© 2003 by Society
© 2003 by the Society forNeuro-Oncology
Leptomeningeal dissemination at diagnosis of pediatric low-gradeneuroepithelial tumors
Division of Neurology and Oncology, Department ofPediatrics, Children's and Women's Hospital, Vancouver, BC V6H 3V4, Canada(J.H.); CNS Pfizer, Inc., New York, NY 10017(J.S.); 3629 6th Avenue West, Seattle, WA 98119(H.C.); Institute of Neurology and Neurosurgery, BethIsrael Medical Center, New York, NY 10128 (L.V., J.A.); andNew York University Hospital, New York, NY 10016 (D.Z.);USA
1 Addresss correspondence to Juliette Hukin, K3, Neurology Division, Children'sand Women's Hospital, 4480 Oak Street, Vancouver, BC V6H 3V4, Canada(jhukin{at}cw.bc.ca).
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Abstract |
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The goal of this study was to describe the demographic, histologic, andprognostic features of children with low-grade neuroepithelial tumors (LGN) ofthe CNS presenting with leptomeningeal metastases (LM) at diagnosis. Weidentified 528 newly diagnosed LGN children, 13 (3%) of whom had LM atdiagnosis. LM was defined by neuroimaging, clinical evidence, and/or biopsy.The charts were reviewed and patients contacted to validate the demographicdata, treatment, and clinical status. The distribution of LM patients byprimary tumor site was diencephalon, 5; cerebrum, 2; spinal cord, 3;brainstem, 2; and cerebellum, 1. Six of 8 patients with LM had durableobjective responses to chemotherapy. The 5-year progression-free survival ofpatients with LM at diagnosis was 17%, compared to 85% (95% CI, 80%-91%) forthose with localized LGN who had a gross total resection and 51% (95% CI,44%-52%) for those with localized LGN who had less aggressive surgery(P < 0.0001). Only 1 of these 13 LM patients died. The 5-yearoverall survival of the localized LGN group with a gross total resection was97% (95% CI, 92%-99.9%), and that of the localized LGN group with lessaggressive surgery was 88% (95% CI, 84%-95%) (P = 0.004). The 3%frequency of LM at diagnosis is likely an underestimate since patients withnewly diagnosed LGN were not routinely staged. We suggest that staging beconsidered in the following circumstances: diencephalic primary site,unexplained hydrocephalus, clinical features suggestive of LM, and beforeadjuvant therapy is initiated. The prognosis for children with LM at diagnosisis favorable, and its identification alters therapeutic strategies.
Received July 10, 2002; Accepted February 10, 2003