Primary mediastinal large B-cell lymphoma in Japanese children and adolescents.

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.