title: Central nervous system involvement in anaplastic large cell lymphoma in childhood: results from a multicentre European and Japanese study.
abstract: 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.
authors.affiliation: Department of Pediatric Oncology, Cambridge University Hospital NHS Trust, Cambridge, United Kingdom. denise.williams@addenbrookes.nhs.uk
authors.forename: Denise
authors.initials: D
authors.lastname: Williams
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Mori
authors.forename: Alfred
authors.initials: A
authors.lastname: Reiter
authors.forename: Wilhelm
authors.initials: W
authors.lastname: Woessman
authors.forename: Angelo
authors.initials: A
authors.lastname: Rosolen
authors.forename: Grazyna
authors.initials: G
authors.lastname: Wrobel
authors.forename: Jozsef
authors.initials: J
authors.lastname: Zsiros
authors.forename: Anne
authors.initials: A
authors.lastname: Uyttebroeck
authors.forename: Ildiko
authors.initials: I
authors.lastname: Marky
authors.forename: Marie-Cécile
authors.initials: MC
authors.lastname: Le Deley
authors.forename: Laurence
authors.initials: L
authors.lastname: Brugières
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D016543:Central Nervous System Neoplasms / Q000188:drug therapy* / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D003561:Cytarabine / Q000008:administration & dosage; D018572:Disease-Free Survival; D005060:Europe; D005500:Follow-Up Studies; D006801:Humans; D007564:Japan; D017728:Lymphoma, Large-Cell, Anaplastic / Q000188:drug therapy* / Q000401:mortality; D008297:Male; D008727:Methotrexate / Q000008:administration & dosage; D015996:Survival Rate
chemical_list: D003561:Cytarabine; D008727:Methotrexate
keywords: CNS disease; anaplastic large cell lymphoma; childhood; lymphohistiocytic; non-Hodgkin lymphoma