Somatostatinoma
diseaseOn this page
Also known as ampullary somatostatinomacarcinoid somatostatinomaDelta cell tumorDelta cell tumourmalignant islet cell tumormalignant islet cell tumourSomatomedin-secreting carcinoidsomatostatin cell tumorsomatostatin producing tumorsomatostatin producing tumoursomatostatin-producing NETsomatostatin-producing neuroendocrine tumorsomatostatin-producing neuroendocrine tumoursomatostatin-producing tumorsomatostatin-producing tumoursomatostatin-secreting pancreatic neoplasmtumor of Delta cellstumor of the Delta cellstumour of Delta cells
Summary
Somatostatinoma (MONDO:0006976) is a disease and 15 clinical trials. Top therapeutic interventions include edotreotide gallium ga-68, gefitinib, and indium in 111 pentetreotide. A subtype of carcinoid 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): 35
- Clinical trials: 15
Clinical features
Epidemiology
Prevalence records
2 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.0025 | Europe | Validated |
| Point prevalence | <1 / 1 000 000 | Europe | Validated |
Signs & symptoms
Clinical features (HPO)
35 HPO clinical features (Orphanet curated; top 35 by frequency):
| HPO ID | Term | Frequency |
|---|---|---|
| HP:0000819 | Diabetes mellitus | Frequent (30-79%) |
| HP:0001824 | Weight loss | Frequent (30-79%) |
| HP:0002014 | Diarrhea | Frequent (30-79%) |
| HP:0002017 | Nausea and vomiting | Frequent (30-79%) |
| HP:0002019 | Constipation | Frequent (30-79%) |
| HP:0002039 | Anorexia | Frequent (30-79%) |
| HP:0002240 | Hepatomegaly | Frequent (30-79%) |
| HP:0002570 | Steatorrhea | Frequent (30-79%) |
| HP:0002574 | Episodic abdominal pain | Frequent (30-79%) |
| HP:0002894 | Neoplasm of the pancreas | Frequent (30-79%) |
| HP:0004396 | Poor appetite | Frequent (30-79%) |
| HP:0004840 | Hypochromic microcytic anemia | Frequent (30-79%) |
| HP:0005609 | Gallbladder dysfunction | Frequent (30-79%) |
| HP:0012432 | Chronic fatigue | Frequent (30-79%) |
| HP:0100833 | Neoplasm of the small intestine | 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:0002239 | Gastrointestinal hemorrhage | Occasional (5-29%) |
| HP:0005214 | Intestinal obstruction | Occasional (5-29%) |
| HP:0012334 | Extrahepatic cholestasis | Occasional (5-29%) |
| HP:0030145 | Lack of bowel sounds | Occasional (5-29%) |
| 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:0002865 | Medullary thyroid carcinoma | Very rare (<1-4%) |
| HP:0002893 | Pituitary adenoma | Very rare (<1-4%) |
| HP:0002897 | Parathyroid adenoma | Very rare (<1-4%) |
| HP:0003072 | Hypercalcemia | Very rare (<1-4%) |
| HP:0003118 | Increased circulating cortisol level | 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 | somatostatinoma |
| Mondo ID | MONDO:0006976 |
| EFO | EFO:1001187 |
| MeSH | D013005 |
| Orphanet | 97283 |
| DOID | DOID:4430 |
| ICD-11 | 219879696 |
| NCIT | C3379 |
| SNOMED CT | 253006001 |
| UMLS | C0037661 |
| MedGen | 52416 |
| GARD | 0004900 |
| MedDRA | 10041329 |
| Is cancer (heuristic) | no |
Also known as: ampullary somatostatinoma · carcinoid somatostatinoma · Delta cell tumor · Delta cell tumour · malignant islet cell tumor · malignant islet cell tumour · Somatomedin-secreting carcinoid · somatostatin cell tumor · somatostatin producing tumor · somatostatin producing tumour · somatostatin-producing NET · somatostatin-producing neuroendocrine tumor · somatostatin-producing neuroendocrine tumour · somatostatin-producing tumor · somatostatin-producing tumour · somatostatin-secreting pancreatic neoplasm · somatostatinoma · tumor of Delta cells · tumor of the Delta cells · tumour of Delta cells (+1 more)
Data availability: 1 cell line.
Disease family
This is a subtype of carcinoid 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 etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › endocrine gland neoplasm › neuroendocrine neoplasm › carcinoid tumor › somatostatinoma
Related subtypes (6): lung carcinoid tumor, atypical carcinoid tumor, gastric neuroendocrine tumor G1, intestinal neuroendocrine tumor G1, pancreatic neuroendocrine tumor G1, childhood carcinoid 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
Drugs indicated for this disease
No approved or late-stage (phase ≥3) drug is indicated for this disease; the following are in earlier-phase trials only.
Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Bevacizumab, Temsirolimus.
Clinical trials & evidence
Clinical trials
Clinical trials: 15.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2 | 11 |
| PHASE1 | 4 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT01824875 | PHASE2 | ACTIVE_NOT_RECRUITING | Temozolomide With or Without Capecitabine in Treating Patients With Advanced Pancreatic Neuroendocrine Tumors |
| NCT00075439 | PHASE2 | COMPLETED | Gefitinib in Treating Patients With Progressive Metastatic Neuroendocrine Tumors |
| NCT00084461 | PHASE2 | TERMINATED | Romidepsin in Treating Patients With Locally Advanced or Metastatic Neuroendocrine Tumors |
| NCT00131911 | PHASE2 | COMPLETED | Sorafenib Tosylate in Treating Patients With Progressive Metastatic Neuroendocrine Tumors |
| NCT00454363 | PHASE2 | COMPLETED | Pazopanib Hydrochloride in Treating Patients With Advanced Neuroendocrine Cancer |
| NCT01525082 | PHASE2 | COMPLETED | Capecitabine, Temozolomide, and Bevacizumab for Metastatic or Unresectable Pancreatic Neuroendocrine Tumors |
| NCT01869725 | PHASE2 | COMPLETED | Comparative Study of Sensitivity of Ga-DOTATOC PET vs Octreoscan SPECT + CT |
| NCT02031536 | PHASE2 | TERMINATED | Everolimus in Patients With Pancreatic Neuroendocrine Tumors Metastatic to the Liver Previously Treated With Surgery |
| NCT02108782 | PHASE2 | WITHDRAWN | Dovitinib Lactate in Treating Patients With Pancreatic Neuroendocrine Tumors |
| NCT02259725 | PHASE2 | COMPLETED | Regorafenib in Treating Patients With Advanced or Metastatic Neuroendocrine Tumors |
| NCT02273752 | PHASE2 | TERMINATED | Pharmacokinetically Guided Everolimus in Patients With Breast Cancer, Pancreatic Neuroendocrine Tumors, or Kidney Cancer |
| NCT00004074 | PHASE1 | COMPLETED | Interleukin-12 and Trastuzumab in Treating Patients With Cancer That Has High Levels of HER2/Neu |
| NCT00655655 | PHASE1 | COMPLETED | Everolimus and Vatalanib in Treating Patients With Advanced Solid Tumors |
| NCT01204476 | PHASE1 | COMPLETED | Cixutumumab, Everolimus, and Octreotide Acetate in Treating Patients With Advanced Low to Intermediate Grade Neuroendocrine Carcinoma |
| NCT02831179 | PHASE1 | WITHDRAWN | Veliparib, Capecitabine, and Temozolomide in Patients With Advanced, Metastatic, and Recurrent Neuroendocrine Tumor |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| EDOTREOTIDE GALLIUM GA-68 | 4 | 1 |
| GEFITINIB | 4 | 1 |
| INDIUM IN 111 PENTETREOTIDE | 4 | 1 |
| OCTREOTIDE ACETATE | 4 | 1 |
| PAZOPANIB HYDROCHLORIDE | 4 | 1 |
| REGORAFENIB | 4 | 1 |
| ROMIDEPSIN | 4 | 1 |
| SORAFENIB | 4 | 1 |
| VATALANIB | 3 | 2 |
| VELIPARIB | 3 | 2 |
| CIXUTUMUMAB | 2 | 1 |
| DOVITINIB LACTATE | 2 | 1 |
| EDODEKIN ALFA | 2 | 1 |
| CHEMBL4066465 | 0 | 1 |