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jdetyy
15 hours, 33 minutes ago
Edited
public
Rhabdomyosarcoma: Precision Oncology in Pediatric Sarcomas
https://jdetyy.com/disease/rhabdomyosarcoma-precision-oncology-in-pediatric-sarcomas.html
#canine
#rhabdomyosarcoma
I. Disease Profile & Epidemiology
1. Pathological Features
Origin: Embryonal mesenchymal-derived sarcoma mimicking immature skeletal muscle.
Molecular Subtypes:
Embryonal (80%): Favorable prognosis; head/neck, genitourinary sites.
Alveolar (15-20%): PAX3/7-FOXO1 fusions (t(2;13)/t(1;13)); poor outcome.
Pleomorphic...
more
Rhabdomyosarcoma: Precision Oncology in Pediatric Sarcomas
https://jdetyy.com/disease/rhabdomyosarcoma-precision-oncology-in-pediatric-sarcomas.html
#canine
#rhabdomyosarcoma
I. Disease Profile & Epidemiology
1. Pathological Features
Origin: Embryonal mesenchymal-derived sarcoma mimicking immature skeletal muscle.
Molecular Subtypes:
Embryonal (80%): Favorable prognosis; head/neck, genitourinary sites.
Alveolar (15-20%): PAX3/7-FOXO1 fusions (t(2;13)/t(1;13)); poor outcome.
Pleomorphic/Spindle Cell: Rare; adult-predominant.
Genetic Predisposition: Li-Fraumeni (TP53), NF1 (↑5-fold risk).
2. Epidemiological Data
Alert: 5-year survival <30% for alveolar/metastatic RMS.
II. Diagnostic Innovations: From Morphology to Molecular Stratification
1. Multimodal Diagnostic Pathway
2. Core Diagnostic Tools
Imaging:
Head/neck: MRI (↑30% soft-tissue resolution).
Whole-body: PET-CT sensitivity 92% (occult metastasis detection).
Pathology Gold Standards:
Biopsy + IHC (Myogenin+ >95%).
Molecular confirmation of PAX-FOXO1 fusion dictates therapy intensity.
Metastasis Screening:
Bone marrow biopsy + LP (40% positive in alveolar).
ctDNA monitoring (sensitivity 88%).
3. Risk Stratification (2025 European Guidelines)
III. Risk-Adapted Therapy: From Chemo to Immunotherapy
1. Multimodal Treatment
2. Risk-Tailored Intensification
Low-risk:
VAC ×1yr vs VA: 5-yr EFS 83% vs 76% (P=0.18).
Intermediate/High-risk:
Anthracycline-based (VAC+Doxorubicin): EFS ↑15%.
Maintenance (Vinorelbine+Cyclophosphamide ×12mo): ↓Relapse 32%.
Alveolar (Fusion+):
PAX-FOXO1 Targeting: MDM2 inhibitors (e.g., APG-115) + chemo → ORR ↑58%.
3. Relapsed/Refractory RMS Breakthroughs
IV. Survivorship & Late Effects
1. Acute Toxicity Management
2. Long-Term Surveillance
Secondary Malignancies: ↑8-fold sarcoma risk in RT fields.
Fertility Preservation: Gonadal shielding → fertility >70%.
Functional Outcomes: Limb salvage >90% (neoadjuvant chemo + precision surgery).
V. Future Directions
1. Overcoming Resistance
ctDNA Monitoring: Predicts resistance (sensitivity 92%).
Epigenetic Modulation: HDAC inhibitors (Chidamide) reverse chemo-resistance.
2. Global Equity Initiatives
Africa-Adapted Protocol: Dexamethasone + Cyclophosphamide ($<300), survival ↑11%→39%.
AI-Assisted Diagnosis: Smartphone microscopy ↓misdiagnosis 60% in low-resource settings.
3. Clinical Trial Highlights
Conclusion: From Survival to Functional Cure
RMS management has achieved:
1. Molecular Stratification: PAX-FOXO1 status guides precision therapy (↑survival 3-fold in high-risk).
2. Function-Preserving Approaches: Brachytherapy/limb salvage → function retention >80%.
3. Global Standardization: European guidelines unify care; 5-yr OS reaches 74.3%.
Future Imperatives: Targeting clonal evolution, reducing toxicity, and expanding global access to achieve "cure without disability."
Data Sources:
European RMS Guidelines (2022) | IRS-V Trial | PARP inhibitor studies | Global equity programs.
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