The goal of this study was to describe the demographic, histologic, and prognostic features of @entity1 with low-grade @entity10053 (LGN) of the CNS presenting with leptomeningeal metastases (LM) at diagnosis. We identified 528 newly diagnosed LGN @entity1 , 13 (3%) of whom had LM at diagnosis. LM was defined by neuroimaging, clinical evidence, and/or biopsy. The charts were reviewed and @entity1 contacted to validate the demographic data, treatment, and clinical status. The distribution of LM @entity1 by primary @entity5 site was diencephalon, 5; cerebrum, 2; spinal cord, 3; brainstem, 2; and cerebellum, 1. Six of 8 @entity1 with LM had durable objective responses to chemotherapy. The 5-year progression-free survival of @entity1 with LM at diagnosis was 17%, compared to 85% (95% CI, 80%-91%) for those 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 @entity1 died. The 5-year overall survival of the localized LGN group with a gross total resection was 97% (95% CI, 92%-99.9%), and that of the localized LGN group with less aggressive surgery was 88% (95% CI, 84%-95%) ( P = 0.004). The 3% frequency of LM at diagnosis is likely an underestimate since @entity1 with newly diagnosed LGN were not routinely staged. We suggest that staging be considered in the following circumstances: diencephalic primary site, unexplained @entity1276 , clinical features suggestive of LM, and before adjuvant therapy is initiated. The prognosis for @entity1 with LM at diagnosis is favorable, and its identification alters therapeutic strategies.

Leptomeningeal dissemination at diagnosis of pediatric low-grade XXXX .

@entity1 :: ('9606', 'Species') :: ['children', 'patients'] @entity5 :: ('MESH:D009369', 'Disease') :: ['tumor'] @entity10053 :: ('MESH:D018302', 'Disease') :: ['neuroepithelial tumors'] @entity1276 :: ('MESH:D006849', 'Disease') :: ['hydrocephalus']
@entity10053 :: (MESH:D018302,Disease) :: ['neuroepithelial tumors']
