Rhabdomyosarcoma: Prognosis and Prognostic Factors Influencing Survival in Pediatric Soft Tissue Sarcomas

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in childhood, representing a significant challenge in pediatric oncology. Despite advances in treatment, the rhabdomyosarcoma prognosis remains variable, depending on multiple factors that influence the life expectancy of patients with soft tissue sarcoma. This article explores key prognostic factors and emerging strategies that could improve pediatric tumor survival.
Prognostic Factors and Treatment Strategies
The prognosis of rhabdomyosarcoma is influenced by several factors, including histological subtype, the presence of genetic fusions, and the extent of disease at the time of diagnosis. The main histological subtypes are embryonal rhabdomyosarcoma (eRMS) and alveolar rhabdomyosarcoma (aRMS), with the latter being associated with a more unfavorable prognosis due to the presence of characteristic chromosomal translocations such as PAX3::FOXO1 or PAX7::FOXO1 [1].
The survival of pediatric tumors is also affected by age at diagnosis, tumor size, and the presence of metastasis. Recent studies have shown that mutations in genes such as TP53 and RAS are associated with poorer clinical outcomes, suggesting that these genetic markers could be useful for risk stratification [2].
Regarding treatment strategies, maintenance chemotherapy has been shown to improve survival in patients with high-risk rhabdomyosarcoma, although toxicity remains a challenge [3]. Additionally, immunotherapy and nanomedicine are emerging as promising approaches to treat rhabdomyosarcoma with fewer long-term side effects [1].
Conclusions
The prognosis of rhabdomyosarcoma is complex and depends on a variety of clinical and genetic factors. Identifying genetic markers and implementing personalized treatments are essential to improve the life expectancy of patients with soft tissue sarcoma. As research advances, it is crucial to integrate new findings into clinical practice to optimize outcomes in this vulnerable population.
Referencias
- [1] Targeted immunotherapy and nanomedicine for rhabdomyosarcoma: The way of the future
- [2] Genomic Classification and Clinical Outcome in Rhabdomyosarcoma: A Report From an International Consortium
- [3] Vinorelbine and continuous low-dose cyclophosphamide as maintenance chemotherapy in patients with high-risk rhabdomyosarcoma (RMS 2005): a multicentre, open-label, randomised, phase 3 trial
Created 14/1/2025