Long-Term Clinical Outcome and QoL of children and AYA with Chordoma or Chondrosarcoma treated with Pencil Beam Scanning Proton Therapy

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Background: and aims: To assess clinical outcomes and quality of life (QoL) of children, adolescents, and young adults (C-AYAs) with chordomas (CH) or chondrosarcomas (CS) after pencil beam scanning proton therapy (pbsPT). Methods: : Seventy-six C-AYAs treated between 1999-2023 were included. Median age was 16.6 years (1.5-25.4). Forty-four (57.9%) were AYAs (15-25 years). Most tumors were CHs (n=49; 64.5%) and located in the skull-base (68.4%). Median radiation dose was 73.8 Gy (RBE) (54-75.6). Twenty-six (34.2%) patients underwent complete resection. PEDQOL questionnaire was used to assess QoL. Kaplan–Meier and log-rank test were used for the analysis. Results: : With a median follow-up of 88.4 months (range, 1.8 – 257.6), 9 (11.8%) patients died. Local failure only developed in 9 (11.8%) patients. Two (2.6%) presented with distant failure only and another (1.3%) had both. Seven-years overall survival (OS), local control (LC), and distant control (DC) were 86.3%, 87.7% and 95.7%, respectively. Children when compared to AYAs had worse 7-years DC (90% vs. 100%, p=0.049). Surgical resection vs. biopsy was associated with better 7-years LC (87.1% vs. 67.7%, p=0.031). Recurrent tumors showed worse OS, LC, and DC (94.5% vs. 44.6%, p<0.001; 93.2% vs. 36.6%, p<0.001; 98.3% vs 77.6%, p=0.003). Seven-years freedom from grade ≥3 late toxicity was 83.1%. Three (3.9%) children with CH developed secondary tumors. QoL did not differ from a healthy cohort 2 years after pbsPT. Conclusions: : Excellent clinical outcomes with acceptable long-term toxicity and QoL were observed for C-AYAs with CH/CS after pbsPT. Recurrent tumors, children, and lack of surgical resection was associated with worse prognosis.
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Long-Term Clinical Outcome and QoL of children and AYA with Chordoma or Chondrosarcoma treated with Pencil Beam Scanning Proton Therapy | Authorea try { document.documentElement.classList.add('js'); } catch (e) { } var _gaq = _gaq || []; _gaq.push(['_setAccount', 'G-8VDV14Y67G']); _gaq.push(['_trackPageview']); (function() { var ga = document.createElement('script'); ga.type = 'text/javascript'; ga.async = true; ga.src = ('https:' == document.location.protocol ? 'https://ssl' : 'http://www') + '.google-analytics.com/ga.js'; var s = document.getElementsByTagName('script')[0]; s.parentNode.insertBefore(ga, s); })(); Skip to main content Preprints Collections Wiley Open Research IET Open Research Ecological Society of Japan All Collections About About Authorea FAQs Contact Us Quick Search anywhere Search for preprint articles, keywords, etc. Search Search ADVANCED SEARCH SCROLL Pediatric Blood & Cancer This is a preprint and has not been peer reviewed. Data may be preliminary. 4 March 2025 V1 Latest version Share on Long-Term Clinical Outcome and QoL of children and AYA with Chordoma or Chondrosarcoma treated with Pencil Beam Scanning Proton Therapy Authors : Miriam Vazquez , Alexey Cherchik , Claudio de Angelis , Alessia Pica , Gabriele Calaminus , and Damien Weber 0000-0003-1166-8236 [email protected] Authors Info & Affiliations https://doi.org/10.22541/au.174105731.12389938/v1 345 views 190 downloads Contents Abstract Supplementary Material Information & Authors Metrics & Citations View Options References Figures Tables Media Share Abstract Background and aims: To assess clinical outcomes and quality of life (QoL) of children, adolescents, and young adults (C-AYAs) with chordomas (CH) or chondrosarcomas (CS) after pencil beam scanning proton therapy (pbsPT). Methods: Seventy-six C-AYAs treated between 1999-2023 were included. Median age was 16.6 years (1.5-25.4). Forty-four (57.9%) were AYAs (15-25 years). Most tumors were CHs (n=49; 64.5%) and located in the skull-base (68.4%). Median radiation dose was 73.8 Gy (RBE) (54-75.6). Twenty-six (34.2%) patients underwent complete resection. PEDQOL questionnaire was used to assess QoL. Kaplan–Meier and log-rank test were used for the analysis. Results : With a median follow-up of 88.4 months (range, 1.8 – 257.6), 9 (11.8%) patients died. Local failure only developed in 9 (11.8%) patients. Two (2.6%) presented with distant failure only and another (1.3%) had both. Seven-years overall survival (OS), local control (LC), and distant control (DC) were 86.3%, 87.7% and 95.7%, respectively. Children when compared to AYAs had worse 7-years DC (90% vs. 100%, p=0.049). Surgical resection vs. biopsy was associated with better 7-years LC (87.1% vs. 67.7%, p=0.031). Recurrent tumors showed worse OS, LC, and DC (94.5% vs. 44.6%, p<0.001; 93.2% vs. 36.6%, p<0.001; 98.3% vs 77.6%, p=0.003). Seven-years freedom from grade ≥3 late toxicity was 83.1%. Three (3.9%) children with CH developed secondary tumors. QoL did not differ from a healthy cohort 2 years after pbsPT. Conclusions: Excellent clinical outcomes with acceptable long-term toxicity and QoL were observed for C-AYAs with CH/CS after pbsPT. Recurrent tumors, children, and lack of surgical resection was associated with worse prognosis. Supplementary Material File (figures.docx) Download 5.18 MB File (manuscript.docx) Download 5.44 MB File (tables.docx) Download 5.25 MB Information & Authors Information Version history V1 Version 1 04 March 2025 Copyright This work is licensed under a Non Exclusive No Reuse License. Collection Pediatric Blood & Cancer Keywords pediatric oncology quality of life radiation therapy sarcomas Authors Affiliations Miriam Vazquez Paul Scherrer Institut Zentrum fur Protonentherapie View all articles by this author Alexey Cherchik Paul Scherrer Institut Zentrum fur Protonentherapie View all articles by this author Claudio de Angelis Paul Scherrer Institut Zentrum fur Protonentherapie View all articles by this author Alessia Pica Paul Scherrer Institut Zentrum fur Protonentherapie View all articles by this author Gabriele Calaminus University Hospital Bonn View all articles by this author Damien Weber 0000-0003-1166-8236 [email protected] Paul Scherrer Institut Zentrum fur Protonentherapie View all articles by this author Metrics & Citations Metrics Article Usage 345 views 190 downloads .FvxKWukQNSOunydq8rnd { width: 100px; } Citations Download citation Miriam Vazquez, Alexey Cherchik, Claudio de Angelis, et al. Long-Term Clinical Outcome and QoL of children and AYA with Chordoma or Chondrosarcoma treated with Pencil Beam Scanning Proton Therapy. Authorea . 04 March 2025. DOI: https://doi.org/10.22541/au.174105731.12389938/v1 If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. Simply select your manager software from the list below and click Download. For more information or tips please see 'Downloading to a citation manager' in the Help menu . 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