Study finds age-related survival disparities in patients with sarcoma
Adolescents with a diagnosis of bone or soft-tissue sarcoma had notably poorer outcomes compared to their younger counterparts for certain subtypes of sarcoma, according to a study. Specifically, undifferentiated small round cell sarcoma (USRCS), alveolar rhabdomyosarcoma (ARMS), and epithelioid sarcoma exhibited worse prognoses among adolescents.
The retrospective study analyzed data from the National Registry of Childhood Cancers Database, encompassing 1637 patients aged 0 to 17 years with a diagnosis of bone or soft-tissue sarcoma. The cohort consisted of children (0-9 years) comprising 40% of the sample, preadolescents (10-14 years) representing 35%, and adolescents (15-17 years) accounting for 25%.
An analysis of the genetic characteristics of the tumors revealed significant differences in survival rates within specific sarcoma subtypes. Adolescents with CIC-rearranged USRCS had a five-year overall survival rate of 47% (95%CI: 21-69), significantly lower than other USRCS cases. Similarly, PAX3::FOXO1-positive ARMS patients experienced a 5-year overall survival rate of 44% (95%CI: 32-55), also lower than other ARMS cases. The prevalence of PAX3::FOXO1 increased significantly with age among patients with ARMS.
After adjusting for stage and genomic-profiling status, it was determined that age was an independent prognostic factor of outcome only in patients with USRCS. Adolescents diagnosed with USRCS were 1.6 times more likely to die compared to children with the same condition. The association between age and survival of patients with ARMS, however, could be partly attributed to the differences in the prevalence of PAX3::FOXO1.
Reference
Desandes E, Lapouble E, Lacour B, et al. Impact of age on survival according to molecular tumor findings in children and adolescents with soft-tissue and bone sarcoma: The BIOSCA project. Cancer Epidemiol. 2023;102398. doi: 10.1016/j.canep.2023.102398. Epub ahead of print. PMID: 37357067.