Prognostic factors and treatment results for supratentorial primitive neuroectodermal tumors in children using radiation and chemotherapy: A Childrens Cancer Group randomized trial

Bruce H. Cohen, Paul M. Zeltzer, James M. Boyett, J. Russell Geyer, Jeffrey C. Allen, Jonathan L. Finlay, Patricia McGuire-Cullen, Jerrold M. Milstein, Lucy B. Rorke, Philip Stanley, James A. Stehbens, Susan B. Shurin, Jeffrey Wisoff, Kenneth R. Stevens, A. Leland Albright

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151 Scopus citations

Abstract

Purpose: To determine clinical characteristics and response to treatment for children with supratentorial primitive neuroectodermal tumors (S-PNETs). Patients and Methods: After surgery and staging, 55 patients aged 1.5 to 19.3 years with S-PNETs were randomized to receive craniospinal radiotherapy (RT) followed by eight cycles of 1-(2-chloro-ethyl)-3-cyclohexyl-nitrosourea (CCNU), vincristine (VCR), and prednisone (standard treatment) or two cycles of 8-in-1 chemotherapy followed by RT and then eight additional cycles of 8- in-1. Results: Three-year Kaplan-Meier estimates (estimate ± SE) of survival and progression-free survival (PFS) rates for patients with confirmed diagnoses of S-PNET were 57% ± 8% and 45% ± 8%, respectively; survival and PFS rates for children with PNETs located in the pineal region were 73% ± 12% and 61% ± 13%, respectively, and were significantly different from the other S-PNETs (P < .03). The 8-in-1 arm had greater toxicity than the standard-treatment arm. Distributions of PFS between the two treatment groups were not significantly different (P > .5). Other univariate prognostic factors that influenced PFS included metastasis (M) stage (P < .03: M0 50% ± 9% v M1-4 0%) and age (P < .02: 1.5 to 2 years 25% ± 13% v ≥ 3 years 53% ± 9%). Conclusion: In this first randomized treatment trial for S-PNETs in children, no significant differences were detected between the two treatment groups. M0 and pineal site of involvement were independent predictors of a better outcome. However, survival was better than previously reported.

Original languageEnglish (US)
Pages (from-to)1687-1696
Number of pages10
JournalJournal of Clinical Oncology
Volume13
Issue number7
DOIs
StatePublished - Jul 1995

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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    Cohen, B. H., Zeltzer, P. M., Boyett, J. M., Geyer, J. R., Allen, J. C., Finlay, J. L., McGuire-Cullen, P., Milstein, J. M., Rorke, L. B., Stanley, P., Stehbens, J. A., Shurin, S. B., Wisoff, J., Stevens, K. R., & Albright, A. L. (1995). Prognostic factors and treatment results for supratentorial primitive neuroectodermal tumors in children using radiation and chemotherapy: A Childrens Cancer Group randomized trial. Journal of Clinical Oncology, 13(7), 1687-1696. https://doi.org/10.1200/JCO.1995.13.7.1687