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An aggressive malignant mesenchymal neoplasm with skeletal muscle differentiation, occurring in adults and rarely in children. The tumor is characterized by the presence of bizarre round, spindle, and polygonal cells. Clinical presentation includes a rapidly enlarging painful mass usually of the lower extremities.
Biomarker and diagnostic research for pleomorphic rhabdomyosarcoma has been reported in the published literature.
Estimated prevalence: <1 in 1,000,000 (VERY_RARE).
5 clinical trials registered, 3 recruiting. Interventions under study include procedural interventions, other interventions, biologic therapy, and drug therapy. Pipeline includes 3 PHASE2, 1 PHASE1, 1 NA. Research is sponsored by a mix of industry and academic institutions.
NCT ID | Title | Phase | Sponsor | Status |
|---|---|---|---|---|
[NCT07173972](https://clinicaltrials.gov/study/NCT07173972) |
Data assembled from 4 of 12 sources · Last updated Sep 20, 2026, 3:01 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Dose-escalated, Hypofractionated, Definitive Proton Radiotherapy for Patients With Inoperable Soft Tissue Sarcoma. |
PHASE2 |
Oslo University Hospital |
RECRUITING |
[NCT06526897](https://clinicaltrials.gov/study/NCT06526897) | Evaluation of Chest CT Versus Chest X-Ray for Lung Surveillance After Curative-Intent Resection of High-Risk Truncal-Extremity Soft Tissue Sarcoma | NA | ECOG-ACRIN Cancer Research Group | NOT_YET_RECRUITING |
[NCT06277154](https://clinicaltrials.gov/study/NCT06277154) | MASCT-I Combined With Doxorubicin and Ifosfamide for First-line Treatment of Advanced Soft Tissue Sarcoma | PHASE2 | HRYZ Biotech Co. | RECRUITING |
[NCT07169344](https://clinicaltrials.gov/study/NCT07169344) | Hypofractionated, 3-week, Preoperative Proton or X-ray Radiotherapy for Patients With Localized Soft Tissue Sarcoma | PHASE2 | Oslo University Hospital | RECRUITING |
[NCT04420975](https://clinicaltrials.gov/study/NCT04420975) | Nivolumab and BO-112 Before Surgery for the Treatment of Resectable Soft Tissue Sarcoma | PHASE1 | Jonsson Comprehensive Cancer Center | ACTIVE_NOT_RECRUITING |
34 publications have been identified in PubMed for pleomorphic rhabdomyosarcoma. Research spans Case Report / Case Series (68%), Basic Science / Preclinical (15%), and Diagnostic / Biomarker (6%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 23 | 68% |
Laboratory research | 5 | 15% |
Testing and diagnosis research | 2 | 6% |
Clinical study results | 2 | 6% |
Research summaries | 1 | 3% |
Disease patterns and progression | 1 | 3% |
Baldi GG (2026). [PMID: 41819027](https://pubmed.ncbi.nlm.nih.gov/41819027/). *Eur J Cancer*. [Case Report / Case Series]
Odintsov I (2026). [PMID: 42281350](https://pubmed.ncbi.nlm.nih.gov/42281350/). *Am J Surg Pathol*. [Diagnostic / Biomarker]
Giani C (2026). [PMID: 41564817](https://pubmed.ncbi.nlm.nih.gov/41564817/). *ESMO Open*. [Clinical Trial Publication]
Gunasekaran K (2026). [PMID: 41728531](https://pubmed.ncbi.nlm.nih.gov/41728531/). *Cureus*. [Case Report / Case Series]
Ma JQ (2026). [PMID: 41607761](https://pubmed.ncbi.nlm.nih.gov/41607761/). *World J Gastrointest Oncol*. [Case Report / Case Series]
Ulici V (2026). [PMID: 41780801](https://pubmed.ncbi.nlm.nih.gov/41780801/). *Mod Pathol*. [Basic Science / Preclinical]
Cai H (2025). [PMID: 37715691](https://pubmed.ncbi.nlm.nih.gov/37715691/). *Ear Nose Throat J*. [Case Report / Case Series]
Kopantseva EE (2025). [PMID: 41373578](https://pubmed.ncbi.nlm.nih.gov/41373578/). *Int J Mol Sci*. [Case Report / Case Series]
Stanton J (2025). [PMID: 41245819](https://pubmed.ncbi.nlm.nih.gov/41245819/). *J Surg Case Rep*. [Case Report / Case Series]
Zieba D (2025). [PMID: 39660950](https://pubmed.ncbi.nlm.nih.gov/39660950/). *Am J Dermatopathol*. [Case Report / Case Series]
AI-curated news mentioning pleomorphic rhabdomyosarcoma
Updated Jun 12, 2026
A study of 39 mismatch repair-deficient sarcomas reveals recurrent histologic patterns, advocating for universal screening of pleomorphic rhabdomyosarcoma, uterine leiomyosarcoma, and undifferentiated and unclassified sarcomas. This research could influence future diagnostic and treatment strategies.
A retrospective study from the PUSH consortium evaluates outcomes in patients with advanced pleomorphic rhabdomyosarcoma treated with systemic agents. The findings contribute to understanding treatment efficacy in this ultra-rare sarcoma subtype.