Long-Term Clinical Outcome and Quality of Life of Children, Adolescents, and Young Adults 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: A total of 76 C-AYAs treated between 1999 and 2023 were included. Median age was 16.6 years (1.5-25.4). A total of 44 (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). A total of 26 (34.2%) patients underwent complete resection. PEDQOL questionnaire was used to assess QoL. Kaplan-Meier and log-rank tests 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, young age, and lack of surgical resection were associated with worse prognosis.