Pilocytic astrocytoma with leptomeningeal dissemination

Purpose: Pilocytic astrocytoma (PA) is a common pediatric glioma that is generally characterized by indolent growth. However, there are reports of PA disseminating throughout the central nervous system. Given the rarity of dissemination, the appropriate treatment for these patients is poorly defined. In this case series, we describe the clinical characteristics and treatment outcomes of six children treated for disseminated PA at our institution and review the current published literature. Methods: Six cases of disseminated PA treated at the University of Texas MD Anderson Cancer Center were identified. Demographics, disease characteristics, and follow-up data were compiled. Fifty-three reported cases were identified in the published literature. Results: Our cohort's mean age at presentation was 7 years, and the mean time to identification of disseminated disease was 12 months after initial diagnosis. Two patients underwent chemotherapy, and all underwent proton beam radiation therapy to all or part of the craniospinal axis. With a median follow-up of 24 months after radiation therapy, five of six patients were alive, four with stable disease and one with progressive disease. Conclusions: Treatment of disseminated PA is frequently multi-modal, including surgical resection, chemotherapy, and radiation therapy. On the basis of early clinical data, extended-field radiation therapy is a viable option for treating disseminated PA.