ORPHA:97283
Somatostatinoma
Publications
1,559
Trials
16
Interventional, condition-specific
Researchers
1,123
Distinct authors in sample
Gene link
—
Readiness
3/6
Stages with a signal
Clinical definition (Orphanet)
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.
How rare: <1 / 1 000 000 — fewer than one in a million. In a city the size of Kolkata, that might mean on the order of fifteen people.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0006976
- MeSH:D013005
- UMLS:C0037661
- NCIT:C3379
Additional Mondo synonyms (15)
Delta cell tumor · Delta cell tumour · 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 · somatostatinoma · tumor of Delta cells · tumor of the Delta cells · tumour of Delta cells · tumour of the Delta cells
Research stages
Trial readiness signals
Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”
3/6 stages with a signal
An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
1,559 matched papers (718 in last 10 years) Source
- Phenotype characterisedPresent
35 HPO annotations (e.g. Constipation; Anorexia; Poor appetite) Source
- Animal modelNot found
No Alliance genotype “model of” associations via Monarch for these Mondo IDs
- Orphan designationNot found
No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source
- Interventional trialPresent
16 matched on ClinicalTrials.gov
Biology
Genes and phenotypes
Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.
Do we know what causes it?
Not yet — the cause hasn't been pinned down in GenCC.
No strong gene–disease assertion joined for this Orphanet entity.
Phenotypes (Monarch / HPO)
35
Associated phenotypes · MONDO:0006976
- Constipation
- Anorexia
- Poor appetite
- Neoplasm of the small intestine
- Abnormal abdomen morphology
Showing 5 of 35 — open Monarch for the full list.
Animal models (Monarch / Alliance)
None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.
Monarch fetch 2026-07-29
Therapies
Designations, candidates, and chemicals
FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.
Orphan designation (FDA · EMA)
No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.
Open Targets candidates
No drugs or clinical candidates returned for this Mondo ID on Open Targets.
CTD chemicals (MyDisease.info)
No CTD chemical associations returned for this Mondo ID.
Literature
Is anyone studying this?
1,559
1,559 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.
1,559 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).
718 in the last 10 years · low confidence
Phrase hits: 1,559 · MeSH hits: 45
Who's working on it?
1,123
Distinct author names in 200 sampled papers — named people below.
Who's working on it?
People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.
- 01Pacak K9 papers · 2026
Section on Medical Neuroendocrinology, Eunice Kennedy Shriver, National Institute of Child Health and Human Development, NIH, Bethesda, Maryland, USA.
Papers in Europe PMC - 02Zhuang Z8 papers · 2026
Neuro-Oncology Branch, National Cancer Institute, NIH, Bethesda, Maryland, USA.
Papers in Europe PMC - 03Bala MM5 papers · 2024
Chair of Epidemiology and Preventive Medicine; Department of Hygiene and Dietetics; Systematic Reviews Unit, Jagiellonian University Medical College, Kopernika 7, Krakow, Poland, 31-034.
Papers in Europe PMC - 04Mitus JW5 papers · 2024
The Maria Sklodowska-Curie Institute, Oncology Center, Krakow Branch; Department of Anatomy, Jagiellonian University Medical College, Department of Surgical Oncology, ul. Garncarska 11, Krakow, Poland, 31-115.
Papers in Europe PMC - 05Pedziwiatr M5 papers · 2024
Jagiellonian University Medical College, 2nd Department of General Surgery, Jakubowskiego Street 2, Krakow, Malopolska, Poland, 30-688.
Papers in Europe PMC - 06Rosenblum JS5 papers · 2025
Neuro-Oncology Branch, National Cancer Institute, NIH, Bethesda, Maryland, USA.
Papers in Europe PMC - 07Storman D5 papers · 2024
Jagiellonian University Medical College, Chair of Epidemiology and Preventive Medicine, Department of Hygiene and Dietetics; Systematic Reviews Unit, Krakow, Poland.
Papers in Europe PMC - 08Swierz MJ5 papers · 2024
Jagiellonian University Medical College, Chair of Epidemiology and Preventive Medicine, Department of Hygiene and Dietetics; Systematic Reviews Unit, Krakow, Poland.
Papers in Europe PMC - 09Wolff R5 papers · 2024
Kleijnen Systematic Reviews Ltd, Unit 6, Escrick Business Park, Riccall Road, Escrick, York, UK, YO19 6FD.
Papers in Europe PMC - 10Chen Y4 papers · 2026
1st Department of General Surgery, The First Affiliated Hospital of Dali University, Dali, Yunnan, China.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
16
interventional trials for this specific condition
16 interventional trials matched this specific condition name; none in our sample are currently recruiting.
Data as of 11 September 2026
16 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 94.2th percentile).
low confidence · 94.2th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
16 interventional trials matched after quoted-phrase search and title/condition post-filter.
No currently recruiting studies in the matched set. Open the same search on ClinicalTrials.gov.
Other registries (secondary)
Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.
raw 4 · after dedupe 4 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 4 · dropped 0 · fetched 2026-07-30
Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri
No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).
Uncertain / not reviewed (4)
- isrctn·ISRCTN26113637·Stopped·ImmuniCell® in patients with advanced cancers
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN95053737·No longer recruiting·A prospective randomized study of adjuvant chemotherapy with navelbine and cisplatin in completely resected non small cell lung cancer
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN91655550·No longer recruiting·Introducing a non-endoscopic diagnostic test into the clinical pathway to identify high-risk patients with Barrett's oesophagus
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN37909269·No longer recruiting·Understanding the mechanism of the acute phase response following intravenous (IV) bisphosphonates and its prevention: a study of the effects of zoledronic acid and co-prescription with fluvastatin or placebo
skipped — LLM skipped (--skip-llm)
Where to find support
Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.
Orphanet entry for Somatostatinoma — check Associations / patient organisations on that page.
India — NPRD
Last verified 2026-07-26This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.
Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.
How we counted this
Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.
"Somatostatinoma" OR "Delta cell tumor" OR "Delta cell tumour" OR "somatostatin cell tumor" OR "somatostatin producing tumor" OR "somatostatin producing tumour" OR "somatostatin-producing NET" OR "somatostatin-producing neuroendocrine tumor" OR "somatostatin-producing neuroendocrine tumour" OR "somatostatin-producing tumor" OR "somatostatin-producing tumour" OR "tumor of Delta cells" OR "tumor of the Delta cells" OR "tumour of Delta cells" OR "tumour of the Delta cells"
MeSH descriptor terms unioned into the query: Somatostatinoma
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Somatostatinoma" OR "Delta cell tumor" OR "Delta cell tumour" OR "somatostatin cell tumor" OR "somatostatin producing tumor" OR "somatostatin producing tumour" OR "somatostatin-producing NET" OR "somatostatin-producing neuroendocrine tumor" OR "somatostatin-producing neuroendocrine tumour" OR "somatostatin-producing tumor" OR "somatostatin-producing tumour" OR "tumor of Delta cells" OR "tumor of the Delta cells" OR "tumour of Delta cells" OR "tumour of the Delta cells"
Interventional trials matched via: both, phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).
Study-type breakdown: 16 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.
Query health: ok — strategies attempted: phrase, mesh; with hits: phrase, mesh
Run this search on ClinicalTrials.gov
Confidence reasoning
- Preferred label is short or not clearly distinctive
- No synonyms dropped by stoplist
- No label/synonym collisions with other diseases in this corpus
- Publication count (1559) is implausibly high for prevalence class "<1 / 1 000 000" — treat as possible over-matching, not a measure of research intensity
Ingested 2026-07-27T05:05:50.203Z