Somatostatinoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- Adult, Elderly
- Status
- active
Somatostatinoma (SSoma) is an extremely rare pancreatic neuroendocrine tumor or duodenal endocrine tumor that originates either in the pancreas (50%) or the gastrointestinal tract (50%) and mainly presents with non-specific symptoms of abdominal pain, weight loss, jaundice and diarrhea but, in approximately 20% of pancreatic cases, leads to a somatostatin hypersecretion syndrome (somatostatinoma syndrome) characterized by diabetes mellitus, cholelithiasis, steatorrhea and hypochlorhydria.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Somatostatinoma | |
| fr | Somatostatinome | |
| de | Somatostatinom | |
| es | Somatostatinoma | |
| it | Somatostatinoma | |
| nl | Somatostatinoom | |
| pt | Somatostatinoma | |
| pl | Somatostatinoma | |
| cs | Somatostatinom | |
| uk | Соматостатинома | |
| zh | 生长抑素瘤 | |
| tr | Somatostatinom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C17.0 | NTBT | attributed | validated |
| ICD-11 | 2C10.1 | NTBT | index | validated |
| MONDO | 6976 | E | validated | |
| MeSH | D013005 | E | validated | |
| UMLS | C0037661 | E | validated | |
| GARD | 4900 | E | validated | |
| MedDRA | 10041329 | 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 (3):
- ORPHA:100076Duodenal neuroendocrine tumor
rare gastroenterological diseases; rare endocrine diseases; rare neoplastic diseases; rare transplant-related disorders - ORPHA:506060Functioning neuroendocrine tumor of pancreas
rare gastroenterological diseases; rare endocrine diseases; rare neoplastic diseases - ORPHA:100077Jejunal neuroendocrine tumor
rare gastroenterological diseases; rare endocrine diseases; rare neoplastic diseases; rare transplant-related disorders
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 | 0.0025 / 100 000 | Europe | Value and class | Validated | [EXPERT]_17645474[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
35 annotations · validated 2017-02-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0002039 | Anorexia | |
| HP:0012432 | Chronic fatigue | |
| HP:0002019 | Constipation | |
| HP:0000819 | Diabetes mellitus | |
| HP:0002014 | Diarrhea | |
| HP:0002574 | Episodic abdominal pain | |
| HP:0005609 | Gallbladder dysfunction | |
| HP:0002240 | Hepatomegaly | |
| HP:0004840 | Hypochromic microcytic anemia | |
| HP:0002017 | Nausea and vomiting | |
| HP:0002894 | Neoplasm of the pancreas | |
| HP:0100833 | Neoplasm of the small intestine | |
| HP:0004396 | Poor appetite | |
| HP:0002570 | Steatorrhea | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0001438 | Abnormality of abdomen morphology | |
| HP:0001541 | Ascites | |
| 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 | |
| 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:0002865 | Medullary thyroid carcinoma | |
| HP:0002897 | Parathyroid adenoma | |
| HP:0002893 | Pituitary adenoma | |
| HP:0008200 | Primary hyperparathyroidism | |
| HP:0001031 | Subcutaneous lipoma | |
Annotation source: 26742109[PMID]_11340706[PMID]_17645474[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/97283
- This record as JSON: /data/d/97283.json