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Published on: January 6, 2023
Long-term control of central nervous system leukaemia
Insights
Central nervous system (CNS) leukaemia in children with acute lymphoblastic leukaemia can lead to prolonged survival. However, eradicating established CNS leukaemia remains a significant challenge in treatment.
Area of Science:
- Pediatric Oncology
- Hematology
- Neuro-oncology
Background:
- Central nervous system (CNS) involvement is a critical complication in childhood acute lymphoblastic leukaemia (ALL).
- CNS leukaemia significantly impacts patient prognosis and treatment strategies.
- Understanding the long-term outcomes of CNS leukaemia is essential for improving survival rates.
Purpose of the Study:
- To evaluate the outcomes of children with acute lymphoblastic leukaemia and central nervous system involvement.
- To assess the efficacy of different treatment modalities for CNS leukaemia.
- To determine the feasibility of prolonged survival despite CNS disease.
Main Methods:
- Retrospective analysis of 74 children with acute lymphoblastic leukaemia and CNS leukaemia.
- Evaluation of combination chemotherapy, craniospinal irradiation, and intrathecal methotrexate.
- Assessment of remission duration and survival rates based on treatment received.
Main Results:
- CNS involvement at diagnosis was associated with poor survival (<1 year in 4/5 children).
- Combination chemotherapy provided a median remission duration of almost 3 years after first CNS relapse.
- Craniospinal irradiation led to sustained remission in 4/5 children, while intrathecal methotrexate offered a median CNS remission of 2 years.
Conclusions:
- Central nervous system leukaemia in children with ALL is compatible with prolonged survival.
- Established CNS leukaemia presents significant challenges for complete eradication.
- Treatment strategies including chemotherapy, irradiation, and intrathecal methotrexate can achieve durable remissions but long-term neurotoxicity is a concern.
Abstract:
Seventy-four children with acute lymphoblastic leukaemia had one or more episodes of central nervous system (CNS) leukaemia. 5 children had CNS involvement at diagnosis; 4 survived for less than one year. In 35 children who had not had a previous bone marrow relapse on treatment and who received combination chemotherapy, the median duration of haematological remission from the time of first CNS relapse was almost 3 years. 5 children received full dose (2400 rads) craniospinal irradiation after their first CNS relapse; 4 have remained in CNS and haematological remission for 2 1/2 years or more. 18 children who had a CNS relapse after irradiation received 4-weekly intrathecal methotrexate; in 8 children this was given via an intraventricular reservoir. The median duration of CNS remission in children receiving intrathecal methotrexate was 2 years. Systemic and intrathecal treatment was stopped in 7 children after 2 1/2 years in continuous remission and in 2 children after 2 years. 4 of these 9 children remain in remission at intervals from 41 to 69 weeks off treatment but one is severely retarded. These results show that CNS disease is compatible with prolonged survival, but illustrate the difficulties of eradicating established CNS leukaemia.

