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J Neurooncol (2015) 121:389397 DOI 10.1007/s11060-014-1648-2
CLINICAL STUDY
Tumor control and QoL outcomes of very young childrenwith atypical teratoid/rhabdoid Tumor treated with focalonly chemo-radiation therapy using pencil beam scanning proton therapy
Damien C. Weber Carmen Ares Robert Malyapa Francesca Albertini
Gabriele Calaminus Ulrike Kliebsch Lorentzos Mikroutsikos
Petra Morach Alessandra Bolsi Tony Lomax Ralf Schneider
Received: 23 June 2014 / Accepted: 26 October 2014 / Published online: 2 November 2014 Springer Science+Business Media New York 2014
Abstract The aim of this analysis was to assess the early clinical results of pencil beam scanning proton therapy (PT) in the treatment of young children with non-metastatic atypical teratoid/rhabdoid tumor (ATRT) of the CNS. Fifteen children (male, n = 8, 53 %) were treated with PT between May 2008 and January 2013. Mean age at diagnosis was 17.4 7.0 months. The localization was infra-tentorial in 9 (60 %) patients. Gross total resection of the primary tumors was achieved in 7 (47 %) patients. The dose administered focally under sedation was 54 Gy (RBE). After a median follow-up of 33.4 months (range
9.769.2), 3 (20 %), 4 (27 %) and 2 (13 %) patients presented with local failure (LF), distant brain failure (DBF) and spinal failure (SF), respectively. Six patients died, all of tumor progression. The 2-year overall- and progression-free survival was 64.6 and 66.0 %. Tumor location (supratentorial) and the extent of surgical resection (non-gross total resection) were negative prognostic factors for both OS and PFS. PT was well tolerated. No grade [2 acute toxicity was observed. The estimated 2-year toxicity-free survival was 90 %. As assessed by the PedsQoL proxy, no decrease in QoL was observed after PT. We conclude that PBS PT is an effective treatment for young children with ATRT. After PT, with or without concomitant chemotherapy, two third of the patients survived [2 years. Acute toxicity was manageable. Longer follow-
This work has been submitted for oral presentation to the 2014 SIOP meeting, Toronto, Canada.
D. C. Weber (&) R. Malyapa F. Albertini U. Kliebsch
L. Mikroutsikos P. Morach A. Bolsi T. Lomax
R. SchneiderCenter for Proton Therapy, Paul Scherrer Institute, WPTA 144, CH-5232 Villigen West, Switzerlande-mail: [email protected]; [email protected]
R. Malyapae-mail: [email protected]
F. Albertinie-mail: [email protected]
U. Kliebsche-mail: [email protected]
L. Mikroutsikose-mail: [email protected]
P. Morache-mail: [email protected]
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