Regional Nodal Control for Head and Neck Alveolar Rhabdomyosarcoma
Menée sur 14 patients pédiatriques atteints d'un rhabdomyosarcome alvéolaire de la tête et du cou et ayant reçu un traitement systémique en combinaison avec une protonthérapie ciblant le site primitif de la tumeur et les ganglions atteints (durée médiane de survie : 4,3 ans), cette étude analyse la survie sans maladie et la survie globale à 5 ans, puis identifie les formes de récidive
Objectives : To assess clinical outcomes and patterns of failure, particularly regional nodal control, for pediatric patients treated with proton beam therapy (PBT) for head and neck alveolar rhabdomyosarcoma (HN-ARMS). Methods : Between 2006 and 2015, 14 patients with HN-ARMS were enrolled on a prospective registry protocol and treated with PBT at a single institution. Eight patients (57%) presented with localized disease, and 6 (43%) presented with regional nodal metastases. All patients were treated with systemic therapy per accepted cooperative group regimens. All patients received PBT to the primary site and involved nodal disease to a median dose of 50.4Gy(RBE). Elective nodal irradiation (ENI) was not delivered. Results : Median follow-up for surviving patients was 4.3 years. Five-year overall survival and disease-free survival for the cohort (n=14) were 45% and 25%, respectively. There were 10 relapses in the cohort: 7 regional nodal, 1 combination local and regional nodal, and 2 leptomeningeal. Of 8 patients with no nodal disease at diagnosis, 6 developed isolated regional nodal relapse (75%). All nodal relapses occurred in first-echelon draining lymph node basins relative to the primary tumor site. Of 6 patients who presented with nodal metastases, two experienced regional nodal relapse; both of these nodal relapses occurred in the same nodal basin that was initially involved by disease, but was not completely targeted as part of the primary treatment plan. Conclusions : High rates of regional nodal relapse are observed for HN-ARMS patients, including patients with no nodal disease at diagnosis. These data suggest that HN-ARMS patients may benefit from ENI to treat at-risk draining lymph node stations relative to the primary tumor site. We further recommend coverage of the entire nodal level for any sites of initial nodal disease at diagnosis, due to high risk of failure at these sites.
http://www.redjournal.org/article/S0360-3016(18)30124-X/fulltext 2018