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Leptomeningeal dissemination in children with progressive low-grade neuroepithelial 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, Beth Israel 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 of Neurology, Children's and Women's Hospital, 4480 Oak St., Vancouver, BC, Canada V6H3V4.

Abstract

Our purpose is to describe the incidence and clinical features of leptomeningeal dissemination (LM) in children with progressive low-grade neuroepithelial tumor (LGN). We have continuously tracked all patients with primary CNS tumors since 1986. Satisfactorily followed data were obtained on 427 of the 588 patients with localized LGN at diagnosis between 1986 and 1998, 177 (42%) of whom developed progressive or recurrent disease. LM was identified in 13/177 (7%). The median age at initial diagnosis was 5 years and at LM diagnosis was 8.5 years. The primary tumor sites were diencephalon (6), brainstem (3), cerebellum (2), cerebrum (1), and spinal cord (1). The histologies were pilocytic astrocytoma (4), ganglioglioma (4), fibrillary astrocytoma (3), mixed glioma (1), and glioneurofibroma (1). Management included chemotherapy (2) or radiotherapy (3) or both (7); 1 patient received only radical resections of symptomatic lesions. The 5-year progression-free survival rates for patients with localized versus LM disease at recurrence were 22% (95% confidence interval [CI], 13%-25%) versus 15% (95% CI, 0.1%-36%), respectively (P = 0.28). The 5- and 10-year overall survival 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 a low estimate because patients were not routinely staged at recurrence. Tumors arising from the diencephalon appeared to predispose to LM; no other predisposing features were identified. We strongly urge that for optimum treatment planning all patients with recurrent LGN be staged with an enhanced spine and brain MRI before adjuvant therapy is initiated. The good survival of patients with LGN and LM reflects a more indolent disease than malignant CNS tumors with LM.

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				L. Massimi, D. Battaglia, G. Paternoster, D. Martinelli, C. Sturiale, and C. Di Rocco Segmental Spinal Myoclonus and Metastatic Cervical Ganglioglioma: An Unusual Association J Child Neurol, March 1, 2009; 24(3): 365 - 369. [Abstract] [PDF]

				J. Hukin, J. Siffert, H. Cohen, L. Velasquez, D. Zagzag, and J. Allen Leptomeningeal dissemination at diagnosis of pediatric low-grade neuroepithelial tumors Neuro-oncol, July 1, 2003; 5(3): 188 - 196. [Abstract] [PDF]