Outcome for children with supratentorial primitive neuroectodermal tumors treated with surgery, radiation, and chemotherapy

BAGKGROUND, The outcome of a child with a primitive neuroectodermal tumors arising supratentorially (SPNET) is not well characterized and may differ f rom the outcome of a patient with a histologically similar cerebellar tumor (medulloblastoma [MB]). Recently, 5-year progression free survival rates a s high as 80% have been reported for children with MB treated with craniosp inal radiation (CRT) and chemotherapy including cisplatin, lomustine (CCNU) , and vincristine (VCR). METHODS, The authors reviewed the outcome of 22 consecutive patients age 3 years and older (mean age, 10 years; range, 3-18 years) with SPNET who were treated at the study institutions between 1981 and 1996. Tumor location in cluded was 13 pineal, 6 cortical, and 3 thalamic or suprasellar. Five patie nts had disease dissemination at diagnosis. All patients underwent surgery and staging, followed by CRT and chemotherapy with cisplatin, CCNU, and VCR . RESULTS. Of the 22 patients, 13 had developed disease progression and 10 ha d died at the time of last follow-up. Overall progression free survival (PF S) was 47% +/- 11% at 3 years and 37% +/- 11% at 5 years. There was a signi ficant difference in PFS between patients with localized disease versus tho se with disseminated disease (P = 0.04). There was no statistical associati on between tumor location and survival. Although not significant (P = 0.21) , there was a trend toward better survival of those patients with complete or near-complete resection compared with those with partial resection or bi opsy. CONCLUSIONS, The results of the current study demonstrate that the outcome for children with SPNET treated with radiation and chemotherapy appears wor se than for children with MB treated with identical therapy. This suggests that there may be biologic differences between supratentorial and infratent orial primitive neuroectodermal tumors, thus requiring refinements in treat ment. (C) 2000 American Cancer Society.