Hyperfractionated craniospinal radiation therapy for primitive neuroectodermal tumors: results of a Phase II study.
OBJECTIVE
To report the results of a Phase II study of hyperfractionated craniospinal radiation therapy, with and without adjuvant chemotherapy for primitive neuroectodermal brain tumors (PNETs) and malignant ependymomas.


METHODS
Newly diagnosed PNET or malignant ependymomas were treated with hyperfractionated craniospinal radiation therapy. The primary tumor site was treated to a dose of 72 Gy, with 30 Gy given to the rest of the craniospinal axis. The fraction size was 1.0 Gy, given twice a day. Patients with poor risk factors also received adjuvant chemotherapy with CCNU, cisplatin, and vincristine. Patients had follow-up for survival, time to tumor progression, and patterns of relapse.


RESULTS
A total of 39 patients (21 males/18 females) were treated between March 12, 1990 and October 29, 1992. The median age was 16 years (range 3-59 years). Tumor types included 25 medulloblastomas, 5 pineoblastomas, 5 cerebral PNETs, 1 spinal cord PNET, and 3 malignant ependymomas. Twenty cases were staged as poor-risk and received adjuvant chemotherapy following radiation. Three-year progression-free survival (PFS) was 60% and 63% for poor-risk and good-risk patients, respectively. Overall 3-year survival for these groups was 70% and 79%, respectively. For the 25 patients with medulloblastoma, there were 16 good-risk and 9 poor-risk patients. Three-year PFSs were 63% and 56%, respectively. The 5-year survival for good-risk medulloblastoma was 69% with 43.7% of these patients having failures outside the primary site.


CONCLUSIONS
Survival in patients with good-risk medulloblastoma was no better than that seen in previous studies with single-fraction radiation, and the rate of failure outside the primary site is excessive. Those with poor-risk features had comparable survival to that seen in patients with good risk factors, but these patients were treated with chemotherapy, and the role that hyperfractionated radiation played in their outcome is uncertain.
Department of Neurological Surgery, University of California, San Francisco 94117, USA.
M D
MD
Prados
M S
MS
Edwards
S M
SM
Chang
C
C
Russo
R
R
Davis
J
J
Rabbitt
M
M
Page
K
K
Lamborn
W M
WM
Wara
D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000188:drug therapy / Q000532:radiotherapy*; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D016371:Cranial Irradiation / Q000379:methods*; D018450:Disease Progression; D019583:Dose Fractionation, Radiation; D004806:Ependymoma / Q000188:drug therapy / Q000532:radiotherapy; D005260:Female; D006801:Humans; D008297:Male; D008527:Medulloblastoma / Q000188:drug therapy / Q000532:radiotherapy; D008875:Middle Aged; D009367:Neoplasm Staging; D018242:Neuroectodermal Tumors, Primitive / Q000188:drug therapy / Q000532:radiotherapy*; D010870:Pineal Gland; D010871:Pinealoma / Q000188:drug therapy / Q000532:radiotherapy; D012008:Recurrence