title: Primary mediastinal large B-cell lymphoma in Japanese children and adolescents.
abstract: This is the first case series to describe primary mediastinal large B-cell lymphoma (PMLBL) patients in children and adolescents in Asia. We retrospectively identified 17 PMLBL patients diagnosed between 1991 and 2014; in seven of these cases, the diagnosis was confirmed by central review, representing 1.0% of all NHL and 2.2% of all B-NHL cases registered. All patients were teenagers, including seven adolescents, with a median age of 14 years (range 12-18 years). Ten patients were male, and seven were female. The 5-year EFS and OS rates were 81.9 and 84.4%, respectively. All seven recent cases remain alive, of which three received rituximab combination therapy. Incidence, characteristics, and outcome varied considerably from those of Western populations. Further studies, including molecular analysis, are warranted.
authors.affiliation: Children's Cancer Center, National Center for Child Health and Development, 2-10-1, Okura, Setagaya-ku, Tokyo, 157-0074, Japan. osumi-t@ncchd.go.jp.
authors.forename: Tomoo
authors.initials: T
authors.lastname: Osumi
authors.affiliation: Department of Pediatrics, Saiseikai Yokohamashi Nanbu Hospital, Yokohama, Kanagawa, Japan.
authors.forename: Fumiko
authors.initials: F
authors.lastname: Tanaka
authors.affiliation: Department of Pediatrics, St. Marianna University School of Medicine, Kawasaki, Kanagawa, Japan.
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Mori
authors.affiliation: Department of Pediatrics, National Kyushu Cancer Center, Fukuoka, Japan.
authors.forename: Reiji
authors.initials: R
authors.lastname: Fukano
authors.affiliation: Department of Pediatrics, Aichi Medical University, Nagakute, Aichi, Japan.
authors.forename: Masahito
authors.initials: M
authors.lastname: Tsurusawa
authors.affiliation: Department of Pathology, Kurume University School of Medicine, Fukuoka, Japan.
authors.forename: Koichi
authors.initials: K
authors.lastname: Oshima
authors.affiliation: Department of Pathology, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Atsuko
authors.initials: A
authors.lastname: Nakazawa
authors.affiliation: Department of Pediatrics, Sapporo Hokuyu Hospital, Sapporo, Hokkaido, Japan.
authors.forename: Ryoji
authors.initials: R
authors.lastname: Kobayashi
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D044466:Asian People; D002648:Child; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D015994:Incidence; D016393:Lymphoma, B-Cell; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology* / Q000401:mortality; D008228:Lymphoma, Non-Hodgkin / Q000175:diagnosis; D008297:Male; D008479:Mediastinal Neoplasms; D012189:Retrospective Studies; D000069283:Rituximab / Q000627:therapeutic use; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D000069283:Rituximab
keywords: Adolescents; Children; Non-Hodgkin lymphoma; PMLBL; Primary mediastinal large B-cell lymphoma