VIPoma
Synonyms: Diarrheogenic islet cell tumor · Pancreatic cholera · VIP-secreting tumor · Verner-Morrison syndrome · WDHA syndrome · Watery diarrhea-hypokalemia-achlorhydria syndrome
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
VIPoma is an extremely rare type of pancreatic neuroendocrine tumor that secretes vasoactive intestinal polypeptide (VIP) leading to the manifestations of watery diarrhea, hypokalemia and achlorhydia or hypochhlorhydia (known as WDHA syndrome).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | VIPoma | Diarrheogenic islet cell tumor · Pancreatic cholera · VIP-secreting tumor · Verner-Morrison syndrome · WDHA syndrome · Watery diarrhea-hypokalemia-achlorhydria syndrome |
| fr | VIPome | Choléra endocrine · Choléra endocrinien · Choléra pancréatique · Syndrome WDHA · Syndrome de Verner-Morrison · Syndrome de diarrhée aqueuse avec hypokaliémie et achlorhydrie · Tumeur sécrétant du VIP |
| de | VIPom | Pankreatische Cholera · VIP-produzierender Tumor · Verner-Morrison-Syndrom · WDHA-Syndrom · Wässrige Durchfälle - Hypokaliämie - Achlorhydrie |
| es | VIPoma | Cólera pancreático · Diarrea acuosa-hipopotasemia-aclorhidria · Síndrome WDHA · Síndrome de Verner-Morrison · Tumor diarreogénico de las células de los islotes · Tumor secretor de VIP |
| it | VIPoma | Colera pancreatico · Diarrea acquosa - ipokaliemia - acloridria · Sindrome WDHA · Sindrome di Verner-Morrison · Tumore diarreogenico delle cellule degli isolotti · Tumore secernente VIP |
| nl | VIPoom | Diarreeveroorzakende tumor van de eilandjes van Langerhans · Pancreatische cholera · Syndroom van Verner-Morrison · VIP-secreterende tumor · WDHA-syndroom · Waterige diarree - hypokaliëmie - achloorhydrie-syndroom |
| pt | VIPoma | |
| pl | VIPoma | Cholera trzustkowa · Guz wydzielający VIP · Wodnista biegunka - hipokalemia - bezkwaśność · Zespół Vernera i Morrisona · Zespół WDHA |
| cs | VIPom | Pankreatická cholera · Nádor produkující VIP · WDHA syndrom · Diareogenní nádor ostrůvkových buněk · Vernerův-Morrisonův syndrom · Syndrom zahrnující vodnatý průjem, hypokalémii a achlorhydrii · Verner-Morrisonův syndrom |
| uk | VIPoma | Діареогенна острівцево-клітинна пухлина · Панкреатична холера · Синдром Вернера-Моррісона · VIP-секретуюча пухлина · Синдром водянистої діареї-гіпокаліємії-ахлоргідрії · Синдром WDHA |
| zh | 胰腺瘤 | 水样泻-低钾血症-胃酸缺乏综合征 |
| tr | VIPom | WDHA sendromu · Pankreatik kolera · VIP-salgılayan tümör · Verner-Morrison sendromu · Diyareojenik adacık hücre tümörü · Sulu diyare-hipokalemi-aklorhidri sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D37.7 | NTBT | attributed | validated |
| ICD-10 | E16.8 | ND | attributed | validated |
| ICD-11 | 2C10.1 | NTBT | index | validated |
| ICD-11 | XH72E5 | E | specific | validated |
| MONDO | 0019960 | E | validated | |
| MeSH | D003969 | E | validated | |
| UMLS | C0011993 | E | validated | |
| GARD | 3787 | E | validated | |
| MedDRA | 10047430 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:506060Functioning neuroendocrine tumor of pancreas
rare gastroenterological diseases; rare endocrine diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | — | Europe | Class only | Validated | [EXPERT] |
| Point prevalence | <1 / 1 000 000 | — | Europe | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
41 annotations · validated 2017-02-06| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0002900 | Hypokalemia | |
| HP:0002894 | Neoplasm of the pancreas | |
| HP:0005208 | Secretory diarrhea | |
| Frequent (79-30%) | ||
| HP:0002039 | Anorexia | |
| HP:0012432 | Chronic fatigue | |
| HP:0001944 | Dehydration | |
| HP:0000819 | Diabetes mellitus | |
| HP:0002574 | Episodic abdominal pain | |
| HP:0010783 | Erythema | |
| HP:0003324 | Generalized muscle weakness | |
| HP:0002240 | Hepatomegaly | |
| HP:0003072 | Hypercalcemia | |
| HP:0002024 | Malabsorption | |
| HP:0003394 | Muscle spasm | |
| HP:0002017 | Nausea and vomiting | |
| HP:0001895 | Normochromic anemia | |
| HP:0004396 | Poor appetite | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0030895 | Abnormal gastrointestinal motility | |
| HP:0001438 | Abnormality of abdomen morphology | |
| HP:0001541 | Ascites | |
| HP:0012334 | Extrahepatic cholestasis | |
| HP:0001046 | Intermittent jaundice | |
| HP:0001406 | Intrahepatic cholestasis | |
| Very rare (<4-1%) | ||
| HP:0000820 | Abnormality of the thyroid gland | |
| HP:0008256 | Adrenocortical adenoma | |
| HP:0006719 | Benign gastrointestinal tract tumors | |
| HP:0003528 | Elevated calcitonin | |
| HP:0000845 | Elevated circulating growth hormone concentration | |
| HP:0006731 | Follicular thyroid carcinoma | |
| HP:0003005 | Ganglioneuroma | |
| HP:0003118 | Increased circulating cortisol level | |
| HP:0000837 | Increased circulating gonadotropin level | |
| HP:0000870 | Increased circulating prolactin concentration | |
| HP:0002896 | Neoplasm of the liver | |
| HP:0002897 | Parathyroid adenoma | |
| HP:0002893 | Pituitary adenoma | |
| HP:0008200 | Primary hyperparathyroidism | |
| HP:0002747 | Respiratory insufficiency due to muscle weakness | |
| HP:0001031 | Subcutaneous lipoma | |
| Excluded (0%) | ||
| HP:0002573 | Hematochezia | |
Run the differential on the 3 obligate / very frequent terms
Annotation source: 13492596[PMID]_10834901[PMID]_15317894[PMID]_23993192[PMID]_22997445[PMID]_16416161[PMID]_22261872[PMID]_23582916[PMID]_18798544[PMID]_10940685[PMID]_2440390[PMID]_18941127[PMID]_22052063[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/97282
- This record as JSON: /data/d/97282.json