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Published on: September 7, 2022
Primary central nervous system lymphoma in children
Oussama Abla1, Sheila Weitzman
1Division of Hematology/Oncology, The Hospital for Sick Children and Department of Paediatrics, University of Toronto, Ontario, Canada.
Insights
Childhood primary central nervous system lymphoma (PCNSL) is rare, but prognosis is better than in adults. Intensive chemotherapy alone offers long-term remission for most children with PCNSL.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Rare Cancers
Background:
- Primary central nervous system lymphoma (PCNSL) is a rare brain tumor in children.
- Optimal management and prognosis for pediatric PCNSL remain undefined.
- Childhood PCNSL prognosis is generally better than in adults, excluding HIV-related cases.
Purpose of the Study:
- To review the incidence, clinical features, diagnosis, and treatment of childhood PCNSL.
- To evaluate current understanding and identify needs for future research in pediatric PCNSL.
Main Methods:
- Review of existing literature and data on childhood PCNSL.
- Analysis of incidence rates, clinical presentations, diagnostic approaches, and treatment outcomes.
- Comparison of pediatric versus adult PCNSL prognosis.
Main Results:
- The exact incidence of childhood PCNSL is difficult to determine due to a lack of prospective studies.
- Intensive chemotherapy alone can achieve long-term remissions in the majority of children (estimated 70% 5-year event-free survival).
- Cranial irradiation is typically reserved for disease relapse.
Conclusions:
- Childhood PCNSL, while rare, has a favorable prognosis compared to adult PCNSL.
- Intensive chemotherapy is the primary treatment, with a high success rate for long-term remission.
- Prospective multinational studies are essential for advancing the management of childhood PCNSL.
Abstract:
Primary central nervous system lymphoma (PCNSL) is a very rare brain tumor in children, and the optimal management and prognosis of such patients have yet to be defined. In this study, the incidence rate, clinical features, diagnosis, and treatment of childhood PCNSL are reviewed. Except for human immunodeficiency virus-related PCNSL, the prognosis for patients with this tumor type is significantly better in children than in adults. In the absence of prospective studies, it is very difficult to determine the true incidence and the best therapeutic strategy for this rare entity. The majority of children with PCNSL, however, can achieve long-term remissions with intensive chemotherapy alone (an estimated 70% 5-year event-free survival rate), and cranial irradiation can be reserved for relapse of the disease. Further progress in the management of childhood PCNSL will require prospective multinational studies.
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