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somatostatinoma - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

somatostatinoma

MONDO:0006976

Also known as:Delta cell tumorDelta cell tumoursomatostatin cell neoplasm

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Overview

A rare, usually malignant neuroendocrine tumor arizing from delta cells. This neoplasm produces large amounts of somatostatin, which may result in a syndrome characterized by diarrhea, steatorrhea, weight loss, and gastric hyposecretion. Sixty percent are found in the pancreas and 40% in the duodenum or jejunum. The peak incidence occurs between 40 and 60 years of age; women are affected more than men by 2:1.

Clinical Trials2 registered · 1 recruiting

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include common findings: Diabetes mellitus, Weight loss, Diarrhea, and Nausea and vomiting and others; and sometimes findings: Intermittent jaundice, Intrahepatic cholestasis, Abnormal abdomen morphology, and Ascites and others. 35 total HPO annotations.

Organ System	Phenotype Count	Example Features
Digestive system	13	Diarrhea, Nausea and vomiting, Constipation

Diagnosis

Biomarker and diagnostic research for somatostatinoma has been reported in the published literature.

Prognosis

Phenotype severity distribution: 15 common features.

Estimated prevalence: <1 in 1,000,000 (VERY_RARE).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

2 clinical trials registered, 1 recruiting. Interventions under study include drug therapy and other interventions. Pipeline includes 1 PHASE2. Research is primarily sponsored by academic and government institutions.

214 publications have been identified in PubMed for somatostatinoma. Kisho has analyzed 93 by research type. Research spans Basic Science / Preclinical (51%), Review / Meta-Analysis (25%), and Gene Therapy / Novel Therapeutics (12%).

Research Type	Count	% of Total
Laboratory research	47

Data assembled from 5 of 12 sources · Last updated Sep 18, 2026, 7:51 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:97283

GARD

Genetic and Rare Diseases Info Center

GARD:4900

Questions for Your Care Team

→Are any of the 2 active clinical trials a good fit for my situation?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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somatostatinoma

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

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Questions for Your Care Team

Explore clinical features

Recent Publications

somatostatinoma

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications