VIPoma

Summary

VIPoma (MONDO:0019960) is a disease and 5 clinical trials. Top therapeutic interventions include bevacizumab, edotreotide gallium ga-68, and octreotide acetate. A subtype of pancreatic neuroendocrine tumor — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: <1 / 1 000 000 (Europe) [Orphanet-validated]
• Phenotypes (HPO): 41
• Clinical trials: 5

Clinical features

Epidemiology

Prevalence records

2 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	<1 / 1 000 000		Europe	Validated
Point prevalence	<1 / 1 000 000		Europe	Validated

Signs & symptoms

Clinical features (HPO)

41 HPO clinical features (Orphanet curated; top 41 by frequency):

HPO ID	Term	Frequency
HP:0002894	Neoplasm of the pancreas	Very frequent (80-99%)
HP:0002900	Hypokalemia	Very frequent (80-99%)
HP:0005208	Secretory diarrhea	Very frequent (80-99%)
HP:0000819	Diabetes mellitus	Frequent (30-79%)
HP:0001824	Weight loss	Frequent (30-79%)
HP:0001895	Normochromic anemia	Frequent (30-79%)
HP:0001944	Dehydration	Frequent (30-79%)
HP:0002017	Nausea and vomiting	Frequent (30-79%)
HP:0002024	Malabsorption	Frequent (30-79%)
HP:0002039	Anorexia	Frequent (30-79%)
HP:0002240	Hepatomegaly	Frequent (30-79%)
HP:0002574	Episodic abdominal pain	Frequent (30-79%)
HP:0003072	Hypercalcemia	Frequent (30-79%)
HP:0003324	Generalized muscle weakness	Frequent (30-79%)
HP:0003394	Muscle spasm	Frequent (30-79%)
HP:0004396	Poor appetite	Frequent (30-79%)
HP:0010783	Erythema	Frequent (30-79%)
HP:0012432	Chronic fatigue	Frequent (30-79%)
HP:0001046	Intermittent jaundice	Occasional (5-29%)
HP:0001406	Intrahepatic cholestasis	Occasional (5-29%)
HP:0001438	Abnormality of abdomen morphology	Occasional (5-29%)
HP:0001541	Ascites	Occasional (5-29%)
HP:0012334	Extrahepatic cholestasis	Occasional (5-29%)
HP:0030895	Abnormal gastrointestinal motility	Occasional (5-29%)
HP:0002573	Hematochezia	Excluded (0%)
HP:0000820	Abnormality of the thyroid gland	Very rare (<1-4%)
HP:0000837	Increased circulating gonadotropin level	Very rare (<1-4%)
HP:0000845	Elevated circulating growth hormone concentration	Very rare (<1-4%)
HP:0000870	Increased circulating prolactin concentration	Very rare (<1-4%)
HP:0001031	Subcutaneous lipoma	Very rare (<1-4%)
HP:0002747	Respiratory insufficiency due to muscle weakness	Very rare (<1-4%)
HP:0002893	Pituitary adenoma	Very rare (<1-4%)
HP:0002896	Neoplasm of the liver	Very rare (<1-4%)
HP:0002897	Parathyroid adenoma	Very rare (<1-4%)
HP:0003005	Ganglioneuroma	Very rare (<1-4%)
HP:0003118	Increased circulating cortisol level	Very rare (<1-4%)
HP:0003528	Elevated calcitonin	Very rare (<1-4%)
HP:0006719	Benign gastrointestinal tract tumors	Very rare (<1-4%)
HP:0006731	Follicular thyroid carcinoma	Very rare (<1-4%)
HP:0008200	Primary hyperparathyroidism	Very rare (<1-4%)
HP:0008256	Adrenocortical adenoma	Very rare (<1-4%)

Identifiers

Disease identifiers

Field	Value
Canonical name	VIPoma
Mondo ID	MONDO:0019960
MeSH	D003969
Orphanet	97282
DOID	DOID:5574
ICD-11	20634476
NCIT	C26749
SNOMED CT	253005002
UMLS	C0011993
MedGen	41532
GARD	0003787
MedDRA	10047430
Is cancer (heuristic)	no

Also known as: Diarrheogenic islet cell tumor · Diarrheogenic islet cell tumour · malignant vasoactive intestinal peptide-secreting tumour · pancreatic cholera · pancreatic vipoma · vasoactive intestinal peptide (VIP) tumor · vasoactive intestinal peptide (VIP) tumour · vasoactive intestinal peptide producing neoplasm · vasoactive intestinal peptide producing tumor · vasoactive intestinal peptide producing tumour · vasoactive intestinal peptide secreting neoplasm · vasoactive intestinal peptide-producing tumor · vasoactive intestinal peptide-producing tumour · vasoactive intestinal peptide-secreting tumour · Verner-Morrison syndrome · VIP producing neoplasm · VIP- secreting neoplasm · VIP- secreting tumor · VIP- secreting tumour · VIP-producing NET (+6 more)

Data availability: 1 cell line.

Disease family

This is a subtype of pancreatic neuroendocrine tumor. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › digestive system disorder › digestive system neuroendocrine neoplasm › digestive system neuroendocrine tumor, grade 1/2 › pancreatic neuroendocrine tumor › VIPoma

Related subtypes (10): pancreatic delta cell neuroendocrine tumor, pancreatic gastrin-producing neuroendocrine tumor, non-functional pancreatic neuroendocrine tumor, pancreatic insulin-producing neuroendocrine tumor, somatostatinoma, GRFoma, PPoma, glucagonoma, pancreatic neuroendocrine tumor G1, functional pancreatic neuroendocrine tumor

Subtypes (2): small intestinal vasoactive intestinal peptide producing tumor, pancreatic vasoactive intestinal peptide producing tumor

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 5.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	2
PHASE1	2
Not specified	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT01229943	PHASE2	COMPLETED	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
NCT04915144	PHASE2	WITHDRAWN	177Lu-DOTATOC for the Treatment of Patients With Somatostatin Receptor Positive NETs
NCT02831179	PHASE1	WITHDRAWN	Veliparib, Capecitabine, and Temozolomide in Patients With Advanced, Metastatic, and Recurrent Neuroendocrine Tumor
NCT03147768	PHASE1	COMPLETED	Laser Tissue Welding - Distal Pancreatectomy Sealing Study
NCT03583528	Not specified	ACTIVE_NOT_RECRUITING	DOTATOC PET/CT for Imaging NET Patients

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
BEVACIZUMAB	4	1
EDOTREOTIDE GALLIUM GA-68	4	1
OCTREOTIDE ACETATE	4	1
VELIPARIB	3	2
LUTETIUM LU177 EDOTREOTIDE	2	1