32	9.9	GAST, SST, VIP
5	secretoneurin32	9.8	VIP, SCT, CHGA
6	loxiglumide32	9.7	SST, GAST, NTS, SCT
7	5-hydroxyindoleacetic acid32 18	10.7	VIP, SST, GAST, CHGA
8	pirenzepine32 9 9	11.6	GH1, SST, GAST, GHRH
9	gnrh32	9.6	GHRH, SMAD4, NTS, GH1
10	lanreotide32	9.6	CHGA, GH1, GHRH, SST
11	omeprazole32 42 34 9 18 9	14.5	GAST, CHGA, SCT, SST
12	pepsinogen32	9.5	SCT, GAST, CHGA
13	111in-pentetreotide32	9.5	SST, CALCA, CHGA
14	lafutidine32	9.5	SST, CALCA, SCT, GAST
15	epinephrine32 9 18 9	12.4	CHGA, VIP, NTS, GAST, SCT
16	naloxone32 34 9 9	12.4	SST, NTS, GH1, GHRH, VIP
17	bromocriptine32 9 9	11.4	GHRH, GH1, VIP, SST
18	sumatriptan32 34 9 18 9	13.4	VIP, CALCA, GH1, GHRH
19	glucose32	9.3	NTS, SMAD4, GAST, SCT
20	levodopa32 9 9	11.3	SST, NTS, GHRH, GH1, CHGA
21	glutamate32	9.2	VIP, CALCA, GHRH, NTS, SST
22	oxyntomodulin32 42	10.2	NTS, SST, CHGA, SCT, VIP, GAST
23	gaba32 42	10.1	VIP, SCT, CHGA, SST, NTS, GAST
24	corticosterone32 18	10.0	VIP, GH1, CALCA, NTS, GAST
25	ibmx32	9.0	GH1, VIP, SCT, CALCA, SST
26	5-hydroxytryptamine32	8.9	GAST, VIP, CHGA, CALCA, SST, NTS
27	propranolol32 34 9 18 9	12.9	GAST, GHRH, CALCA, CHGA, VIP, SCT
28	tetrodotoxin32	8.8	SCT, CALCA, NTS, VIP
29	nifedipine32 9 9	10.8	CHGA, GAST, GH1, GHRH, SST, VIP
30	guanine32 9 18 9	11.8	NTS, GH1, CHGA, SCT, VIP, CALCA
31	histamine32 18	9.6	CHGA, CALCA, SST, NTS, VIP, SCT
32	clonidine32 9 9	10.5	GHRH, NTS, GH1, CALCA, CHGA, SCT
33	forskolin32 42 9 9	11.5	VIP, SCT, CHGA, CALCA, GH1, NTS
34	dexamethasone32 42 34 9 9	12.4	GAST, GHRH, VIP, GH1, SST, CHGA
35	atropine32 9 9	10.3	GH1, VIP, SCT, CALCA, GHRH, SST
36	arginine32	8.3	GH1, SMAD4, NTS, SST, GHRH, SCT
37	thyroxine32 18	9.3	CALCA, GHRH, VIP, NTS, SCT, GAST
38	isoproterenol32 9 9	10.2	GH1, SST, GAST, CALCA, VIP, SCT
39	acetylcholine32 9 18 9	11.2	GAST, NTS, SST, GH1, CALCA, CHGA
40	thymidine32 18	9.0	GH1, NTS, GAST, SCT, MEN1, SMAD4
41	vitamin d32	8.0	GAST, GH1, CALCA, MEN1, CHGA, GHRH
42	acth32	8.0	GAST, NTS, SST, GHRH, GH1, CALCA
43	norepinephrine32 9 18 9	11.0	GAST, VIP, NTS, SST, GHRH, GH1
44	dopamine32 9 18 9	10.9	VIP, SCT, GAST, NTS, SST, CHGA
45	adenylate32	7.9	CHGA, CALCA, GH1, GHRH, SST, NTS
46	testosterone32 9 18 9	10.6	GAST, SST, VIP, GHRH, CHGA, SMAD4
47	vegf32	7.5	CHGA, SMAD4, SST, GH1, MEN1, GAST
48	octreotide32 42 9 9	10.5	GH1, GHRH, SST, SSTR3, VIP, GAST
49	estrogen32	7.3	MEN1, GH1, CALCA, GHRH, SMAD4, GAST
50	serine32	7.0	CHGA, VIP, SCT, NTS, GHRH, SMAD4

Cellular components related to vipoma according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	extracellular region	GO:005576	7.1	VIP, PPY, NTS, SST, GHRH, GH1

Biological processes related to vipoma according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	induction of apoptosis by hormones	GO:008628	9.8	SST, SSTR3
2	positive regulation of insulin-like growth factor receptor signaling pathway	GO:043568	9.7	GHRH, GH1
3	positive regulation of transforming growth factor beta receptor signaling pathway	GO:030511	9.3	MEN1, SMAD4
4	cell-cell signaling	GO:007267	8.9	CALCA, GHRH, SST, SSTR3
5	negative regulation of cell proliferation	GO:008285	8.2	MEN1, SST, SSTR3, SMAD4

Molecular functions related to vipoma according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	R-SMAD binding	GO:070412	9.3	MEN1, SMAD4
2	hormone activity	GO:005179	7.7	VIP, SCT, CALCA, GH1, SST, PPY