Impact of age on survival according to molecular tumor findings in children and adolescents with soft-tissue and bone sarcoma: The BIOSCA project

• Published in: Cancer epidemiology Vol. 92; p. 102398
• Main Authors: Desandes, Emmanuel, Lapouble, Eve, Lacour, Brigitte, Guissou, Sandra, Goujon, Stéphanie, Defachelles, Anne-Sophie, Marechal, Valérie, Gaspar, Nathalie, Gomez-Mascard, Anne, Karanian, Marie, Marec-Berard, Perrine, Minard-Colin, Véronique, Orbach, Daniel, Tabone, Marie-Dominique, Delattre, Olivier, Pierron, Gaelle
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Ltd 01-10-2024; Elsevier Limited
• Subjects: Adolescent; Adolescents; Age; Age Factors; Age groups; Bone cancer; Bone Neoplasms - genetics; Bone Neoplasms - mortality; Bone Neoplasms - pathology; Bone tumors; Child; Child, Preschool; Children; Classification; Diagnosis; Ewings sarcoma; Female; Forkhead Box Protein O1 - genetics; Forkhead Box Protein O1 - metabolism; FOXO1 protein; Genetic analysis; Humans; Infant; Infant, Newborn; Male; Medical prognosis; Medical records; Metastasis; Molecular biology; Molecular biomarker; Patients; Pax3 protein; Pediatrics; Population genetics; Population studies; Prognosis; Retrospective Studies; Rhabdomyosarcoma; Sarcoma; Sarcoma - genetics; Sarcoma - mortality; Sarcoma - pathology; Soft Tissue Neoplasms - genetics; Soft Tissue Neoplasms - mortality; Soft Tissue Neoplasms - pathology; Survival; Survival analysis; Survival Rate; Teenagers; Tumors; Young adults; France; BIOSCA; ICCC-3; LGFMS; ARMS; AYA; Molecular biomarker; MPNST; FaR-RMS; SEER; Children; Adolescents; CI95; US; FDFSP; WHO; EWS; SS; OS; Sarcoma; RNIPP; ICDO-3; ASPS; Survival; ES; USRCS; DSRCT; ERMS; RNCE; EpSSG
• ISSN: 1877-7821; 1877-783X; 1877-783X
• DOI: 10.1016/j.canep.2023.102398

Adolescents (15–19 years) with sarcoma are known to have significantly worse survival than children (0–14 years). One possible reason may be that the adolescent sarcomas exhibit specific biological characteristics resulting in differences in clinical presentation and treatment resistance behaviors. The BIOSCA project aims to further explore these age-related differences in survival accounting for molecular tumor characteristic in children and adolescents with sarcoma.
A retrospective national population-based observational study with documented somatic genetic analyses was conducted between 2011 and 2016 of all patients aged from 0 to 17 years with a diagnosis of sarcoma using the National Registry of Childhood Cancers Database.
A total of 1637 children (0–9years: 40%), preadolescents (10–14years: 35%) and adolescents (15–17 years: 25%) with a diagnosis of bone (N = 845) or soft-tissue (N = 792) sarcoma were included. Adolescents had significantly worse outcome for undifferentiated small round cell sarcoma (USRCS), alveolar rhabdomyosarcoma (ARMS), and epithelioid sarcoma. Five-year overall survivals were worse among CIC-rearranged USRCS cases (47% [95%CI:21–69]) as compared to other USRCS, and PAX3::FOXO1 ARMS patients (44% [95%CI:32–55]) as compared to other ARMS. Adjusting for stage and genomic-profiling status, adolescents with USRCS were 1.6-fold more likely to die than children (P = 0.05), while the difference in survival between age of ARMS patients was weaken. Indeed, the prevalence of PAX3::FOXO1 increased significantly with age.
Age was an independent prognostic factor of outcome only in patients with USRCS, while the association between age and survival of patients with ARMS could be partly explained by differences in prevalence of PAX3::FOXO1.
•Adolescents had worse outcome for undifferentiated small round cell sarcoma (USRCS) and alveolar rhabdomyosarcoma (ARMS).•5yOS was worse among CIC-rearranged USRCS cases (47% [21–69]) as compared to other USRCS.•5yOS was worse among PAX3::FOXO1 ARMS patients (44% [32–55]) as compared to PAX7::FOXO1 ARMS cases (88% [61–97]).•Age was an independent prognostic factor of outcome only in patients with USRCS.•Association between age and survival of patients with ARMS could be explained by prevalence of PAX3::FOXO1.