© Copyright 2008 by the Society for Neuro-Oncology
Received November 21, 2007
Accepted March 25, 2008
A pilot study of risk adapted radiotherapy and chemotherapy in patients with supratentorial PNET
Murali Chintagumpala 1*,
Tim Hassall 2,
Shawna Palmer 3,
David Ashley 4,
Dana Wallace 5,
Kimberly Kasow 6,
Thomas E. Merchant 7,
Matthew J. Krasin 7,
Robert Dauser 8,
Frederick Boop 9,
Robert Krance 10,
Shiao Woo 11,
Robyn Cheuk 2,
Ching Lau 10,
Richard Gilbertson 12,
Amar Gajjar 13
1 Texas Children's Cancer Center, Baylor College of Medicine, Houston TX
2 Royal Children's Hospital, Department of Haematology/Oncology, Herston, Brisbane, Australia
3 Department of Oncology; Division of Behavioral Medicine, St. Jude Children's Research Hospital, Memphis, TN
4 Royal Children's Hospital, Department of Clinical Haematology and Oncology, Parkville, Australia
5 Department of Biostatistics, St. Jude Children's Research Hospital, Memphis, TN
6 Division of Bone Marrow Transplantation, St. Jude Children's Research Hospital, Memphis, TN
7 Division of Radiation Oncology, St. Jude Children's Research Hospital, Memphis, TN
8 Department of Pediatric Neurosurgery, Texas Children's Hospital, Houston, TX
9 Department of Radiological Sciences; Division of Neurosurgery, St. Jude Children's Research Hospital, Memphis, TN
10 Texas Children's Cancer Center, Houston, TX
11 M. D. Anderson Cancer Center, Radiation Oncology, Houston, TX
12 Department of Developmental Neurobiology, St. Jude Children's Research Hospital, Memphis, TN
13 Division of Neuro-Oncology, St. Jude Children's Research Hospital, Memphis, TN
* To whom correspondence should be addressed. E-mail: mxchinta{at}txccc.org.
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Abstract |
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Purpose: To estimate the 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.
Patients and Methods: 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 (M1-3). Patients received risk-adapted CSI: those with AR disease received 23.4 Gy; those with HR disease, 36 to 39.6 Gy. The tumor bed received a total of 55.8 Gy. Subsequently all patients received 4 cycles of high-dose cyclophosphamide, cisplatin and vincristine with stem cell support.
Results: The median age at diagnosis was 7.9 years; 8 patients were female. Seven patients had pineal PNET. Twelve patients are alive at a median follow-up of 5.4 years. The 5-year event-free survival (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 8 patients with AR disease and 60% ± 19% and 58%±19%, respectively, for the 8 with HR disease. No deaths were due to toxicity.
Conclusions: 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.
Key Words:
Supratentorial PNET, risk-adapted therapy, autologous stem cell rescue, event-free survival, dose-intensive chemotherapy, craniospinal radiotherapy
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