• Neuro-oncology · Feb 2009

    Multicenter Study

    A pilot study of risk-adapted radiotherapy and chemotherapy in patients with supratentorial PNET.

    • Murali Chintagumpala, Tim Hassall, Shawna Palmer, David Ashley, Dana Wallace, Kimberly Kasow, Thomas E Merchant, Matthew J Krasin, Robert Dauser, Frederick Boop, Robert Krance, Shiao Woo, Robyn Cheuk, Chin... more g Lau, Richard Gilbertson, and Amar Gajjar. less
    • Texas Children's Cancer Center, Baylor College of Medicine, Houston, TX 77030, USA. mxchinta@txccc.org
    • Neuro-oncology. 2009 Feb 1; 11 (1): 33-40.

    AbstractWe undertook this study to estimate the event-free survival (EFS) of patients with newly diagnosed supratentorial primitive neuroectodermal tumor (SPNET) treated with risk-adapted craniospinal irradiation (CSI) with additional radiation to the primary tumor site and subsequent high-dose chemotherapy supported by stem cell rescue. Between 1996 and 2003, 16 patients with SPNET were enrolled. High-risk (HR) disease was differentiated from average-risk (AR) disease by the presence of residual tumor (M(0) and tumor size > 1.5 cm(2)) or disseminated disease in the neuraxis (M(1)-M(3)). Patients received risk-adapted CSI: those with AR disease received 23.4 Gy; those with HR disease, 36-39.6 Gy. The tumor bed received a total of 55.8 Gy. Subsequently, all patients received four cycles of high-dose cyclophosphamide, cisplatin, and vincristine with stem cell support. The median age at diagnosis was 7.9 years; eight patients were female. Seven patients had pineal PNET. Twelve patients are alive at a median follow-up of 5.4 years. The 5-year EFS and overall survival (OS) estimates for all patients were 68% +/- 14% and 73% +/- 13%. The 5-year EFS and OS estimates were 75% +/- 17% and 88% +/- 13%, respectively, for the eight patients with AR disease and 60% +/- 19% and 58% +/- 19%, respectively, for the eight with HR disease. No deaths were due to toxicity. High-dose cyclophosphamide-based chemotherapy with stem cell support after risk-adapted CSI results in excellent EFS estimates for patients with newly diagnosed AR SPNET. Further, this chemotherapy allows for a reduction in the dose of CSI used to treat AR SPNET without compromising EFS.

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