Proton-Based Radiation Therapy for Skull Base Chondrosarcomas in Children and Adolescents: 40-Year Experience From the Massachusetts General Hospital.

Abstract

The literature on skull base chondrosarcoma (CHS) is scarce. We report outcomes for pediatric and young patients ≤22 years with base of skull CHSs treated with proton-based radiation therapy (PBRT). We retrospectively reviewed all patients treated with PBRT between 1981 and 2023. Primary endpoints were overall survival (OS), chondrosarcoma-specific survival (CSS), progression-free survival (PFS), local control, and distal control. Eighty-four patients were identified. Median age at diagnosis was 19 years (range, 6-22). Most patients (n = 79, 94%) had conventional CHS, whereas 5 (6%) had mesenchymal CHS. Nine patients (11%) underwent gross total resection, 64 (76%) subtotal resection, and 11 patients (13%) underwent biopsy. Twenty-four (29%) patients progressed before radiation therapy. The median prescription dose was 70 Gy (relative biological equivalent) (range, 50-79.7). At a median follow-up of 18.0 years (IQR, 9.2-26.2) from diagnosis, 11 patients recurred (7 local, 4 distant). Six patients died of disease. Five patients died of other causes. Ten-year OS, CSS, and PFS rates for all patients were 93.3%, 94.7%, and 88%, respectively. Twenty-year OS, CSS, and PFS rates for conventional CHS (n = 79) were 93.1%, 97.1%, and 89.2%, respectively. Mesenchymal histology was significantly associated with worse OS and PFS. Preradiation therapy tumor progression portended worse OS and CSS. Eight patients (9.9 %) experienced a late toxicity grade 3 or greater. This is the largest cohort of pediatric base of skull CHSs in literature to date. High-dose PBRT after surgical resection achieves excellent disease control with minimal toxicity.