© 2002 by Society
© 2002 by the Society forNeuro-Oncology
Leptomeningeal dissemination in children with progressive low-gradeneuroepithelial tumors
Division of Neurology, Department of Pediatrics,Children's and Women's Hospital, Vancouver, British Columbia, Canada V6H3V4(J.H.); Institute of Neurology and Neurosurgery, BethIsrael Medical Center, New York, NY 10128 (J.S., L.V., J.A.); and New York University Hospital, New York, NY 10016(D.Z.)
1 Address correspondence and reprint requests to Juliette Hukin, K3, Division ofNeurology, Children's and Women's Hospital, 4480 Oak St., Vancouver, BC,Canada V6H3V4.
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Abstract |
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Our purpose is to describe the incidence and clinical features ofleptomeningeal dissemination (LM) in children with progressive low-gradeneuroepithelial tumor (LGN). We have continuously tracked all patients withprimary CNS tumors since 1986. Satisfactorily followed data were obtained on427 of the 588 patients with localized LGN at diagnosis between 1986 and 1998,177 (42%) of whom developed progressive or recurrent disease. LM wasidentified in 13/177 (7%). The median age at initial diagnosis was 5 years andat LM diagnosis was 8.5 years. The primary tumor sites were diencephalon (6),brainstem (3), cerebellum (2), cerebrum (1), and spinal cord (1). Thehistologies were pilocytic astrocytoma (4), ganglioglioma (4), fibrillaryastrocytoma (3), mixed glioma (1), and glioneurofibroma (1). Managementincluded chemotherapy (2) or radiotherapy (3) or both (7); 1 patient receivedonly radical resections of symptomatic lesions. The 5-year progression-freesurvival rates for patients with localized versus LM disease at recurrencewere 22% (95% confidence interval [CI], 13%-25%) versus 15% (95% CI,0.1%-36%), respectively (P = 0.28). The 5- and 10-year overallsurvival rates for patients with localized disease versus LM were 87% (95% CI,82%-92%) and 83% (95% CI, 77%-89%) versus 68% (95% CI, 39%-91%) and 68% (95%CI, 39%-91%), respectively (P = 0.05). The 7% incidence of LM is alow estimate because patients were not routinely staged at recurrence. Tumorsarising from the diencephalon appeared to predispose to LM; no otherpredisposing features were identified. We strongly urge that for optimumtreatment planning all patients with recurrent LGN be staged with an enhancedspine and brain MRI before adjuvant therapy is initiated. The good survival ofpatients with LGN and LM reflects a more indolent disease than malignant CNStumors with LM.
Received December 27, 2001; Accepted May 14, 2002
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