Eleven infants with
pineoblastomas were treated with prolonged postoperative
chemotherapy in an attempt to delay radiation and reduce neurotoxicity. These infants were part of the Pediatric Oncology Group infant
brain tumor study but the outcome of infants with
pineoblastomas was not previously reported. Ages ranged from 1 month to 35 months, with eight of 11 < or = 12 months at diagnosis. Four had + cytology and three had + myelograms at diagnosis. The majority had partial surgical resection (25-75% reduction in
tumor) and 10 had shunts.
Chemotherapy consisted of two 28-day cycles of
cyclophosphamide plus
vincristine, followed by one 28-day cycle of
cisplatin plus
etoposide. Craniospinal radiation was planned following completion of either 2 years of
chemotherapy (children less than 24 months at diagnosis) or following one year (children 24-36 months at diagnosis). Neuroimaging results following two cycles of
cyclophosphamide and
vincristine were one partial response, five stable disease, and five progressive disease. There were no responders in the leptomeninges. All children ultimately failed
chemotherapy (2 months-11 months). Nine failed in the primary site. Of those eight children in whom a metastatic workup was performed at time of progression, all had evidence of leptomeningeal disease. Six received radiation following failure on
chemotherapy. All failed either in the primary site, leptomeninges or extraneurally (peritoneal cavity). All children died. Survival following diagnosis ranged from 4 months to 13 months. This
chemotherapy regimen was neither effective in controlling
tumor in the primary site nor in treating or preventing leptomeningeal spread.