Glucagonoma
Synonyms: Glucagonoma syndrome
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Glucagonoma is a rare, functioning type of pancreatic neuroendocrine tumor (PNET) that hypersecretes glucagon, leading to a syndrome comprised of necrolytic migratory erythema, diabetes mellitus, anemia, weight loss, mucosal abnormalities, thromboembolism, gastrointestinal and neuropsychiatric symptoms.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Glucagonoma | Glucagonoma syndrome |
| fr | Glucagonome | Syndrome du glucagonome |
| de | Glucagonom | Glucagonom-Syndrom |
| es | Glucagonoma | Síndrome de glucagonoma |
| it | Glucagonoma | Sindrome da glucagonoma |
| nl | Glucagonoom | Glucagonoomsyndroom |
| pt | Glucagonoma | |
| pl | Glukagonoma | Zespół glucagonoma |
| cs | Glukagonom | Glukagonový syndrom |
| uk | Глюкагонома | Синдром глюкагономи |
| zh | 胰高血糖素瘤 | 胰高血糖素瘤综合征 |
| tr | Glukagonom | Glukagonom 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 |
| MONDO | 0019959 | E | validated | |
| MeSH | D005935 | E | validated | |
| UMLS | C0017689 | E | validated | |
| GARD | 2496 | E | validated | |
| MedDRA | 10018404 | 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 | 0.005 / 100 000 | Europe | Value and class | Validated | 9113318[PMID] |
| 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
42 annotations · validated 2017-02-06| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0002894 | Neoplasm of the pancreas | |
| Frequent (79-30%) | ||
| HP:0008066 | Abnormal blistering of the skin | |
| HP:0001927 | Acanthocytosis | |
| HP:0002039 | Anorexia | |
| HP:0012432 | Chronic fatigue | |
| HP:0002019 | Constipation | |
| HP:0000819 | Diabetes mellitus | |
| HP:0002014 | Diarrhea | |
| HP:0002574 | Episodic abdominal pain | |
| HP:0000206 | Glossitis | |
| HP:0002240 | Hepatomegaly | |
| HP:0002017 | Nausea and vomiting | |
| HP:0031181 | Necrolytic migratory erythema | |
| HP:0001895 | Normochromic anemia | |
| HP:0004396 | Poor appetite | |
| HP:0000989 | Pruritus | |
| HP:0000988 | Skin rash | |
| HP:0010280 | Stomatitis | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0030895 | Abnormal gastrointestinal motility | |
| HP:0001438 | Abnormality of abdomen morphology | |
| HP:0001541 | Ascites | |
| HP:0000716 | Depression | |
| HP:0012334 | Extrahepatic cholestasis | |
| HP:0002239 | Gastrointestinal hemorrhage | |
| HP:0001046 | Intermittent jaundice | |
| HP:0005214 | Intestinal obstruction | |
| HP:0001406 | Intrahepatic cholestasis | |
| HP:0030145 | Lack of bowel sounds | |
| HP:0002570 | Steatorrhea | |
| HP:0001907 | Thromboembolism | |
| Very rare (<4-1%) | ||
| HP:0000820 | Abnormality of the thyroid gland | |
| HP:0008256 | Adrenocortical adenoma | |
| HP:0000845 | Elevated circulating growth hormone concentration | |
| HP:0003072 | Hypercalcemia | |
| HP:0003118 | Increased circulating cortisol level | |
| HP:0000837 | Increased circulating gonadotropin level | |
| HP:0000870 | Increased circulating prolactin concentration | |
| HP:0002897 | Parathyroid adenoma | |
| HP:0002893 | Pituitary adenoma | |
| HP:0008200 | Primary hyperparathyroidism | |
| HP:0001031 | Subcutaneous lipoma | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 17122523[PMID]_16699310[PMID]_15538929[PMID]_4134714[PMID]_18957496[PMID]_21763589[PMID]_22997445[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/97280
- This record as JSON: /data/d/97280.json