Long-term survival following additive radiotherapy in patients with atypical teratoid rhabdoid tumors

摘要: Atypical teratoid rhabdoid tumor (ATRT) is a highly aggressive disease of embryonic origin accounting for <5% all pediatric central nervous system (CNS) tumors. We describe a series five cases CNS ATRT. The first three patients underwent subtotal resection. Gross total resection the was achieved in fourth and fifth patients. Only 4 patients received chemotherapy, whereas 5 patients additive radiotherapy (RT). latter included dimensional (3D) conformal RT or intensity modulated (IMRT) with a median dose 54 Gy (range 50.4–59.0 Gy) applied daily fractions 1.8 Gy. median interval between surgery 5 months 2–11 months). Two months after completion RT, had complete radiologic remission. event-free survival period 46 months 10–90 months). However, patient died 17 months developing an out-of-field recurrence. third developed a recurrence 11 months salvage RT. other 3 patients (cases 2, 4, 5) remain alive no evidence disease 59, 46 90 months therapy, respectively. Overall, survived 48 months 25–90 months) from time initial diagnosis they tolerated well, without severe acute late onset toxicities. results imply a potential gain irradiation at acceptable toxicity level.