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A multimodal strategy based on surgery, radiotherapy, ICE regimen and high dose chemotherapy in atypical teratoid/rhabdoid tumours: a single institution experience

  • Clinical study - patient study
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Abstract

Purpose Atypical Teratoid/Rhabdoid Tumour is a rare and aggressive childhood tumour. The outcome of a series treated with the same multimodal strategy was reported. Patients The patients were treated with surgery, 2 courses of ifosfamide/carboplatin/etoposide(ICE), 2 courses of cyclophosphamide/etoposide/carboplatino/thiotepa (CECAT) or 2 other ICE courses, high dose chemotherapy (HDC) and radiotherapy. Results Eight patients underwent primary surgery achieving a complete removal in 3. Progressive disease (PD) occurred in 2/8 patients during ICE courses and in 3/4 during CECAT courses. After 4 courses 5 patients presented a PD. HDC was performed in 3 patients followed by local radiotherapy. The Kaplan Meier OS and EFS probability at 5 years are, respectively, 50% (CI 11–80%) and 33% (CI 6–66%). Conclusion A strategy based on surgery, including a second surgical look, and on radiotherapy appears the best option. ICE regimen and HDC correlate with good prognosis in some patients but this approach needs further evaluation.

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Acknowledgements

The authors would like to thank G. Izzi MD who referred one patient after conventional chemotherapy and F. Giangaspero MD who reviewed the histology of the same patient. The authors would also like to thank L. Ravà PhD for statistical support and R. Trenchard PhD for editing the manuscript.

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Authors and Affiliations

  1. Division of Pediatric Oncology Unit, Ospedale Pediatrico Bambino Gesù, Piazza S.Onofrio 4, Rome, 00100, Italy

    Paola Fidani, Maria Antonietta De Ioris, Annalisa Serra, Luigi De Sio, Ilaria Ilari, Raffaele Cozza, Giuseppe Maria Milano & Alberto Donfrancesco

  2. Department of Pathology, Ospedale Pediatrico Bambino Gesù, Rome, Italy

    Renata Boldrini

  3. Division of Pediatric Haematology and Oncology, Ospedale G.Gaslini, Genoa, Italy

    Maria Luisa Garrè

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  1. Paola Fidani
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  2. Maria Antonietta De Ioris
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  3. Annalisa Serra
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  10. Alberto Donfrancesco
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Correspondence to Paola Fidani.

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Fidani, P., De Ioris, M.A., Serra, A. et al. A multimodal strategy based on surgery, radiotherapy, ICE regimen and high dose chemotherapy in atypical teratoid/rhabdoid tumours: a single institution experience. J Neurooncol 92, 177–183 (2009). https://doi.org/10.1007/s11060-008-9750-y

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  • DOI: https://doi.org/10.1007/s11060-008-9750-y

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