Primary peripheral T-cell lymphoma, not otherwise specified, of the central nervous system in a child.

Primary peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), is a rare disease that infrequently involves the central nervous system (CNS), and it is even rarer in pediatric patients. Here, we report of a 13-year-old male with primary CNS PTCL-NOS who exhibited a malignant clinical course with recurrence after radiochemotherapy followed by bone marrow transplantation; he died 43 months after diagnosis. Pathology revealed the proliferation of cytotoxic T-cells and clonal T-cell receptor gene rearrangements. Although the optimal therapy for PTCL remains controversial, intensive radiochemotherapy may be required for some patients.