Sarcoma
Recent clinical, regulatory, research and industry developments relating to this disease.
Video Education With Result Dependent dIsclosure
Generic medicine authorisation: Trabectedin Accord (EMA)
What's happening now
An analyst briefing on current research, clinical, regulatory and industry activity surrounding this disease.
- 4 clinical trials expected to report results, the earliest in Q1 2027.
- 4 industry developments reported.
- Q1 2027A Randomized Phase II Trial of Cabozantinib Combined With PD-1 and CTLA-4 Inhibition in Metastatic Soft Tissue Sarcoma
- Q2 2027An Open-Label, Phase II Efficacy Trial of Doxorubicin in Combination With Dual Checkpoint Blockade Using Zalifrelimab (AGEN1884) or Botensilimab (AGEN1181) With Balstilimab (AGEN2034) for Advanced or Metastatic Soft Tissue Sarcomas
- Q3 2027Phase 2 Study of Rogaratinib (BAY 1163877) in the Treatment of Patients With Sarcoma Harboring Alterations in Fibroblast Growth Factor Receptor (FGFR) 1-4 and SDH-Deficient Gastrointestinal Stromal Tumor (GIST)
- Q3 2027Phase II Trial of Ubamatamab Alone or in Combination With Cemiplimab in MUC16-Expressing SMARCB1-Deficient Malignancies
Clinical MilestonesView all 9Hide
- 2026-08-06Phase II Trial of Ubamatamab Alone or in Combination With Cemiplimab in MUC16-Expressing SMARCB1-Deficient MalignanciesResults expected Q3 2027
- 2026-07-31Phase 2 Study of Rogaratinib (BAY 1163877) in the Treatment of Patients With Sarcoma Harboring Alterations in Fibroblast Growth Factor Receptor (FGFR) 1-4 and SDH-Deficient Gastrointestinal Stromal Tumor (GIST)Results expected Q3 2027
- 2026-04-20An Open-Label, Phase II Efficacy Trial of Doxorubicin in Combination With Dual Checkpoint Blockade Using Zalifrelimab (AGEN1884) or Botensilimab (AGEN1181) With Balstilimab (AGEN2034) for Advanced or Metastatic Soft Tissue SarcomasResults expected Q2 2027
- 2025-11-12A Randomized Phase II Trial of Cabozantinib Combined With PD-1 and CTLA-4 Inhibition in Metastatic Soft Tissue SarcomaResults expected Q1 2027
- 2030-08-01A Phase 2 Study of 9-ING-41, a Glycogen Synthase Kinase-3 Beta (GSK-3β) Inhibitor, Combined With Chemotherapy in Advanced Soft Tissue and Bone SarcomasWithdrawn
- 2028-10-13Brightline-3: A Phase III, Randomized, Open-label, Multi-center Trial of Brigimadlin + Ezabenlimab Compared to Gemcitabine + Docetaxel for Second-line Treatment of Patients With Advanced TP53 Wild-type Soft Tissue Sarcoma SubtypesWithdrawn
- 2026-09-22Randomized Phase 2/3 Trial of Vincristine-Irinotecan-Regorafenib in Combination With Vincristine-Doxorubicin-Cyclophosphamide (VDC) and Ifosfamide-Etoposide (IE) in Patients With Newly Diagnosed Metastatic Ewing SarcomaTerminated
- 2026-05-25ADI-PEG 20 or Placebo Plus Gemcitabine and Docetaxel in Previously Treated Subjects With Leiomyosarcoma (ARGSARC): A Randomized, Double Blind, Multi-Center Phase 3 TrialTerminated
Industry & MarketViewHide
- 2026-10-14Erratum: Circulating Tumor DNA Profiling Defines Risk Classification in Patients With Ewing Sarcoma: A Report From the Children's Oncology Group and the LEOPARD StudyResearch news · Journal of Clinical Oncology
- 2026-09-23Kaposi Sarcoma presenting as recurrent serous pleural effusionsIndustry · Kidney International
- 2026-09-15Circulating Tumor DNA Profiling Defines Risk Classification in Patients With Ewing Sarcoma: A Report From the Children's Oncology Group and the LEOPARD StudyResearch news · Journal of Clinical Oncology
- 2026-07-31Long-Term Analysis of Patients With Ewing Sarcoma Included in the Euro-EWING99 StudyResearch news · Journal of Clinical Oncology
- 2030-08-01ClinicalA Phase 2 Study of 9-ING-41, a Glycogen Synthase Kinase-3 Beta (GSK-3β) Inhibitor, Combined With Chemotherapy in Advanced Soft Tissue and Bone SarcomasWithdrawn
- 2028-10-13ClinicalBrightline-3: A Phase III, Randomized, Open-label, Multi-center Trial of Brigimadlin + Ezabenlimab Compared to Gemcitabine + Docetaxel for Second-line Treatment of Patients With Advanced TP53 Wild-type Soft Tissue Sarcoma SubtypesWithdrawn
- 2026-10-14IndustryErratum: Circulating Tumor DNA Profiling Defines Risk Classification in Patients With Ewing Sarcoma: A Report From the Children's Oncology Group and the LEOPARD StudyResearch news · Journal of Clinical Oncology
- 2026-09-23IndustryKaposi Sarcoma presenting as recurrent serous pleural effusionsIndustry · Kidney International
- 2026-09-22ClinicalRandomized Phase 2/3 Trial of Vincristine-Irinotecan-Regorafenib in Combination With Vincristine-Doxorubicin-Cyclophosphamide (VDC) and Ifosfamide-Etoposide (IE) in Patients With Newly Diagnosed Metastatic Ewing SarcomaTerminated
- 2026-09-15IndustryCirculating Tumor DNA Profiling Defines Risk Classification in Patients With Ewing Sarcoma: A Report From the Children's Oncology Group and the LEOPARD StudyResearch news · Journal of Clinical Oncology
- 2026-08-06ClinicalPhase II Trial of Ubamatamab Alone or in Combination With Cemiplimab in MUC16-Expressing SMARCB1-Deficient MalignanciesResults expected Q3 2027
- 2026-07-31IndustryLong-Term Analysis of Patients With Ewing Sarcoma Included in the Euro-EWING99 StudyResearch news · Journal of Clinical Oncology
- 2026-07-31ClinicalPhase 2 Study of Rogaratinib (BAY 1163877) in the Treatment of Patients With Sarcoma Harboring Alterations in Fibroblast Growth Factor Receptor (FGFR) 1-4 and SDH-Deficient Gastrointestinal Stromal Tumor (GIST)Results expected Q3 2027
- 2026-05-26ClinicalNeoadjuvant Intralesional Injection of Talimogene Laherparepvec With Concurrent Preoperative Radiation in Patients With Locally Advanced Soft Tissue SarcomasPrimary completion
Therapeutic landscape
Therapies with a regulatory footing for this condition, alongside the wider set of agents co-studied with it in the literature.
Product authorisation ended — Celdoxome pegylated liposomal is indicated in adults: as monotherapy for patients with m… (2025)
Generic approval — Trabectedin Accord is indicated for the treatment of adult patients with advanced soft ti… (2025)
Original application approval — Renal-cell carcinoma (RCC) Votrient is indicated in adults for the first-line treatment o… (2010)
Original application approval — Beromun is indicated in adults as an adjunct to surgery for subsequent removal of the tum… (1999)
Original application approval — Caelyx pegylated liposomal is indicated: as monotherapy for patients with metastati… (1996)
Clinical trials
The current development programme across all trial phases.
Regulatory timeline
Drug regulatory events matched to this condition by indication — EMA.
European Medicines Agency (CC BY 4.0). Events are matched to this condition by drug indication text — approvals/updates for drugs indicated for it, not disease-specific acts.
Research activity
Key research shaping understanding of this disease, combining the latest publications with the most influential evidence.
Major themes6
- Sarcoma5
- Soft Tissue Neoplasms4
- Immunotherapy1
- Neoplasms, Second Primary1
- Nivolumab1
- Sarcoma, Ewing1
Leading journals6
- Cancer research communications1
- Frontiers in immunology1
- Journal of the National Comprehensive Cancer Network : JNCCN1
- Nature1
- Oncoimmunology1
- Pathologica1
Leading researchers8
- Clancy T2
- Keung EZ2
- Stratford R2
- Tawbi HA2
- Adam J1
- Agulnik M1
- Albieri B1
- Anzar I1
Affiliations (unnormalised)6
- The University of Texas MD Anderson Cancer Center2
- 10O'Neal Comprehensive Cancer Center at UAB.1
- 11UT Southwestern Simmons Comprehensive Cancer Center.1
- 12Vanderbilt-Ingram Cancer Center.1
- 13Memorial Sloan Kettering Cancer Center.1
- 14Fred Hutchinson Cancer Research Center/Seattle Cancer Care Alliance.1
Associated genes
Genes associated with this disease in the canonical knowledge graph (Open Targets evidence). Number shows the association score.
Disease biology
Proteins whose encoding gene is associated with this disease, reached through the canonical gene→disease graph. Number shows the gene's association score.
Related conditions
Diseases frequently studied alongside this one. Number shows shared papers.
Disease profile
A grounded synthesis of the condition — overview, causes, mechanism, risk factors and current standard of care.
Sarcoma is a malignant neoplasm of connective tissue arising from mesodermal cells. It includes a heterogeneous group of tumors that can originate in fat, muscle, nerves, blood vessels, and other connective tissues, and it is usually highly malignant.
The supplied grounding does not identify a specific cause or aetiology for sarcoma. It does indicate that classification and diagnosis are important because sarcomas comprise multiple distinct mesenchymal tumor entities, including some with defined genetic features such as NTRK-rearranged soft tissue tumors.
Sarcoma is a mesenchymal tumor driven by proliferation of connective-tissue-derived cells. The literature grounding emphasizes heterogeneity in clinical and pathologic behavior, the importance of pathologic classification, and the role of genetics in defining entities and guiding treatment; tumor microenvironment is also a co-studied mechanism.
The supplied grounding does not provide specific risk factors for sarcoma. It does support that diagnosis and risk assessment are influenced by tumor subtype and pathologic classification, including risk assessment schemes for some entities such as solitary fibrous tumor.
Management is centered on multidisciplinary evaluation, diagnosis, and treatment guided by pathology and tumor subtype. The grounding supports therapy at the level of surgery and broader treatment planning, with drug therapy used in some settings and classification-informed approaches for entities such as NTRK-rearranged soft tissue tumors.
AI-generated summary grounded in MeSH and 2 peer-reviewed sources. Informational only — not medical advice. Generated 2026-07-07.
Reference
Authoritative identity, definition & identifiers.
A connective tissue neoplasm formed by proliferation of mesodermal cells; it is usually highly malignant.
- Disease identity & definition — NLM Medical Subject Headings (MeSH), public domain
- Clinical trials — ClinicalTrials.gov (U.S. National Library of Medicine)
- Research activity — Europe PMC (EMBL-EBI) + OpenAlex-derived paper links
- Related entities are derived from literature co-mention (studied together) — associative, not causal.