title: Clinical presentation of anaplastic large-cell lymphoma in the central nervous system.
abstract: The majority of primary central nervous system (CNS) lymphomas are diffuse large B-cell lymphomas (DLBCLs) and anaplastic large-cell lymphoma (ALCL) is a type of T-cell tumor that is rare in the CNS. The aim of this study was to elucidate the clinical presentation and standard therapy of ALCLs by investigating reported cases. Additionally, a case of anaplastic lymphoma kinase (ALK)-positive ALCL in a 20-year-old man who exhibited no recurrence for >5 years following high-dose methotrexate (HD-MTX) treatment was described. Twenty-six immunocompetent patients with ALCL of the CNS that were previously reported and 1 case of ALCL of the CNS treated at our hospital were investigated. Overall survival (OS) was analyzed in relation to survival factors such as age, ALK status and the treatment regimen. The male:female ratio of the patients was 19:8. Of the 27 patients, 13 (48.1%) were ALK-positive, 9 (33.3%) were ALK-negative and the ALK status was not determined in the remaining 5 patients (18.5%). ALK-positive ALCL occurred at a younger age (median age, 17 years) and exhibited a favorable course (5-year OS, 75.0%), whereas ALK-negative ALCL presented at an older age (median age, 65 years) and resulted in fatal outcomes (5-year OS, <12.5%). Similar to the findings for systemic ALCL, ALK positivity, age <40 years and chemotherapy are associated with long-term survival for ALCL of the CNS. Chemoradiotherapy including methotrexate is recommended for ALCL and the possibility of treatment with chemotherapy alone for ALK-positive ALCL is currently under consideration.
authors.affiliation: Department of Neurosurgery and Neuro-Oncology, National Cancer Center, Tokyo 104-0045;
authors.forename: Masashi
authors.initials: M
authors.lastname: Nomura
authors.affiliation: Department of Neurosurgery and Neuro-Oncology, National Cancer Center, Tokyo 104-0045;
authors.forename: Yoshitaka
authors.initials: Y
authors.lastname: Narita
authors.affiliation: Department of Neurosurgery and Neuro-Oncology, National Cancer Center, Tokyo 104-0045;
authors.forename: Yasuji
authors.initials: Y
authors.lastname: Miyakita
authors.affiliation: Department of Neurosurgery and Neuro-Oncology, National Cancer Center, Tokyo 104-0045;
authors.forename: Makoto
authors.initials: M
authors.lastname: Ohno
authors.affiliation: Division of Pathology and Clinical Laboratories, National Cancer Center, Tokyo 104-0045;
authors.forename: Shintaro
authors.initials: S
authors.lastname: Fukushima
authors.affiliation: Department of Neurosurgery, Tokyo Women's Medical University, Tokyo 116-8567, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Maruyama
authors.affiliation: Department of Neurosurgery, Tokyo Women's Medical University, Tokyo 116-8567, Japan.
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Muragaki
authors.affiliation: Department of Neurosurgery and Neuro-Oncology, National Cancer Center, Tokyo 104-0045;
authors.forename: Soichiro
authors.initials: S
authors.lastname: Shibui
keywords: T-cell lymphoma; anaplastic large-cell lymphoma; anaplastic lymphoma kinase; primary central nervous system lymphoma