Purpose/Objective(s): ATRT is a rare and aggressive CNS tumor usually presenting in very young children. Aggressive treatments have improved outcomes. Such strategies have included radiation therapy. However, at such a young age, short and long term radiation toxicities are prevalent. We prospectively enrolled pediatric CNS ATRT patients onto the Proton Collaborative Group registry protocol to evaluate the efficacy and toxicities of proton radiation therapy in this population.Materials/Methods: 12 consecutive pediatric ATRT patients were treated with at the Central DuPage Hospital Proton Center and the Oklahoma City Procure Proton Therapy Center between March 2010 - December 2013 utilizing 3D Conformal Proton Therapy.Results: 12 patients were evaluated. They were all under 3 years of age. Six patients had gross total resections, while 4 had subtotal resections along with another 2 not documented. All patients received multiagent intensive chemotherapy while one had stem cell transplant as part of his regimen. Radiation was to local fields for 9 patients, while 3 had craniospinal irradiation. The mean follow up was 11.9 months (range of 1-43 months). At last follow up 10 patients were alive without evidence of disease. Only 4 children had grade 3 toxicities (all acute nausea, vomiting and anorexia during radiation therapy that responded to steroids). Proton therapy was able to reduce the dose to the cochlea, optic chiasm, hippocampus, temporal lobes and integral whole brain.Conclusion: The initial results on the largest prospective series of CNS ATRT patients treated with proton therapy seem to be favorable. The aggressive treatment regimens utilizing proton beam therapy yield proven efficacy and improved toxicity profiles, which is critically important in this young patient population with such an aggressive disease.
Chang, J,
Prospective Study on Pediatric Patients with Atypical Teratoid Rhabdoid Tumors (ATRT) of the Central Nervous System (CNS). Radiological Society of North America 2014 Scientific Assembly and Annual Meeting, - ,Chicago IL.
http://archive.rsna.org/2014/14042662.html
