A pilot study of risk-adapted radiotherapy and chemotherapy in patients with supratentorial PNET.
We 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.
Chintagumpala
Murali
M
Texas Children's Cancer Center, Baylor College of Medicine, Houston, TX 77030, USA. mxchinta@txccc.org
Hassall
Tim
T
Palmer
Shawna
S
Ashley
David
D
Wallace
Dana
D
Kasow
Kimberly
K
Merchant
Thomas E
TE
Krasin
Matthew J
MJ
Dauser
Robert
R
Boop
Frederick
F
Krance
Robert
R
Woo
Shiao
S
Cheuk
Robyn
R
Lau
Ching
C
Gilbertson
Richard
R
Gajjar
Amar
A
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001932:Brain Neoplasms / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D016371:Cranial Irradiation; D003520:Cyclophosphamide / Q000008:administration & dosage; D018572:Disease-Free Survival; D004307:Dose-Response Relationship, Radiation; D005260:Female; D005720:Gamma Rays; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008297:Male; D018242:Neuroectodermal Tumors, Primitive / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy / Q000628:therapy*; D010865:Pilot Projects; D012307:Risk Factors; D015173:Supratentorial Neoplasms / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy / Q000628:therapy*; D015996:Survival Rate; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
D014750:Vincristine; D003520:Cyclophosphamide; D002945:Cisplatin
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