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

Journal of Neuro-Oncology (Impact Factor: 3.07). 04/2009; 92(2):177-183. DOI: 10.1007/s11060-008-9750-y


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 5years 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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Available from: Giuseppe Maria Milano, Jun 13, 2014
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    • "Although this analysis may show a possible benefit to the use of methotrexate and further supports better outcomes with more complete resection, the 3-year estimated EFS is comparable to that achieved with other therapeutic approaches. A group from Italy reported eight patients treated on a clinical trial that included radiotherapy; ifosfamide, carboplatin, and etoposide (ICE); and HDC (Fidani et al., 2009). They had originally included cyclophosphamide, etoposide, carboplatin, and thiotepa, but after three of four patients had progressive disease on this therapy, they decided to exclude these courses and replace them with additional ICE. "
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