Desmoplastic small round cell tumor (DSRCT) is a highly aggressive mesenchymal malignancy characterized by the pathognomonic EWSR1-WT1 fusion gene resulting from t(11;22)(p13;q12). This fusion combines the transcriptional activation domain of EWSR1 with the DNA-binding zinc fingers of WT1, creating an aberrant transcription factor. DSRCT predominantly affects adolescent and young adult males, typically presenting with diffuse peritoneal involvement and bulky abdominal masses. Despite aggressive multimodal treatment, prognosis remains poor with 5-year survival rates under 20%. The unique biology of DSRCT reflects its hybrid phenotype with expression of epithelial, mesenchymal, and neural markers.
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name: Desmoplastic Small Round Cell Tumor
creation_date: '2026-01-26T02:55:13Z'
updated_date: '2026-04-11T21:17:25Z'
description: >-
Desmoplastic small round cell tumor (DSRCT) is a highly aggressive mesenchymal
malignancy characterized by the pathognomonic EWSR1-WT1 fusion gene resulting
from t(11;22)(p13;q12). This fusion combines the transcriptional activation
domain of EWSR1 with the DNA-binding zinc fingers of WT1, creating an aberrant
transcription factor. DSRCT predominantly affects adolescent and young adult
males, typically presenting with diffuse peritoneal involvement and bulky
abdominal masses. Despite aggressive multimodal treatment, prognosis remains
poor with 5-year survival rates under 20%. The unique biology of DSRCT reflects
its hybrid phenotype with expression of epithelial, mesenchymal, and neural
markers.
categories:
- Sarcoma
- Soft Tissue Sarcoma
- Rare Cancer
parents:
- small round cell tumor
has_subtypes:
- name: Intra-Abdominal DSRCT
description: >-
The classic presentation with multiple peritoneal implants studding the
abdominal cavity. Primary tumor may arise from the peritoneum, omentum,
or mesentery.
- name: Extra-Abdominal DSRCT
description: >-
Rare cases arising in other locations including paratesticular region,
ovary, pleura, or soft tissues. Shares the same EWSR1-WT1 fusion and
histologic features.
pathophysiology:
- name: EWSR1-WT1 Fusion Oncogene
description: >-
The t(11;22)(p13;q12) translocation fuses EWSR1 on chromosome 22 with WT1
on chromosome 11. The resulting fusion protein contains the potent
transcriptional activation domain of EWSR1 fused to the zinc finger
DNA-binding domain of WT1. This chimeric transcription factor activates
aberrant gene expression programs including PDGFA.
evidence:
- reference: PMID:20066421
reference_title: "[Cystic desmoplastic small round cell tumor: tumor development from cystic-\"mesothelioblastic\" areas?]."
supports: PARTIAL
snippet: "Using microdissection techniques, EWS-WT1-gene fusion transcripts were detected in the cystic (single-cell-layered), the papillary and the solid tumor proliferations (exon 7 of EWS on chromosome 22 with exon 8 of WT1 on chromosome 11)."
explanation: "Supports the presence of EWS-WT1 fusion transcripts in DSRCT."
biological_processes:
- preferred_term: positive regulation of transcription by RNA polymerase II
modifier: ABNORMAL
term:
id: GO:0045944
label: positive regulation of transcription by RNA polymerase II
locations:
- preferred_term: peritoneum
term:
id: UBERON:0002358
label: peritoneum
downstream:
- target: Multiphenotypic Differentiation
description: EWSR1-WT1 drives expression of epithelial, mesenchymal, and neural markers
- target: PDGFA Activation
description: EWSR1-WT1 directly activates PDGFA transcription
- name: PDGFA Activation
description: >-
EWSR1-WT1 directly activates transcription of PDGFA, creating an autocrine
growth loop that stimulates tumor cell proliferation and may contribute
to the characteristic desmoplastic stromal reaction.
biological_processes:
- preferred_term: cell population proliferation
modifier: INCREASED
term:
id: GO:0008283
label: cell population proliferation
- name: Multiphenotypic Differentiation
description: >-
DSRCT displays a unique multiphenotypic immunophenotype with co-expression
of epithelial (cytokeratin, EMA), mesenchymal (vimentin, desmin), and neural
(NSE) markers. This reflects the aberrant differentiation program driven
by EWSR1-WT1.
biological_processes:
- preferred_term: cell differentiation
modifier: ABNORMAL
term:
id: GO:0030154
label: cell differentiation
histopathology:
- name: Desmoplastic Small Round Cell Tumor
finding_term:
preferred_term: Desmoplastic Small Round Cell Tumor
term:
id: NCIT:C8300
label: Desmoplastic Small Round Cell Tumor
frequency: VERY_FREQUENT
description: Desmoplastic small round cell tumor is a rare and highly aggressive mesenchymal tumor.
evidence:
- reference: PMID:22550424
reference_title: "Desmoplastic small round cell tumor: current management and recent findings."
supports: SUPPORT
snippet: "Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive"
explanation: Abstract characterizes DSRCT as a rare and highly aggressive mesenchymal tumor.
phenotypes:
- category: Abdominal
name: Abdominal Mass
frequency: VERY_FREQUENT
diagnostic: true
description: >-
Bulky abdominal masses are the hallmark presentation. Tumors are typically
large and multiple at diagnosis, studding the peritoneal surfaces.
phenotype_term:
preferred_term: Abdominal mass
term:
id: HP:0031500
label: Abdominal mass
- category: Gastrointestinal
name: Ascites
frequency: FREQUENT
description: >-
Malignant ascites is common due to extensive peritoneal involvement.
phenotype_term:
preferred_term: Ascites
term:
id: HP:0001541
label: Ascites
- category: Constitutional
name: Weight Loss
frequency: FREQUENT
description: >-
Weight loss and cachexia are common with advanced disease burden.
phenotype_term:
preferred_term: Weight loss
term:
id: HP:0001824
label: Weight loss
- category: Constitutional
name: Fever
frequency: OCCASIONAL
description: >-
Fever may occur as a paraneoplastic symptom or with tumor necrosis.
phenotype_term:
preferred_term: Fever
term:
id: HP:0001945
label: Fever
- category: Systemic
name: Metastatic Disease
frequency: VERY_FREQUENT
description: >-
Metastases to liver, lung, and lymph nodes are common. Most patients
have disseminated disease at diagnosis given the diffuse peritoneal
involvement.
phenotype_term:
preferred_term: Neoplasm
term:
id: HP:0002664
label: Neoplasm
biochemical:
- name: EWSR1-WT1 Fusion Detection
notes: >-
RT-PCR or FISH detection of the EWSR1-WT1 fusion is diagnostic. The fusion
is virtually pathognomonic for DSRCT and not seen in other tumors.
- name: Multiphenotypic Markers
notes: >-
Immunohistochemistry shows characteristic co-expression of cytokeratin,
desmin (often with paranuclear dot pattern), and WT1 (C-terminus antibody
positive, which detects the fusion protein).
genetic:
- name: EWSR1-WT1 Fusion
association: Somatic Fusion Oncogene
notes: >-
The t(11;22)(p13;q12) translocation creates the EWSR1-WT1 fusion in
virtually all DSRCT cases. This fusion is both diagnostic and represents
the primary oncogenic driver.
treatments:
- name: Multi-Agent Chemotherapy
description: >-
Intensive chemotherapy regimens such as P6 protocol (cyclophosphamide,
doxorubicin, vincristine, ifosfamide, etoposide) are used. Response rates
are variable and usually partial.
treatment_term:
preferred_term: chemotherapy
term:
id: MAXO:0000647
label: chemotherapy
therapeutic_agent:
- preferred_term: cyclophosphamide
term:
id: CHEBI:4026
label: cyclophosphamide hydrate
- preferred_term: doxorubicin
term:
id: CHEBI:28748
label: doxorubicin
- preferred_term: vincristine
term:
id: CHEBI:28445
label: vincristine
- preferred_term: ifosfamide
term:
id: CHEBI:5864
label: ifosfamide
- preferred_term: etoposide
term:
id: CHEBI:4911
label: etoposide
- name: Cytoreductive Surgery
description: >-
Aggressive surgical debulking with hyperthermic intraperitoneal chemotherapy
(HIPEC) has shown benefit in selected patients. Complete cytoreduction is
associated with improved survival but is often not achievable.
treatment_term:
preferred_term: surgical procedure
term:
id: MAXO:0000004
label: surgical procedure
- name: Radiation Therapy
description: >-
Whole abdominal radiation or intensity-modulated radiation may improve
local control but is challenging given diffuse disease distribution.
treatment_term:
preferred_term: radiation therapy
term:
id: MAXO:0000014
label: radiation therapy
disease_term:
preferred_term: desmoplastic small round cell tumor
term:
id: MONDO:0019373
label: desmoplastic small round cell tumor
classifications:
icdo_morphology:
classification_value: Sarcoma
harrisons_chapter:
- classification_value: cancer
- classification_value: solid tumor
references:
- reference: DOI:10.1007/s00428-017-2207-y
title: 'Desmoplastic small round cell tumor: evaluation of reverse transcription-polymerase chain reaction and fluorescence in situ hybridization as ancillary molecular diagnostic techniques'
found_in:
- Desmoplastic_Small_Round_Cell_Tumor-deep-research-falcon.md
findings:
- statement: 'Desmoplastic small round cell tumor: evaluation of reverse transcription-polymerase chain reaction and fluorescence in situ hybridization as ancillary molecular diagnostic techniques'
supporting_text: 'Desmoplastic small round cell tumor: evaluation of reverse transcription-polymerase chain reaction and fluorescence in situ hybridization as ancillary molecular diagnostic techniques'
- reference: DOI:10.1038/s41467-024-51851-3
title: EWS-WT1 fusion isoforms establish oncogenic programs and therapeutic vulnerabilities in desmoplastic small round cell tumors
found_in:
- Desmoplastic_Small_Round_Cell_Tumor-deep-research-falcon.md
findings:
- statement: EWS-WT1 fusion isoforms establish oncogenic programs and therapeutic vulnerabilities in desmoplastic small round cell tumors
supporting_text: EWS-WT1 fusion isoforms establish oncogenic programs and therapeutic vulnerabilities in desmoplastic small round cell tumors
- reference: DOI:10.1101/2024.07.17.603708
title: Super-enhancer-driven <i>CACNA2D2</i> is an EWSR1::WT1 signature gene encoding a diagnostic marker for desmoplastic small round cell tumor (DSRCT)
found_in:
- Desmoplastic_Small_Round_Cell_Tumor-deep-research-falcon.md
findings:
- statement: Desmoplastic small round cell tumor (DSRCT) is a highly aggressive cancer predominantly occurring in male adolescents and young adults.
supporting_text: Desmoplastic small round cell tumor (DSRCT) is a highly aggressive cancer predominantly occurring in male adolescents and young adults.
evidence:
- reference: DOI:10.1101/2024.07.17.603708
reference_title: Super-enhancer-driven <i>CACNA2D2</i> is an EWSR1::WT1 signature gene encoding a diagnostic marker for desmoplastic small round cell tumor (DSRCT)
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: Desmoplastic small round cell tumor (DSRCT) is a highly aggressive cancer predominantly occurring in male adolescents and young adults.
explanation: Deep research cited this publication as relevant literature for Desmoplastic Small Round Cell Tumor.
- reference: DOI:10.1155/2024/5036102
title: 'Desmoplastic Small Round Cell Tumors: Clinical Presentation, Molecular Characterization, and Therapeutic Approach of Seven Patients'
found_in:
- Desmoplastic_Small_Round_Cell_Tumor-deep-research-falcon.md
findings:
- statement: Desmoplastic small round blue cell tumor (DSRCT) is a highly aggressive fatal sarcoma without evidence‐based therapeutic guidelines.
supporting_text: Desmoplastic small round blue cell tumor (DSRCT) is a highly aggressive fatal sarcoma without evidence‐based therapeutic guidelines.
evidence:
- reference: DOI:10.1155/2024/5036102
reference_title: 'Desmoplastic Small Round Cell Tumors: Clinical Presentation, Molecular Characterization, and Therapeutic Approach of Seven Patients'
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: Desmoplastic small round blue cell tumor (DSRCT) is a highly aggressive fatal sarcoma without evidence‐based therapeutic guidelines.
explanation: Deep research cited this publication as relevant literature for Desmoplastic Small Round Cell Tumor.
- reference: DOI:10.1158/0008-5472.can-23-3334
title: Comprehensive Transcriptomic Analysis of EWSR1::WT1 Targets Identifies CDK4/6 Inhibitors as an Effective Therapy for Desmoplastic Small Round Cell Tumors
found_in:
- Desmoplastic_Small_Round_Cell_Tumor-deep-research-falcon.md
findings:
- statement: Desmoplastic small round cell tumors (DSRCT) are a type of aggressive, pediatric sarcoma characterized by the EWSR1::WT1 fusion oncogene.
supporting_text: Desmoplastic small round cell tumors (DSRCT) are a type of aggressive, pediatric sarcoma characterized by the EWSR1::WT1 fusion oncogene.
evidence:
- reference: DOI:10.1158/0008-5472.can-23-3334
reference_title: Comprehensive Transcriptomic Analysis of EWSR1::WT1 Targets Identifies CDK4/6 Inhibitors as an Effective Therapy for Desmoplastic Small Round Cell Tumors
supports: SUPPORT
evidence_source: OTHER
snippet: Desmoplastic small round cell tumors (DSRCT) are a type of aggressive, pediatric sarcoma characterized by the EWSR1::WT1 fusion oncogene.
explanation: Deep research cited this publication as relevant literature for Desmoplastic Small Round Cell Tumor.
- reference: DOI:10.1186/s12885-025-15390-4
title: 'Management and survival outcomes of desmoplastic small round cell tumor: a retrospective cohort study from a tertiary cancer center'
found_in:
- Desmoplastic_Small_Round_Cell_Tumor-deep-research-falcon.md
findings:
- statement: 'Management and survival outcomes of desmoplastic small round cell tumor: a retrospective cohort study from a tertiary cancer center'
supporting_text: 'Management and survival outcomes of desmoplastic small round cell tumor: a retrospective cohort study from a tertiary cancer center'
- reference: DOI:10.1186/s12957-021-02310-6
title: 'Clinicopathological features of desmoplastic small round cell tumors: clinical series and literature review'
found_in:
- Desmoplastic_Small_Round_Cell_Tumor-deep-research-falcon.md
findings:
- statement: and purpose Desmoplastic small round cell tumor (DSRCT) is a highly malignant sarcoma that occurs in the abdominopelvic cavities of adolescents.
supporting_text: and purpose Desmoplastic small round cell tumor (DSRCT) is a highly malignant sarcoma that occurs in the abdominopelvic cavities of adolescents.
evidence:
- reference: DOI:10.1186/s12957-021-02310-6
reference_title: 'Clinicopathological features of desmoplastic small round cell tumors: clinical series and literature review'
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: and purpose Desmoplastic small round cell tumor (DSRCT) is a highly malignant sarcoma that occurs in the abdominopelvic cavities of adolescents.
explanation: Deep research cited this publication as relevant literature for Desmoplastic Small Round Cell Tumor.
- reference: DOI:10.1186/s13000-019-0825-1
title: Desmoplastic small round cell tumor of the parotid gland-report of a rare case and a review of the literature
found_in:
- Desmoplastic_Small_Round_Cell_Tumor-deep-research-falcon.md
findings:
- statement: Desmoplastic small round cell tumor of the parotid gland-report of a rare case and a review of the literature
supporting_text: Desmoplastic small round cell tumor of the parotid gland-report of a rare case and a review of the literature
- reference: DOI:10.1186/s43046-025-00276-0
title: 'Desmoplastic small round cell tumor: an update of current management practices'
found_in:
- Desmoplastic_Small_Round_Cell_Tumor-deep-research-falcon.md
findings:
- statement: Desmoplastic small round cell tumor (DSRCT) poses a diagnostic challenge, initiating with imaging techniques like ultrasound, CT, MRI, and PET scans, with CT being the primary choice for abdominal tumor visualization.
supporting_text: Desmoplastic small round cell tumor (DSRCT) poses a diagnostic challenge, initiating with imaging techniques like ultrasound, CT, MRI, and PET scans, with CT being the primary choice for abdominal tumor visualization.
evidence:
- reference: DOI:10.1186/s43046-025-00276-0
reference_title: 'Desmoplastic small round cell tumor: an update of current management practices'
supports: SUPPORT
evidence_source: OTHER
snippet: Desmoplastic small round cell tumor (DSRCT) poses a diagnostic challenge, initiating with imaging techniques like ultrasound, CT, MRI, and PET scans, with CT being the primary choice for abdominal tumor visualization.
explanation: Deep research cited this publication as relevant literature for Desmoplastic Small Round Cell Tumor.
- reference: DOI:10.21873/anticanres.15179
title: 'Desmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review'
found_in:
- Desmoplastic_Small_Round_Cell_Tumor-deep-research-falcon.md
findings:
- statement: 'Desmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review'
supporting_text: 'Desmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review'
- reference: DOI:10.3389/fcell.2024.1442488
title: 'Desmoplastic small round cell tumor: from genomics to targets, potential paths to future therapeutics'
found_in:
- Desmoplastic_Small_Round_Cell_Tumor-deep-research-falcon.md
findings:
- statement: Desmoplastic Small Round Cell Tumor (DSRCT) is a highly aggressive pediatric cancer caused by a reciprocal translocation between chromosomes 11 and 22, leading to the formation of the EWSR1::WT1 oncoprotein.
supporting_text: Desmoplastic Small Round Cell Tumor (DSRCT) is a highly aggressive pediatric cancer caused by a reciprocal translocation between chromosomes 11 and 22, leading to the formation of the EWSR1::WT1 oncoprotein.
evidence:
- reference: DOI:10.3389/fcell.2024.1442488
reference_title: 'Desmoplastic small round cell tumor: from genomics to targets, potential paths to future therapeutics'
supports: SUPPORT
evidence_source: OTHER
snippet: Desmoplastic Small Round Cell Tumor (DSRCT) is a highly aggressive pediatric cancer caused by a reciprocal translocation between chromosomes 11 and 22, leading to the formation of the EWSR1::WT1 oncoprotein.
explanation: Deep research cited this publication as relevant literature for Desmoplastic Small Round Cell Tumor.
- reference: DOI:10.3390/cancers16234101
title: Desmoplastic Small Round Cell Tumors of the Gastrointestinal Tract
found_in:
- Desmoplastic_Small_Round_Cell_Tumor-deep-research-falcon.md
findings:
- statement: Desmoplastic small round cell tumors (DSRCTs) of the gastrointestinal (GI) tract are a rare and highly aggressive variant of soft tissue sarcomas, predominantly affecting the abdominal region.
supporting_text: Desmoplastic small round cell tumors (DSRCTs) of the gastrointestinal (GI) tract are a rare and highly aggressive variant of soft tissue sarcomas, predominantly affecting the abdominal region.
evidence:
- reference: DOI:10.3390/cancers16234101
reference_title: Desmoplastic Small Round Cell Tumors of the Gastrointestinal Tract
supports: SUPPORT
evidence_source: OTHER
snippet: Desmoplastic small round cell tumors (DSRCTs) of the gastrointestinal (GI) tract are a rare and highly aggressive variant of soft tissue sarcomas, predominantly affecting the abdominal region.
explanation: Deep research cited this publication as relevant literature for Desmoplastic Small Round Cell Tumor.
- reference: DOI:10.3390/curroncol30040299
title: 'Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review'
found_in:
- Desmoplastic_Small_Round_Cell_Tumor-deep-research-falcon.md
findings:
- statement: Desmoplastic small round cell tumor is a very rare and highly aggressive soft tissue sarcoma, usually presenting with multiple intra-abdominal tumors in young males.
supporting_text: Desmoplastic small round cell tumor is a very rare and highly aggressive soft tissue sarcoma, usually presenting with multiple intra-abdominal tumors in young males.
evidence:
- reference: DOI:10.3390/curroncol30040299
reference_title: 'Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review'
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: Desmoplastic small round cell tumor is a very rare and highly aggressive soft tissue sarcoma, usually presenting with multiple intra-abdominal tumors in young males.
explanation: Deep research cited this publication as relevant literature for Desmoplastic Small Round Cell Tumor.
Question: You are an expert researcher providing comprehensive, well-cited information.
Provide detailed information focusing on: 1. Key concepts and definitions with current understanding 2. Recent developments and latest research (prioritize 2023-2024 sources) 3. Current applications and real-world implementations 4. Expert opinions and analysis from authoritative sources 5. Relevant statistics and data from recent studies
Format as a comprehensive research report with proper citations. Include URLs and publication dates where available. Always prioritize recent, authoritative sources and provide specific citations for all major claims.
Please provide a comprehensive research report on Desmoplastic Small Round Cell Tumor covering all of the disease characteristics listed below. This report will be used to populate a disease knowledge base entry. Be thorough and cite primary literature (PMID preferred) for all claims.
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For symptoms/signs: HPO, OMIM, Orphanet, PubMed For behavioral changes: HPO, DSM, RDoC (Research Domain Criteria), PubMed For laboratory abnormalities: LOINC, SNOMED CT, LabTests Online, PubMed - Phenotype characteristics: Search first: OMIM, Orphanet, HPO, PubMed - Age of symptom onset (neonatal, childhood, adult-onset, late-onset) - Symptom severity (mild, moderate, severe, variable) - Symptom progression (stable, progressive, episodic, fluctuating) - Frequency among affected individuals (percentage or qualitative) - Quality of life impact: Effects on daily functioning and well-being (per-phenotype when possible) Search first: EQ-5D database, SF-36, WHO QOL databases, PubMed - Suggest HPO (Human Phenotype Ontology) terms for each phenotype
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Structure your response as a comprehensive narrative organized by the sections above. For each section, provide: - Factual content with specific details (numbers, percentages, gene names, variant nomenclature) - Ontology term suggestions (HPO, GO, CL, UBERON, CHEBI, MAXO, MONDO) where applicable - Evidence citations with PMIDs - Direct quotes from abstracts to support key claims - Clear indication when information is not available or not applicable for this disease
This report will be used to populate a disease knowledge base entry with: - Pathophysiology descriptions with causal chains - Gene/protein annotations (HGNC, GO terms) - Phenotype associations (HP terms) with frequencies - Cell type involvement (CL terms) - Anatomical locations (UBERON terms) - Chemical entities (CHEBI terms) - Treatment annotations (MAXO terms) - Evidence items with PMIDs and exact abstract quotes - Epidemiology, prognosis, diagnostic, and prevention information - Animal model descriptions with phenotype recapitulation details
DSRCT is an extremely rare, highly aggressive sarcoma that most often presents with multifocal peritoneal/abdominopelvic disease in adolescents and young adults, with strong male predominance, and poor long-term survival despite intensive multimodal therapy (surgery + chemotherapy + radiation) (magrath2024desmoplasticsmallround pages 1-2, magrath2024comprehensivetranscriptomicanalysis pages 1-2, boulay2024ewswt1fusionisoforms pages 1-2). The defining molecular lesion is a reciprocal t(11;22)(p13;q12) translocation generating an oncogenic chimeric transcription factor EWSR1::WT1 (magrath2024desmoplasticsmallround pages 1-2, magrath2024comprehensivetranscriptomicanalysis pages 1-2, boulay2024ewswt1fusionisoforms pages 1-2). Recent 2024 mechanistic studies demonstrate that EWSR1::WT1 is a potent chromatin activator, preferentially binding active distal regulatory elements and driving vulnerabilities such as the cyclin D–CDK4/6–RB axis, supporting clinical translation of CDK4/6 inhibition (e.g., palbociclib) (magrath2024comprehensivetranscriptomicanalysis pages 1-2, boulay2024ewswt1fusionisoforms pages 1-2, gaidzik2024desmoplasticsmallround pages 1-2).
DSRCT is a rare, highly aggressive sarcoma characterized histologically by nests of small round cells embedded in an abundant desmoplastic/fibrous stroma (magrath2024desmoplasticsmallround pages 1-2, wang2021clinicopathologicalfeaturesof pages 1-2). It most commonly originates in the abdominal and pelvic peritoneum and typically presents with disseminated peritoneal implants at diagnosis (magrath2024desmoplasticsmallround pages 1-2).
Evidence in this report is drawn from: - Aggregated resources (reviews; retrospective cohorts; ClinicalTrials.gov records) and - Primary research (mechanistic studies in xenografts/PDX; pathology case series). This is not derived from EHR-only sources.
Primary causal event: DSRCT is caused/defined by a reciprocal chromosomal translocation t(11;22)(p13;q12) producing the EWSR1::WT1 fusion oncogene/oncoprotein (magrath2024desmoplasticsmallround pages 1-2, magrath2024comprehensivetranscriptomicanalysis pages 1-2, boulay2024ewswt1fusionisoforms pages 1-2).
Mechanistic definition: EWSR1::WT1 is an oncogenic fusion transcription factor combining EWSR1’s N-terminal low complexity/prion-like activation domain with WT1 zinc-finger DNA-binding domains (magrath2024comprehensivetranscriptomicanalysis pages 1-2, boulay2024ewswt1fusionisoforms pages 1-2).
No environmental, infectious, or inherited germline risk factors were identified in the retrieved evidence. The consistent defining lesion is somatic fusion-driven oncogenesis (magrath2024desmoplasticsmallround pages 1-2, magrath2024comprehensivetranscriptomicanalysis pages 1-2).
No protective factors or GxE interactions were identified in the retrieved evidence set.
Typical presentation: Extended abdominal mass with symptoms from mass effect such as pain, abdominal distension, acute abdomen, and organ obstruction (gaidzik2024desmoplasticsmallround pages 1-2). A retrospective abdominal series reported common symptoms including abdominal distension, abdominal pain, and constipation (wang2021clinicopathologicalfeaturesof pages 1-2).
Metastatic pattern: High rate of metastatic disease at diagnosis is emphasized in mechanistic/clinical literature; one 2021 series reported 7/8 patients developed metastases to distant organs or lymph nodes (wang2021clinicopathologicalfeaturesof pages 1-2), consistent with “high rate of metastasis at diagnosis” in mechanistic work (magrath2024comprehensivetranscriptomicanalysis pages 1-2).
QoL is not systematically quantified in the retrieved published cohorts. However, an ongoing trial explicitly measures QoL using EORTC QLQ-C30 and pain inventory (NCT07328425) (NCT07328425 chunk 1).
Two key 2024 studies emphasize that EWSR1::WT1 exists as two isoforms differing by inclusion/exclusion of three amino acids (KTS) between WT1 zinc fingers 3 and 4 (magrath2024desmoplasticsmallround pages 1-2, boulay2024ewswt1fusionisoforms pages 1-2, magrath2024comprehensivetranscriptomicanalysis pages 1-2). Both isoforms appear required for tumor phenotypes resembling primary DSRCT (boulay2024ewswt1fusionisoforms pages 1-2). Another 2024 study found the E-KTS isoform plays a dominant role in transcription and directly binds the CCND1 promoter (magrath2024comprehensivetranscriptomicanalysis pages 1-2).
A 2024 Nature Communications study shows EWS-WT1 binding is enriched at distal regulatory elements (enhancers) marked by H3K4me1 and H3K27ac, consistent with a chromatin-activating role (boulay2024ewswt1fusionisoforms pages 1-2). In JN-DSRCT1, 91% of binding loci were distal regulatory elements (boulay2024ewswt1fusionisoforms pages 1-2).
No non-genetic environmental, lifestyle, or infectious contributors were identified in the retrieved evidence.
1) Initiating trigger: t(11;22)(p13;q12) yields EWSR1::WT1 fusion (magrath2024desmoplasticsmallround pages 1-2, magrath2024comprehensivetranscriptomicanalysis pages 1-2). 2) Upstream mechanism: EWSR1::WT1 acts as an aberrant transcription factor/chromatin activator; binding is concentrated at active enhancers and drives tumor-specific oncogenic gene expression programs (boulay2024ewswt1fusionisoforms pages 1-2). 3) Downstream effectors/vulnerabilities: Fusion-driven transcription upregulates the cyclin D–CDK4/6–RB axis via CCND1 promoter binding (magrath2024comprehensivetranscriptomicanalysis pages 1-2), generating sensitivity to CDK4/6 inhibitors (palbociclib) in xenograft/PDX models (magrath2024comprehensivetranscriptomicanalysis pages 1-2, boulay2024ewswt1fusionisoforms pages 1-2).
Evidence supports the following ontology suggestions (mapping requires curator verification): - GO: cell cycle (GO:0007049), positive regulation of transcription by RNA polymerase II (GO:0045944), chromatin organization (GO:0006325) (mechanistically supported by fusion TF/chromatin activator role) (magrath2024comprehensivetranscriptomicanalysis pages 1-2, boulay2024ewswt1fusionisoforms pages 1-2). - CL: likely mesenchymal-derived tumor cells; precise cell of origin is unknown and not resolved in the retrieved evidence (magrath2024desmoplasticsmallround pages 1-2).
DSRCT is not described as a Mendelian inherited disorder in the retrieved evidence; it is defined by a somatic fusion oncogene (magrath2024desmoplasticsmallround pages 1-2).
Because DSRCT resembles other small round cell tumors histologically and can occur at unusual sites, combined morphology + broad IHC + molecular fusion confirmation is emphasized to avoid misclassification (gaidzik2024desmoplasticsmallround pages 1-2, hatanaka2019desmoplasticsmallround pages 1-2).
DSRCT is typically managed with multimodal therapy: intensive systemic chemotherapy plus cytoreductive surgery and radiotherapy when feasible (magrath2024comprehensivetranscriptomicanalysis pages 1-2, magrath2024desmoplasticsmallround pages 1-2, gaidzik2024desmoplasticsmallround pages 1-2).
MAXO suggestions (examples): - Surgical cytoreduction/debulking: MAXO term corresponding to surgical resection/cytoreductive surgery. - Systemic antineoplastic chemotherapy. - Radiation therapy (whole abdominopelvic radiation / IMRT).
Figure/Table evidence: The 2023 review contains a detailed Table 3 (“Publications of HIPEC in DSRCT”) summarizing regimens, selection criteria, and outcomes across small series (reijers2023intraabdominaldesmoplasticsmall media 0abe3cc8, reijers2023intraabdominaldesmoplasticsmall media e3635690, reijers2023intraabdominaldesmoplasticsmall media bbec6d82).
Whole abdominopelvic radiotherapy (WART) is used for diffuse peritoneal involvement; IMRT approaches aim to reduce toxicity (reijers2023intraabdominaldesmoplasticsmall pages 9-10). One French Sarcoma Group study cited in the review improved 3-year OS from 37.6% to 61.2% (p=0.045) after adjuvant radiation (reijers2023intraabdominaldesmoplasticsmall pages 9-10).
A 2024 review highlights preclinical dependencies including NTRK3, EGFR, and immunotherapy strategies targeting B7-H3 (magrath2024desmoplasticsmallround pages 1-2). (These are mentioned as priorities but without outcome statistics in the retrieved excerpt.)
No primary prevention or screening strategies are established in the retrieved evidence, consistent with a rare, non-hereditary, fusion-driven cancer.
No naturally occurring DSRCT analogs in other species were identified in the retrieved evidence.
1) Chromatin-centric mechanism: 2024 Nature Communications provides high-resolution evidence that EWS-WT1 acts as a chromatin activator with enhancer-dominant binding (91% distal elements) and active enhancer marks (H3K4me1/H3K27ac), supporting a model of fusion-driven enhancer reprogramming (boulay2024ewswt1fusionisoforms pages 1-2). 2) Therapeutic vulnerability in cell cycle: 2024 Cancer Research identifies CCND1 promoter binding and cyclin D–CDK4/6–RB axis dependence, providing a rationale for rapid clinical translation of CDK4/6 inhibitors in DSRCT (magrath2024comprehensivetranscriptomicanalysis pages 1-2). 3) Clinical translation evidence: A 2024 7-patient series reports CCND1 gains and cyclin D1 expression with use of palbociclib (median 4.5 months to progression), suggesting modest activity and supporting biomarker-driven trial development (gaidzik2024desmoplasticsmallround pages 1-2). 4) HIPEC remains controversial: 2023 systematic review concludes benefit is unproven and morbidity can be high; complete cytoreduction remains the dominant surgical determinant, with HIPEC potentially reserved to expert centers and selected patients (reijers2023intraabdominaldesmoplasticsmall pages 1-2, reijers2023intraabdominaldesmoplasticsmall pages 9-10).
| NCT ID | Title | Intervention(s) | Target / rationale | Phase | Status | Key eligibility (selected) | Primary endpoint | Start date | Last update post date | URL |
|---|---|---|---|---|---|---|---|---|---|---|
| NCT04022213 | A Study of the Drug I131-Omburtamab in People With Desmoplastic Small Round Cell Tumors and Other Solid Tumors in the Peritoneum | Intraperitoneal 131I-omburtamab; Group A also receives WAP-IMRT (30 Gy in 20 fractions) | B7-H3–directed radioimmunotherapy; protocol includes immunohistochemistry to assess B7H3 expression for eligibility in non-DSRCT assessment arm; intended to reduce recurrence/progression after cytoreduction (NCT04022213 chunk 1) | Phase 2 | Active, not recruiting | DSRCT confirmed at MSKCC; age >1 year; recovery from prior therapy; after surgery must have no definitive radiologic disease in liver/outside abdomen-pelvis or have had gross total resection; excludes prior progression, prior HIPEC, significant organ toxicities, mouse protein allergy, grade 4 hypersensitivity to radiolabeled iodine (NCT04022213 chunk 1) | Progression-free survival after RIT + WA/WAP-IMRT (NCT04022213 chunk 1) | 2019-07-15 | 2025-07-20 | https://clinicaltrials.gov/study/NCT04022213 |
| NCT04145349 | CAMPFIRE: A Study of Ramucirumab (LY3009806) in Children and Young Adults With Desmoplastic Small Round Cell Tumor | Ramucirumab, cyclophosphamide, vinorelbine (NCT04145349 chunk 3, NCT04145349 chunk 2) | VEGFR2 inhibition (ramucirumab) combined with chemotherapy for relapsed/recurrent/refractory DSRCT in children and young adults (inferred from intervention and known drug target; record title identifies ramucirumab study) (NCT04145349 chunk 2, NCT04145349 chunk 3) | Phase 1/2 | Active, not recruiting | Age 12 months to 29 years; relapsed/recurrent/refractory DSRCT; measurable disease by RECIST 1.1; at least one prior systemic therapy; not eligible for surgical resection; adequate cardiac, blood pressure, hematologic, renal, liver, and coagulation function; excludes active infection, recent major surgery, significant bleeding/thrombosis, CNS involvement, bowel obstruction, prior progression on cyclophosphamide+vinorelbine combination (NCT04145349 chunk 2) | Not available in retrieved chunk; record emphasizes efficacy/safety assessments with measurable disease requirements (NCT04145349 chunk 2) | 2020 (record year in retrieved trial summary) | Not available in retrieved chunk | https://clinicaltrials.gov/study/NCT04145349 |
| NCT07328425 | Clinical Study in Adult and Young Adult Patients With Advanced Desmoplastic Small Round Cell Tumor (DSRCT) ISG-TULIPS | Lurbinectedin + irinotecan | Molecularly selected advanced DSRCT (EWSR1-WT1 translocation-positive); rationale is activity of combination in 2nd–4th line advanced disease after anthracycline-based chemotherapy, with optional biologic study to analyze tumor genes/molecules (NCT07328425 chunk 1) | Phase 2 | Recruiting | Histologically and molecularly confirmed DSRCT with documented EWSR1-WT1 translocation; age ≥15 years; locally advanced or metastatic measurable disease; progression after last standard therapy; at least one prior anthracycline-based regimen and ≤3 prior chemotherapy lines; ECOG ≤2; adequate marrow/renal/hepatic/cardiac function; excludes prior lurbinectedin/trabectedin-related agents and significant uncontrolled comorbidity (NCT07328425 chunk 1) | Overall tumour response rate (ORR) by RECIST v1.1 (NCT07328425 chunk 1) | 2025-11-21 | 2026-04-03 | https://clinicaltrials.gov/study/NCT07328425 |
Table: This table summarizes key interventional DSRCT trials retrieved from ClinicalTrials.gov, focusing on interventions, biologic rationale, eligibility, and endpoints. It is useful for quickly comparing current and recent real-world trial implementations in this rare sarcoma.
Key examples: - Compartmental radioimmunotherapy: NCT04022213 uses intraperitoneal 131I-omburtamab (B7-H3-directed) with/without WAP-IMRT after cytoreduction (NCT04022213 chunk 1). - Anti-angiogenic strategy: NCT04145349 evaluates ramucirumab plus chemotherapy backbone in relapsed/refractory pediatric/young adult DSRCT (NCT04145349 chunk 2). - Second-/later-line cytotoxic combinations: NCT07328425 (ISG-TULIPS) tests lurbinectedin + irinotecan in molecularly confirmed EWSR1-WT1 DSRCT after anthracyclines, with ORR as primary endpoint and QoL as secondary endpoints (NCT07328425 chunk 1).
| Item | Details | Source (with DOI/URL and publication year) |
|---|---|---|
| Disease definition | Rare, highly aggressive soft-tissue/pediatric sarcoma composed of nests of small round cells embedded in desmoplastic/fibrous stroma; often described as a small round blue cell tumor. (magrath2024desmoplasticsmallround pages 1-2, gaidzik2024desmoplasticsmallround pages 1-2, wang2021clinicopathologicalfeaturesof pages 1-2) | Magrath et al., 2024, Front Cell Dev Biol; DOI: 10.3389/fcell.2024.1442488; https://doi.org/10.3389/fcell.2024.1442488. Gaidzik et al., 2024, Sarcoma; DOI: 10.1155/2024/5036102; https://doi.org/10.1155/2024/5036102. Wang et al., 2021, World J Surg Oncol; DOI: 10.1186/s12957-021-02310-6; https://doi.org/10.1186/s12957-021-02310-6 |
| Hallmark genetic event | Defining lesion is t(11;22)(p13;q12) creating the EWSR1::WT1 fusion oncogene/oncoprotein. (magrath2024comprehensivetranscriptomicanalysis pages 1-2, magrath2024desmoplasticsmallround pages 1-2, boulay2024ewswt1fusionisoforms pages 1-2) | Magrath et al., 2024, Cancer Research; DOI: 10.1158/0008-5472.CAN-23-3334; https://doi.org/10.1158/0008-5472.CAN-23-3334. Magrath et al., 2024, Front Cell Dev Biol; DOI: 10.3389/fcell.2024.1442488; https://doi.org/10.3389/fcell.2024.1442488. Boulay et al., 2024, Nat Commun; DOI: 10.1038/s41467-024-51851-3; https://doi.org/10.1038/s41467-024-51851-3 |
| Canonical fusion breakpoint example | Example molecular confirmation reported as fusion between EWSR1 exon 7 and WT1 exon 8. (hatanaka2019desmoplasticsmallround pages 1-2) | Hatanaka et al., 2019, Diagnostic Pathology; DOI: 10.1186/s13000-019-0825-1; https://doi.org/10.1186/s13000-019-0825-1 |
| Typical age | Predominantly affects children, adolescents, and young adults; median age in one 2024 series was 24.8 years; commonly occurs in young men aged 20–30 years. (gaidzik2024desmoplasticsmallround pages 1-2, wang2021clinicopathologicalfeaturesof pages 1-2) | Gaidzik et al., 2024, Sarcoma; DOI: 10.1155/2024/5036102; https://doi.org/10.1155/2024/5036102. Wang et al., 2021, World J Surg Oncol; DOI: 10.1186/s12957-021-02310-6; https://doi.org/10.1186/s12957-021-02310-6 |
| Sex distribution | Strong male predominance; incidence in the U.S. reported as 0.22 per million males vs 0.05 per million females; one review notes most cases occur in males. (magrath2024desmoplasticsmallround pages 1-2, hatanaka2019desmoplasticsmallround pages 1-2) | Magrath et al., 2024, Front Cell Dev Biol; DOI: 10.3389/fcell.2024.1442488; https://doi.org/10.3389/fcell.2024.1442488. Hatanaka et al., 2019, Diagnostic Pathology; DOI: 10.1186/s13000-019-0825-1; https://doi.org/10.1186/s13000-019-0825-1 |
| Common primary sites | Most commonly arises in the abdominal/pelvic peritoneum and abdominopelvic cavity; frequent sites include retroperitoneum, pelvis, omentum, mesentery/peritoneal surfaces. (magrath2024desmoplasticsmallround pages 1-2, gaidzik2024desmoplasticsmallround pages 1-2, wang2021clinicopathologicalfeaturesof pages 1-2) | Magrath et al., 2024, Front Cell Dev Biol; DOI: 10.3389/fcell.2024.1442488; https://doi.org/10.3389/fcell.2024.1442488. Gaidzik et al., 2024, Sarcoma; DOI: 10.1155/2024/5036102; https://doi.org/10.1155/2024/5036102. Wang et al., 2021, World J Surg Oncol; DOI: 10.1186/s12957-021-02310-6; https://doi.org/10.1186/s12957-021-02310-6 |
| Tumor spread pattern | Often presents with multiple peritoneal nodules/implants, high metastatic burden, and high metastasis rate at diagnosis. (magrath2024desmoplasticsmallround pages 1-2, magrath2024comprehensivetranscriptomicanalysis pages 1-2, wang2021clinicopathologicalfeaturesof pages 1-2) | Magrath et al., 2024, Front Cell Dev Biol; DOI: 10.3389/fcell.2024.1442488; https://doi.org/10.3389/fcell.2024.1442488. Magrath et al., 2024, Cancer Research; DOI: 10.1158/0008-5472.CAN-23-3334; https://doi.org/10.1158/0008-5472.CAN-23-3334. Wang et al., 2021, World J Surg Oncol; DOI: 10.1186/s12957-021-02310-6; https://doi.org/10.1186/s12957-021-02310-6 |
| Key histopathology | Well-defined nests of small round blue cells separated by abundant/desmoplastic stroma. (wang2021clinicopathologicalfeaturesof pages 1-2, hatanaka2019desmoplasticsmallround pages 1-2) | Wang et al., 2021, World J Surg Oncol; DOI: 10.1186/s12957-021-02310-6; https://doi.org/10.1186/s12957-021-02310-6. Hatanaka et al., 2019, Diagnostic Pathology; DOI: 10.1186/s13000-019-0825-1; https://doi.org/10.1186/s13000-019-0825-1 |
| Key diagnostic immunohistochemistry | Typical polyphenotypic profile includes epithelial markers (cytokeratins, EMA), mesenchymal/myogenic markers (desmin, vimentin), and WT1 C-terminal positivity; desmin may show paranuclear dot-like staining. (gaidzik2024desmoplasticsmallround pages 1-2, hatanaka2019desmoplasticsmallround pages 1-2, wang2021clinicopathologicalfeaturesof pages 1-2) | Gaidzik et al., 2024, Sarcoma; DOI: 10.1155/2024/5036102; https://doi.org/10.1155/2024/5036102. Hatanaka et al., 2019, Diagnostic Pathology; DOI: 10.1186/s13000-019-0825-1; https://doi.org/10.1186/s13000-019-0825-1. Wang et al., 2021, World J Surg Oncol; DOI: 10.1186/s12957-021-02310-6; https://doi.org/10.1186/s12957-021-02310-6 |
| Preferred WT1 antibody region | C-terminal WT1 antibody is diagnostically informative; N-terminal WT1 may be negative in fusion-positive tumors. (hatanaka2019desmoplasticsmallround pages 1-2, wang2021clinicopathologicalfeaturesof pages 1-2) | Hatanaka et al., 2019, Diagnostic Pathology; DOI: 10.1186/s13000-019-0825-1; https://doi.org/10.1186/s13000-019-0825-1. Wang et al., 2021, World J Surg Oncol; DOI: 10.1186/s12957-021-02310-6; https://doi.org/10.1186/s12957-021-02310-6 |
| Molecular diagnostic tests | Molecular confirmation can be performed by FISH for EWSR1 rearrangement and/or RT-PCR/NGS for EWSR1-WT1 fusion; fusion detection is described as the gold standard for definitive diagnosis. (gaidzik2024desmoplasticsmallround pages 1-2, hatanaka2019desmoplasticsmallround pages 1-2, wang2021clinicopathologicalfeaturesof pages 1-2) | Gaidzik et al., 2024, Sarcoma; DOI: 10.1155/2024/5036102; https://doi.org/10.1155/2024/5036102. Hatanaka et al., 2019, Diagnostic Pathology; DOI: 10.1186/s13000-019-0825-1; https://doi.org/10.1186/s13000-019-0825-1. Wang et al., 2021, World J Surg Oncol; DOI: 10.1186/s12957-021-02310-6; https://doi.org/10.1186/s12957-021-02310-6 |
| Incidence statistics | U.S. age-adjusted incidence reported as 0.22/million in males and 0.05/million in females. (magrath2024desmoplasticsmallround pages 1-2) | Magrath et al., 2024, Front Cell Dev Biol; DOI: 10.3389/fcell.2024.1442488; https://doi.org/10.3389/fcell.2024.1442488 |
| Survival statistics | Prognosis remains poor; reported 5-year survival ~15–30%, with one 2024 mechanistic study citing 15–25%. (magrath2024desmoplasticsmallround pages 1-2, magrath2024comprehensivetranscriptomicanalysis pages 1-2, boulay2024ewswt1fusionisoforms pages 1-2) | Magrath et al., 2024, Front Cell Dev Biol; DOI: 10.3389/fcell.2024.1442488; https://doi.org/10.3389/fcell.2024.1442488. Magrath et al., 2024, Cancer Research; DOI: 10.1158/0008-5472.CAN-23-3334; https://doi.org/10.1158/0008-5472.CAN-23-3334. Boulay et al., 2024, Nat Commun; DOI: 10.1038/s41467-024-51851-3; https://doi.org/10.1038/s41467-024-51851-3 |
| Recent biological vulnerability | 2024 studies identified CCND1 / cyclin D–CDK4/6–RB axis as an EWSR1::WT1-driven dependency; palbociclib reduced DSRCT growth in xenograft/PDX models. (magrath2024comprehensivetranscriptomicanalysis pages 1-2, boulay2024ewswt1fusionisoforms pages 1-2) | Magrath et al., 2024, Cancer Research; DOI: 10.1158/0008-5472.CAN-23-3334; https://doi.org/10.1158/0008-5472.CAN-23-3334. Boulay et al., 2024, Nat Commun; DOI: 10.1038/s41467-024-51851-3; https://doi.org/10.1038/s41467-024-51851-3 |
| Small clinical signal for CDK4/6 inhibition | In a 7-patient 2024 series, Cyclin D1 expression was seen in all tumors; CCND1 gains in 2 cases; palbociclib in 4 patients yielded median treatment duration to progression of 4.5 months. (gaidzik2024desmoplasticsmallround pages 1-2) | Gaidzik et al., 2024, Sarcoma; DOI: 10.1155/2024/5036102; https://doi.org/10.1155/2024/5036102 |
| CRS/HIPEC outcome signal | HIPEC evidence is mixed: a 2023 review reported a phase 2 MDACC series with 3-year OS 79% and median OS 58.4 months, but concluded no clear proven survival benefit of adding HIPEC after complete cytoreductive surgery and noted higher morbidity. (reijers2023intraabdominaldesmoplasticsmall pages 9-10, reijers2023intraabdominaldesmoplasticsmall pages 1-2) | Reijers et al., 2023, Current Oncology; DOI: 10.3390/curroncol30040299; https://doi.org/10.3390/curroncol30040299 |
Table: This table summarizes core identifiers, clinicopathologic features, diagnostic markers, and headline epidemiology/survival data for desmoplastic small round cell tumor. It is limited to facts directly supported by the provided evidence snippets and includes source links and publication years for quick reference.
DSRCT is rare; most clinical evidence is retrospective, single-institution, and heterogeneous (small samples, variable selection for cytoreduction/HIPEC, variable systemic regimens), limiting definitive causal inference for many interventions (reijers2023intraabdominaldesmoplasticsmall pages 9-10, jayakrishnan2021desmoplasticsmallroundcell pages 1-3). Mechanistic evidence for targeted therapies (e.g., CDK4/6 inhibition) is strong in 2024 xenograft/PDX models, but prospective DSRCT-specific clinical trial outcomes were not available in the retrieved evidence set (magrath2024comprehensivetranscriptomicanalysis pages 1-2, boulay2024ewswt1fusionisoforms pages 1-2).
References
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(sundaramoorthy2025managementandsurvival pages 4-5): Senthamizhan Sundaramoorthy, George Abraham, Geetha Narayanan, Sreejith G. Nair, Prakash N. Purushothaman, Sugeeth Mangalapilly Thambi, Sherin P. Mathew, Deepa Susan Joy Philip, Sindhu Nair, and Thamizharuvi Muthukumarasamy. Management and survival outcomes of desmoplastic small round cell tumor: a retrospective cohort study from a tertiary cancer center. BMC Cancer, Dec 2025. URL: https://doi.org/10.1186/s12885-025-15390-4, doi:10.1186/s12885-025-15390-4. This article has 0 citations and is from a peer-reviewed journal.
(sundaramoorthy2025managementandsurvival pages 1-2): Senthamizhan Sundaramoorthy, George Abraham, Geetha Narayanan, Sreejith G. Nair, Prakash N. Purushothaman, Sugeeth Mangalapilly Thambi, Sherin P. Mathew, Deepa Susan Joy Philip, Sindhu Nair, and Thamizharuvi Muthukumarasamy. Management and survival outcomes of desmoplastic small round cell tumor: a retrospective cohort study from a tertiary cancer center. BMC Cancer, Dec 2025. URL: https://doi.org/10.1186/s12885-025-15390-4, doi:10.1186/s12885-025-15390-4. This article has 0 citations and is from a peer-reviewed journal.
(gawash2025desmoplasticsmallround pages 2-4): Ahmed Gawash, Alexa Simonetti, Brandon J. Goodwin, and Alissa Brotman O’Neill. Desmoplastic small round cell tumor: an update of current management practices. Journal of the Egyptian National Cancer Institute, 37 1:13, Apr 2025. URL: https://doi.org/10.1186/s43046-025-00276-0, doi:10.1186/s43046-025-00276-0. This article has 2 citations.
(jayakrishnan2021desmoplasticsmallroundcell pages 1-3): THEJUS JAYAKRISHNAN, RYAN MOLL, ARIEL SANDHU, ANGELA SANGUINO, GURVEEN KAUR, and SHIFENG MAO. Desmoplastic small round-cell tumor: retrospective review of institutional data and literature review. Anticancer Research, 41:3859-3866, Jul 2021. URL: https://doi.org/10.21873/anticanres.15179, doi:10.21873/anticanres.15179. This article has 24 citations and is from a peer-reviewed journal.
(reijers2023intraabdominaldesmoplasticsmall pages 1-2): Sophie J. M. Reijers, Caroline C. H. Siew, Niels F. M. Kok, Charles Honoré, and Winan J. van Houdt. Intra-abdominal desmoplastic small round cell tumor (dsrct) and the role of hyperthermic intraperitoneal chemotherapy (hipec): a review. Current Oncology, 30:3951-3963, Mar 2023. URL: https://doi.org/10.3390/curroncol30040299, doi:10.3390/curroncol30040299. This article has 14 citations.
(reijers2023intraabdominaldesmoplasticsmall media 0abe3cc8): Sophie J. M. Reijers, Caroline C. H. Siew, Niels F. M. Kok, Charles Honoré, and Winan J. van Houdt. Intra-abdominal desmoplastic small round cell tumor (dsrct) and the role of hyperthermic intraperitoneal chemotherapy (hipec): a review. Current Oncology, 30:3951-3963, Mar 2023. URL: https://doi.org/10.3390/curroncol30040299, doi:10.3390/curroncol30040299. This article has 14 citations.
(reijers2023intraabdominaldesmoplasticsmall media e3635690): Sophie J. M. Reijers, Caroline C. H. Siew, Niels F. M. Kok, Charles Honoré, and Winan J. van Houdt. Intra-abdominal desmoplastic small round cell tumor (dsrct) and the role of hyperthermic intraperitoneal chemotherapy (hipec): a review. Current Oncology, 30:3951-3963, Mar 2023. URL: https://doi.org/10.3390/curroncol30040299, doi:10.3390/curroncol30040299. This article has 14 citations.
(reijers2023intraabdominaldesmoplasticsmall media bbec6d82): Sophie J. M. Reijers, Caroline C. H. Siew, Niels F. M. Kok, Charles Honoré, and Winan J. van Houdt. Intra-abdominal desmoplastic small round cell tumor (dsrct) and the role of hyperthermic intraperitoneal chemotherapy (hipec): a review. Current Oncology, 30:3951-3963, Mar 2023. URL: https://doi.org/10.3390/curroncol30040299, doi:10.3390/curroncol30040299. This article has 14 citations.
(NCT04145349 chunk 3): CAMPFIRE: A Study of Ramucirumab (LY3009806) in Children and Young Adults With Desmoplastic Small Round Cell Tumor. Eli Lilly and Company. 2020. ClinicalTrials.gov Identifier: NCT04145349
(NCT04145349 chunk 2): CAMPFIRE: A Study of Ramucirumab (LY3009806) in Children and Young Adults With Desmoplastic Small Round Cell Tumor. Eli Lilly and Company. 2020. ClinicalTrials.gov Identifier: NCT04145349