Kisho

Kisho is an information platform, not a medical provider. Nothing on this site constitutes medical advice, diagnosis, or treatment recommendations. All content is aggregated from publicly available sources (including ClinicalTrials.gov, PubMed, FDA.gov, and Orphanet) and is provided for informational purposes only. Clinical trial eligibility, treatment decisions, and any health-related actions should always be discussed with a qualified healthcare professional. Kisho does not endorse any specific therapy, organization, or clinical trial. Terms of use · Privacy policy

desmoplastic small round cell tumor (DSRCT) - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

desmoplastic small round cell tumor

MONDO:0019373

Also known as:DSRCTDesmoplas. small round cell tumorDesmoplas. small round cell tumour

Find Clinical TrialsTrials

Overview

Desmoplastic small round cell tumor (DSRCT) is an aggressive soft tissue cancer that typically arises in serous lined surfaces of the abdominal or pelvic peritoneum, and spreads to the omentum, lymph nodes and hematogenously disseminates especially to the liver. Extraserous primary location has been reported in exceptional cases.

Clinical Trials19 registered · 12 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 very common findings: Abdominal pain, Abnormal peritoneum morphology, Lymphadenopathy, and Abdominal distention and others; and common findings: Weight loss, Nausea and vomiting, Enlarged liver (hepatomegaly), and Ileus and others. 18 total HPO annotations.

Organ System	Phenotype Count	Example Features
Digestive system	5	Ascites, Nausea and vomiting, Abdominal pain

Diagnosis

Biomarker and diagnostic research for desmoplastic small round cell tumor has been reported in the published literature.

Prognosis

Phenotype severity distribution: 5 very common features, 5 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.

19 clinical trials registered, 12 recruiting. Interventions under study include drug therapy, biologic therapy, other interventions, and procedural interventions. Pipeline includes 7 PHASE2, 9 PHASE1, 1 EARLY_PHASE1. Research is sponsored by a mix of industry and academic institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT04690374](https://clinicaltrials.gov/study/NCT04690374)

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 1:05 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:83469

GARD

Genetic and Rare Diseases Info Center

GARD:6265

Questions for Your Care Team

→Are any of the 19 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?

← Back to All Diseases

Research Type	Count	% of Total
Patient case studies	19	43%
Research summaries	10	23%
Testing and diagnosis research	5	11%
Laboratory research	4	9%
Clinical study results	2	5%
New treatment approaches	2	5%
Other research	1	2%
Disease patterns and progression	1	2%

Kisho

desmoplastic small round cell tumor

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications

Latest News & Developments

Orbital Proptosis as the Sentinel Sign of Metastatic Desmoplastic Small Round Cell Tumor: A Rare Ophthalmic Gateway to Systemic Malignancy.

Aggressive Desmoplastic Small Round Cell Tumor in an Adult: A Case Report and Literature Review.

Multi-layered molecular profiling informs the diagnosis and targeted therapy of desmoplastic small round cell tumor | Nature Communications

Desmoplastic Small Round Blue Cell Tumor in a Young Adult Man: A Rare Case of Abdominal Sarcoma.

desmoplastic small round cell tumor

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

Latest News & Developments

Orbital Proptosis as the Sentinel Sign of Metastatic Desmoplastic Small Round Cell Tumor: A Rare Ophthalmic Gateway to Systemic Malignancy.

Aggressive Desmoplastic Small Round Cell Tumor in an Adult: A Case Report and Literature Review.

Multi-layered molecular profiling informs the diagnosis and targeted therapy of desmoplastic small round cell tumor | Nature Communications

Desmoplastic Small Round Blue Cell Tumor in a Young Adult Man: A Rare Case of Abdominal Sarcoma.