Primary central nervous system lymphoma in children - a rare but serious disease with good prognosis from a single center in China

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Abstract Objective:To discuss the clinical efficacy of systemic chemotherapy based on HD-MTX combined with IT therapy in children with PCNSL, and to increase the understanding of the treatment of children with PCNSL. Methods: We reviewed the clinical data, treatment regimens and survival status of 12 children diagnosed with PCNSL in Beijing Children's Hospital, Capital Medical University, during the past 10 years. We retrospectively reported information on PCNSL in children from Eastern countries. Results:Twelve patients among 1500 with non-Hodgkin lymphoma (NHL) were diagnosed with PCNSL in Beijing Children’s Hospital in the past ten years. The male: female ratio was 4:1, and the median age was 5.9 yr (2.3-13.9 yr). The primary sites were ventricle, cerebellum, spinal cord, and spinal canal, each of which was represented by one case; the remaining eight cases were all in multiple areas. Their pathologic diagnoses were lymphoblastic lymphoma in six cases, Burkitt lymphoma in three, anaplasticlarge cell lymphoma (ALCL) in two and diffuse large B-cell lymphoma (DLBCL) in one. All patients received a high-dose methotrexate (HD-MTX)-based induction chemotherapy regimen combined with intrathecal (IT) chemotherapy (methotrexate, cytarabine, dexamethasone) that was administered 10 to 26 times (median 16 times). None of the patients had RT as a first-line treatment. As the understanding of this disease has been updated, patients with invasive mature B-cell origin are no longer receiving rituximab chemotherapy, and the duration of maintenance therapy is gradually shortening. No patient had recurrence or treatment-related mortality. Conclusions: PCNSL is a rare childhood disease that can be successfully treated with HD-MTX-based systemic chemotherapy combined with IT chemotherapy rather than craniospinal RT.

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