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Desmoplastic Small Round Cell Tumor · CancerIndex

Cancers
Neoplasm
Neoplasm by Special Category
Malignant Neoplasm
Sarcoma
Soft Tissue Sarcoma
Desmoplastic Small Round Cell Tumor

Subtype

Desmoplastic Small Round Cell Tumor

CI-CAN-00002515DSRCTExplore in graph →

16 active trials3 evidence items

NCIt: C8300
OncoTree: DSRCT
DOID: 6785
UMLS CUI: C0281508
MeSH: D058405

Overview
Statistics
Survival
Genomics
Variants & evidence3
Drugs
Trials41
Research
Rankings
Sources & provenance

Data completeness3 / 7 dimensions

Epidemiology (no data yet)
Survival (no data yet)
Trials (data present)
Literature (data present)
Genomics (no data yet)
Evidence (data present)
Therapies (no data yet)

Computed from entity_counters.completeness · refreshed after each connector run

Definition

What this entity is

An aggressive malignant soft tissue neoplasm of uncertain differentiation. It is characterized by a recurrent chromosomal translocation t(11;22)(p13;q12) and the presence of small round cells in a desmoplastic stroma. It usually affects children and young adults. The most common site of involvement is the abdomen. Patients usually present with abdominal distention, pain, ascites, and a palpable abdominal mass. The prognosis is usually poor.

Definition text as published by ncit-evsProvenance

Source: NCI Thesaurus (NCIt)
Dataset: NCI Thesaurus
Version: 26.08e
Retrieved: Sep 8, 2026
Layer: raw (as published)
Evidence: expert curation
License: CC BY 4.0
Run: ING-NCITEVS-20260908-000001

Open at source Curated

Data updated 22 days agoSource updated 26.08e

Taxonomy

Position in the hierarchies

Several hierarchies coexist; edges are listed per hierarchy type with their source.

Parents

ncit

Malignant Soft Tissue Neoplasm of Uncertain DifferentiationSubtype
Small Cell SarcomaSubtype
Soft Tissue SarcomaSubtype

Children (4)

ncit

Adult Desmoplastic Small Round Cell Tumor
Childhood Desmoplastic Small Round Cell Tumor
Lung Desmoplastic Small Round Cell Tumor
Peritoneal Desmoplastic Small Round Cell Tumor

Anatomy

Anatomical sites

Connective and Soft TissueC3810
Soft TissueC12471

Record

Identity

CancerIndex ID: CI-CAN-00002515
Entity type: Subtype
Malignant: Yes
Solid / hematologic: Solid tumor
Pediatric relevant: Not flagged
Rare cancer: Unknown (no incidence data yet)
Hierarchy depth: 5
Classification version: 26.08e
Semantic types: Neoplastic Process
Status: Active
Record updated: 2026-09-08

Coverage

Data completeness

Trials (all / active): 41 / 16

Publications (all / 5y)

296 / 95

Evidence items

3

Genes / variants

1 / 1

Drugs (any / approved)

0 / 0

Genomic cohorts

0

Epidemiology / survival obs.

0 / 0

Data updated 18 hours agoSource updated unknowncounters aggregate over descendants

Names

Synonyms (6)

Desmoplastic Small Round Cell Tumormesh_heading · MeSH
Desmoplas. small round cell tumorsynonym · NCIt
Desmoplastic Small Cell Tumorsynonym · MeSH
Desmoplastic Small Round-Cell Neoplasmsynonym · NCIt
Desmoplastic Small-Round-Cell Tumorsynonym · OncoTree
Polyphenotypic Small Round Cell Tumorsynonym · NCIt

CancerIndex is a research and information platform. It does not diagnose and does not recommend treatment.

Key figures

At a glance

Each figure carries its unit, period and source; ranks link to "Why this rank?".

No registry-level ancestor: burden figures are published for the mutually exclusive top-level site groups only, and this entity does not descend from one. Global figures (IARC / GLOBOCAN) stay under license review and SEER awaits credentials.

Active trials16countcurrent registry status · entity + descendantsclinicaltrialsComputedrank #484 of 1,654 (World · both sexes · all ages · latest · all malignant entities)
Recruiting trials11countstatus RECRUITING · entity + descendantsclinicaltrialsComputedrank #433 of 1,277 (World · both sexes · all ages · latest · all malignant entities)
Curated evidence items3countaccepted CIViC items · entity + descendantscivicComputedrank #300 of 492 (World · both sexes · all ages · latest · all malignant entities)
Publications, last 12 months12countPubMed records · stored query · 12-month windowpubmedComputedrank #914 of 2,294 (World · both sexes · all ages · latest · all malignant entities)
Genomic cohorts0countopen GDC projects · entity + descendantsgdcComputednot in a current ranking snapshot

Data updated 18 hours agoSource updated unknownregistry figures: none · counters aggregate over descendants