The DSRCT Initiative
DSRCT Research & Resource Library
A working collection of research, treatment literature, clinical trials, and community resources focused on desmoplastic small round cell tumor. Select a category to expand it; each entry opens the original source in a new tab. Full text links are provided when available.
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Foundational ResearchThe landmark papers that defined DSRCT
- Intra-abdominal desmoplastic small round-cell tumor: report of 19 cases of a distinctive type of high-grade polyphenotypic malignancy affecting young individuals, American Journal of Surgical Pathology (1991)
- Fusion of the EWS and WT1 genes in the desmoplastic small round cell tumor, Cancer Research (1994)
- Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy (P6 protocol), Journal of Clinical Oncology (1996)
- Clinical, pathologic, and molecular spectrum of tumors associated with t(11;22)(p13;q12): desmoplastic small round-cell tumor and its variants, Journal of Clinical Oncology (1998)
- Whole abdominopelvic radiotherapy for desmoplastic small round-cell tumor, International Journal of Radiation Oncology, Biology, Physics (2002)
- Results of multimodal treatment for desmoplastic small round cell tumors, Journal of Pediatric Surgery (2005)
- Novel Treatment for Desmoplastic Small Round Cell Tumor: Hyperthermic Intraperitoneal Perfusion, American Journal of Surgical Pathology (2010)
Overviews & ReviewsBroad summaries of the disease and its management
- The Diagnosis and Management of Desmoplastic Small Round Cell Tumor: A Review, Current Opinion in Oncology (2011)
- Desmoplastic Small Round Cell Tumor: Current Management and Recent Findings, Sarcoma (2012)
- Management of Desmoplastic Small Round Cell Tumor, Seminars in Pediatric Surgery (2016)
- Antiangiogenic effects in patients with progressive desmoplastic small round cell tumor: data from the French national registry dedicated to the use of off-labeled targeted therapy in sarcoma (OUTC’s), Clinical Sarcoma Research (2017)
- Desmoplastic Small Round Blue Cell Tumor: A Review of Treatment and Potential Therapeutic Genomic Alterations, Sarcoma (2017)
- Desmoplastic small round cell tumour (DSRCT): emerging therapeutic targets and future directions for potential therapies, Expert Opinion on Therapeutic Targets (2020)
- The Chicago Consensus on peritoneal surface malignancies, Cancer (2020)
- Intra-Abdominal Desmoplastic Small Round Cell Tumor: Current Treatment Options and Perspectives, Frontiers in Oncology (2021)
- Desmoplastic small round cell tumor: from state of the art to future clinical prospects, Expert Review of Anticancer Therapy (2023)
- NCCN Clinical Practice Guidelines in Oncology: Soft Tissue Sarcoma, National Comprehensive Cancer Network
Published Case ReportsPatient cases reported in medical literature
- Desmoplastic small round cell tumor of the kidney with bone metastasis: A case report, Radiology Case Reports (2026)
- Radiologically Occult Desmoplastic Small Round Cell Tumor in a 36-Year-Old Man: A Case Report, Cureus (2026)
- Durable response to treatment of an atypical desmoplastic small round cell tumor with enfortumab-vedotin, Journal of Cancer Research and Clinical Oncology (2025)
- Unresectable Desmoplastic Small Round Cell Tumor: A Case Report and Review of the Literature, Case Reports in Oncology (2025)
- Intra-abdominal multifocal desmoplastic small round cell tumor: a case report of a rare neoplasm, International Surgery Journal (2025)
- Successful surgical treatment of desmoplastic small round cell tumor in a postmenopausal woman: A rare case report, International Journal of Surgery Case Reports (2024)
- Primary Desmoplastic Small Round Cell Tumor of the Femur: Case Report and Review of a Rare Intraosseous Malignancy, Journal of Surgical Pathology (2022)
- A Classic Presentation of Desmoplastic Small Round Cell Tumor, Cureus (2021)
- Chemotherapy combined with apatinib for the treatment of desmoplastic small round cell tumors, Journal of Cancer Research and Therapeutics (2020)
- Desmoplastic small round cell tumor presenting as an inguinal mass in a 2-year old boy, Journal of Pediatric Surgery Case Reports (2020)
- Effective treatment of apatinib in desmoplastic small round cell tumor: a case report and literature review, BMC Cancer (2018)
Epidemiology & OutcomesIncidence, survival, and prognosis
- Long‐term survivors with desmoplastic small round cell tumor (DSRCT): Results from a retrospective single‐institution case series analysis - PMC Cancer Medicine (2023)
- Changing incidence and survival of desmoplastic small round cell tumor in the USA, PMC (2022)
- Desmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review Anticancer Research (2021)
- A novel image-based system for risk stratification in patients with desmoplastic small round cell tumor - PubMed Journal of Pediatric Surgery (2020)
- A national analysis of patterns of care and outcomes for adults diagnosed with desmoplastic small round cell tumors in the United States - PubMed Journal of Surgical Oncology (2019)
Imaging, Pathology & DiagnosisHow DSRCT is identified and confirmed
- Pelvic Desmoplastic Small Round Cell Tumor on 18F-PSMA PET/CT, Clinical Nuclear Medicine (2025)
- CXCR4-Directed Imaging and Endoradiotherapy in Desmoplastic Small Round Cell Tumors, Journal of Nuclear Medicine (2023)
- ThinPrep Cytological Findings of Desmoplastic Small Round Cell Tumor with Extensive Glandular Differentiation: A Case Study, Korean Journal of Pathology (2013)
- Pediatric Molecular Pathology (Chapter 30), Cell and Tissue Based Molecular Pathology (2009)
Molecular Profiling & GenomicsThe EWSR1::WT1 fusion, tumor biology, and targets
The EWSR1::WT1 Fusion & Tumor Biology
- Frontiers | Desmoplastic small round cell tumor: from genomics to targets, potential paths to future therapeutics, Frontiers in Cell and Developmental Biology (2024)
- EWS-WT1 fusion isoforms establish oncogenic programs and therapeutic vulnerabilities in desmoplastic small round cell tumors - Nature Communications (2024)
- Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy, Cancers (2021)
Molecular Profiling & Sequencing
- Harnessing AACR Project GENIE to Define the Molecular Features of Desmoplastic Small Round Cell Tumor, Current Issues in Molecular Biology (2026)
- Multi-layered molecular profiling informs the diagnosis and targeted therapy of desmoplastic small round cell tumor, Nature Communications (2026)
- Single-cell multiomics profiling reveals heterogeneous transcriptional programs and microenvironment in DSRCTs, Cell Reports Medicine (2024)
- Routine EWS Fusion Analysis in the Oncology Clinic to Identify Cancer-Specific Peptide Sequence Patterns That Span Breakpoints in Ewing Sarcoma and DSRCT, Cancers (2023)
- Molecular profiling in desmoplastic small round cell tumours, The International Journal of Biochemistry & Cell Biology (2023)
- Abstract 130: Multi-omic analysis of desmoplastic small sound cell tumors reveal distinct epithelial and neuroendocrine lineages, ResearchGate
Therapeutic Targets & Preclinical Models
- Desmoplastic Small Round Cell Tumors and the Role of Androgen Receptors, Current Treatment Options in Oncology (2025)
- Comprehensive Transcriptomic Analysis of EWSR1::WT1 Targets Identifies CDK4/6 Inhibitors as an Effective Therapy for Desmoplastic Small Round Cell Tumors, Cancer Research (2024)
- Novel patient-derived models of desmoplastic small round cell tumor confirm a targetable dependency on ERBB signaling - Disease Models & Mechanisms (2022)
- The androgen receptor is a therapeutic target in desmoplastic small round cell sarcoma, Nature Communications (2022)
- Desmoplastic small round cell tumor (DSRCT) xenografts and tissue culture lines: Establishment and initial characterization, Oncology Letters (2013)
- Potential Therapeutic Genomic Alterations in DSRCT — Caris Life Sciences slide, Kymera (industry)
- Abstract 6277: Identification of ATR inhibitors as therapeutic opportunities in Desmoplastic small round cell tumors, AACR
Surgery, HIPEC & RadiotherapyLocal control with surgery, HIPEC, and radiation
- Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review, Current Oncology (2023)
- Desmoplastic Small Round Cell Tumor: Long-Term Complications After Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy, Annals of Surgical Oncology (2019)
- Desmoplastic Small Round Cell Tumor Treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy: Results of a Phase 2 Trial, Annals of Surgical Oncology (2018)
- Multimodality Treatment of Desmoplastic small round cell tumor: Chemotherapy and Complete Cytoreductive Surgery Improve Patient Survival, Clinical Cancer Research (2018)
- Optimal Multimodal Treatment for Desmoplastic Small Round Cell Tumors, JAMA Oncology (2018)
- Desmoplastic Small Round Cell Tumor Treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy: Results of a Phase 2 Trial (2018)
- Survival and toxicity following sequential multimodality treatment including whole abdominopelvic radiotherapy for patients with desmoplastic small round cell tumor, Radiotherapy and Oncology (2016)
- Clinical characteristics and outcomes of abdominal desmoplastic small round cell tumor: a single-center experience of 10 cases, International Journal of Clinical and Experimental Pathology (2016)
- Peritonectomy and hyperthermic intraperitoneal chemotherapy as treatment for desmoplastic small round cell tumour, International Journal of Surgery Case Reports (2014)
Systemic & Targeted TherapyChemotherapy and targeted drug approaches
- Trastuzumab Deruxtecan Is Active in Desmoplastic Small Round Cell Tumor, Journal of Clinical Oncology (2026)
- Recommended phase 2 dose for abemaciclib in combination with irinotecan and temozolomide in pediatric and young adult patients with relapsed/refractory solid tumors, Paediatric Oncology (2025)
- Anlotinib: A Novel Targeted Drug for Bone and Soft Tissue Sarcoma (used in DSRCT in China), Frontiers in Oncology (2021)
- The Role and Clinical Effectiveness of Multiline Chemotherapy in Advanced Desmoplastic Small Round Cell Tumor, Clinical Medicine Insights: Oncology (2021)
ImmunotherapyImmune-based and radioimmunotherapy strategies
- Current Status and Progress of Targeted and Immunotherapy for DSRCT, Cancers (2026)
- HLA-Shuttle: A system for enhancing antigen presentation in immunologically cold tumors, Science Advances (2026)
- Identification of immunotherapy and radioimmunotherapy targets on desmoplastic small round cell tumors, Frontiers in Oncology (2023)
- Immunotherapy and Radioimmunotherapy for Desmoplastic Small Round Cell Tumor, Frontiers in Oncology (2021)
Clinical Trials & ResearchOpen and recent studies worldwide
- Registry to Collect Health Information About Desmoplastic Small Round Cell Tumor - Emily Slotkin, PI
- USA - Trastuzumab Deruxtecan (DS-8201a) for the Treatment of Newly Diagnosed, Recurrent or Refractory Osteosarcoma, Wilms Tumor, and Desmoplastic Small Round Cell Tumor NCT04616560
- USA - Study of Onivyde With Talazoparib or Temozolomide in Children With Recurrent Solid Tumors and Ewing Sarcoma NCT04901702
- USA - First-in-Human Study of ADCE-D01 in Soft Tissue Sarcoma (ADCElerate1) NCT06797999
- USA - Study To Evaluate The Efficacy And Safety Of Ifinatamab Deruxtecan (I-DXd) In Subjects With Recurrent Or Metastatic Solid Tumors (IDeate-PanTumor02) NCT06330064
- Beat Childhood Cancer Research Consortium In:Formation Project, BCC-BIO-001 Clinical Trial
- ITALY - Clinical Study in Adult and Young Adult Patients With Advanced Desmoplastic Small Round Cell Tumor (DSRCT) ISG-TULIPS NCT07328425
- GERMANY - Pasireotide as Maintenance Treatment in Synovial Sarcoma and Desmoplastic Small Round Cell Tumor (PAMSARC) NCT06456359
- UK - Selective Antigen Specific T Cells and CAR T Cells in Subjects With Relapsed/Refractory Embryonal Tumors (SABRE) NCT07172958
- CHINA - A Study of Sirolimus (Albumin-Bound) in Combination With Different ADCs Treatment of Advanced Solid Tumors NCT07241936
- CHINA - Safety and Efficacy of CAR-T Cell Therapy for Relapsed/refractory Neuroblastoma and Desmoplastic Small Round Cell Tumors: a Single-arm, Open-label Trial NCT06836505
Supportive CareResources on symptom management, rehabilitation, palliative care, mental health, and caregiver support. Some address cancer care broadly rather than DSRCT specifically, included for their relevance to patients, families, and caregivers navigating treatment.
Symptom Management & Integrative Care
- Integrative Medicine at Memorial Sloan-Kettering Cancer Center
- Evidence-Based Integrative Medicine in Oncology, City of Hope (2022)
- Glutamine for Amelioration of Radiation and Chemotherapy Associated Mucositis during Cancer Therapy, Nutrients (2020)
Rehabilitation & Physical Function
- Exploring the Impact of Physical Therapy on Patient Outcomes Across the Cancer Care Continuum, Cureus (2025)
- Rehabilitation to Improve the Function and Quality of Life of Soft Tissue and Bony Sarcoma Patients, Patient Related Outcome Measures (2019)
Palliative Care
- An analysis of palliative care in adolescents and young adults with soft tissue and bone sarcomas, Palliative Care and Social Practice (2025)
- Palliative Radiation Therapy in the Treatment of Desmoplastic Small Round Cell Tumors, Cureus (2023)
- Integration of Pediatric Palliative Care in Oncology Journal of Pediatrics: Clinical Practice (2026)
Mental Health & Psychosocial
- Psychosocial issues for adolescent and young adult cancer patients in a global context: A forward-looking approach, Pediatric Blood & Cancer (2019)
- Mindfulness-based interventions in oncology: A scoping review of outcomes and applications, World Journal of Clinical Cases (2025)
- The Impact of Cancer on Mental Health and the Importance of Supportive Services, Galen Medical Journal (2024)
- Mental Health Challenges in Cancer Patients: A Cross-Sectional Analysis of Depression and Anxiety, Cancers (2024)
- Psychological outcomes in patients with rare cancers: a systematic review and meta-analysise, ClinicalMedicine (2024)
- Psychiatric disorders in adolescent cancer survivors: A systematic review of prevalence and predictors, Cancer Reports (2019)
Caregiver & Family Support
- Family Caregivers in Cancer: Roles and Challenges (PDQ®) - National Cancer Institute
- Promoting psychological support services for parents of children with sarcoma through health-social partnership: A quality improvement project, Journal of Pediatric Nursing (2024)
- Psychological burden and depressive symptoms in caregivers of hemato-oncological patients: the role of medical visits, Blood Advances (2024)
Media & CommunityThese sources are useful for orientation, context, and connection, but they are not peer-reviewed primary research. They include foundations, advocacy organizations, institutional DSRCT programs, news, videos, and community pages.
Community & Peer Support
- DSRCT Initiative on Instagram
- Join the DSRCT Virtual Peer Support Community
- DSRCT Support Group on Facebook
Expert Centers & Programs in the US
- Cleveland Clinic Pediatric Solid Tumor Program
- Memorial Sloan Kettering DSRCT Program
- MD Anderson DSRCT Program
People, Experts & Advocates
- DSRCT and Sarcoma Hero, Pediatric/Medical Oncologist Dr Pete Anderson MIB Agents
- Ewing's U: Pete Anderson MD, PhD | Understanding Labs, Scans and Pathology — Little Warrior Foundation
- History-making Surgeon Dr Andrea Hayes-Dixon Howard University
- DSRCT Innovator, Pediatric Oncologist Dr Emily Slotkin
- Chief of the Pediatric Surgery Service at Memorial Sloan Kettering Cancer Center, Dr Ted Gerstle
Foundations & Funding
- FNIH ULTRA Ultra-Rare Cancer Treatment Advancement Program FNIH
- Desmoplastic Small Round Cell Tumor (DSRCT) Research Fund - BC Cancer Foundation
- Capture Desmoplastic Small Round Cell Tumor Charity
- MO’S INITIATIVE | The Maurice "Mo" Campbell Initiative at MSK Kids
- Kadeem Carson Foundation
- Steven Vanover Foundation
- Cory Monzingo Foundation
- The Brett Tashman Foundation
- Kelly Turner Foundation ICR
- Rob's ARTTT
- Alex's Lemonade Stand Foundation for Childhood Cancer 2021 POST Program Grants - Development of Combination Therapies for Desmoplastic Small Round Cell Tumor (DSRCT)
News & Educational Resources
- AACR Grantee Studies a Rare Form of Sarcoma (2023)AACR
- GARD Genetic & Rare Diseases Information Center - DSRCT NIH GARD
- Advancing Research on Desmoplastic Small Round Cell Tumour Through Innovative Models and Collaborative Efforts - OncoDaily
- Shushan Hovsepyan: Only eight interventional clinical trials exist worldwide for DSRCT - OncoDaily
- Consensus Guidelines from EpSSG and QUARTET for Delineating Highly Conformal Whole Abdominopelvic Radiotherapy - OncoDaily
- Research focus: Three Other Breakthroughs to Watch | Gustave Roussy
- Cancer Association of South Africa (CANSA) Fact Sheet on Desmoplastic Small Round Cell Tumor
- Strategies To Reduce Cancer Therapy Toxicities and Improve Eating Habits, Cleveland Clinic ConsultQD