TY - JOUR
T1 - A pilot study of risk-adapted radiotherapy and chemotherapy in patients with supratentorial PNET
AU - Chintagumpala, Murali
AU - Hassall, Tim
AU - Palmer, Shawna
AU - Ashley, David
AU - Wallace, Dana
AU - Kasow, Kimberly
AU - Merchant, Thomas E.
AU - Krasin, Matthew J.
AU - Dauser, Robert
AU - Boop, Frederick
AU - Krance, Robert
AU - Woo, Shiao
AU - Cheuk, Robyn
AU - Lau, Ching
AU - Gilbertson, Richard
AU - Gajjar, Amar
N1 - Copyright:
Copyright 2009 Elsevier B.V., All rights reserved.
PY - 2009/2
Y1 - 2009/2
N2 - We undertook this study to estimate the event-free survival (EFS) of patients with newly diagnosed supratento-rial 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 (M0 and tumor size > 1.5 cm2) or disseminated disease in the neuraxis (M1-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.
AB - We undertook this study to estimate the event-free survival (EFS) of patients with newly diagnosed supratento-rial 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 (M0 and tumor size > 1.5 cm2) or disseminated disease in the neuraxis (M1-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.
KW - Autologous stem cell rescue
KW - Craniospinal radiotherapy
KW - Dose-intensive chemotherapy
KW - Event-free survival
KW - Risk-adapted therapy
KW - Supratentorial PNET
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UR - http://www.scopus.com/inward/citedby.url?scp=61449248205&partnerID=8YFLogxK
U2 - 10.1215/15228517-2008-079
DO - 10.1215/15228517-2008-079
M3 - Article
C2 - 18796696
AN - SCOPUS:61449248205
VL - 11
SP - 33
EP - 40
JO - Neuro-oncology
JF - Neuro-oncology
SN - 1522-8517
IS - 1
ER -