Central nervous system involvement in anaplastic large cell lymphoma in childhood: results from a multicentre European and Japanese study
Williams, Denise × Mori, Tetsuya Reiter, Alfred Woessman, Wilhelm Rosolen, Angelo Wrobel, Grazyna Zsiros, Jozsef Uyttebroeck, Anne Marky, Ildiko Le Deley, Marie-Cécile Brugières, Laurence European Intergroup for Childhood Non-Hodgkin Lymphoma, the Japanese Pediatric Leukemia/Lymphoma Study Group #
Pediatric Blood & Cancer vol:60 issue:10 pages:E118-21
In an international study of systemic childhood ALCL, 12/463 patients had CNS involvement, three of which had isolated CNS disease. Comparative analysis of CNS positive and negative patients showed no difference in ALK positivity, immunophenotype, presence of B symptoms or other sites of disease. The lymphohistiocytic variant was over represented in the CNS positive group (36% vs. 5%). With multi-agent chemotherapy, including high dose methotrexate, Ara-C and intrathecal treatment, the event free and overall survival of the CNS positive group at 5 years were 50% (95%CI, 25-75%) and 74% (45-91%), respectively with a median follow up of 4.1 years.