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Medulloblastoma in childhood. Survival and functional results
Insights
Pediatric medulloblastoma treatment improved survival rates with radiotherapy, but led to significant long-term cognitive and behavioral issues in survivors. Radiotherapy was identified as a contributing factor to these sequelae.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Radiation Oncology
Background:
- Medulloblastoma is a common pediatric brain tumor.
- Treatment historically involved surgery and radiotherapy.
- Long-term sequelae of treatment require careful evaluation.
Purpose of the Study:
- To assess the outcomes of medulloblastoma treatment in children.
- To evaluate the impact of radiotherapy on survival and neurodevelopmental status.
- To identify factors contributing to long-term sequelae.
Main Methods:
- Retrospective analysis of 57 pediatric medulloblastoma cases treated between 1964 and 1976.
- Comparison of outcomes between irradiated and non-irradiated groups (cerebellar astrocytomas).
- Assessment of neurocognitive function, behavior, and endocrine status.
Main Results:
- Postoperative mortality was 10.5%, higher in infants and brain stem tumors.
- Five-year survival was 54% overall, increasing to 71% with radiotherapy.
- Survivors frequently experienced cognitive (IQ 70-90 in 58%) and behavioral disturbances (93%), with radiotherapy implicated as a partial cause.
- High rates of growth hormone deficiency (65%), short stature (55%), and hypothyroidism (58%) were observed.
Conclusions:
- Radiotherapy for pediatric medulloblastoma improves survival but is associated with significant neurodevelopmental and endocrine deficits.
- Careful consideration of radiotherapy's long-term effects is crucial in pediatric neuro-oncology.
- Further research into mitigating treatment-related sequelae is warranted.
Abstract:
The authors report on a series of 57 medulloblastomas in children operated upon -etween 1964 and 1976. Among these children, 44 completed the treatment with radiotherapy to the whole central nervous system. The postoperative mortality rate in 10.5%. Postoperative deaths occurred mainly in infants or when a tumour involved the brain stem. The five-year survival rate is 54% in the whole series. It rose to 71% in the patients who completed the treatment with radiotherapy. The study shows that the life of survivals is frequently impaired by mental or behavioural disturbances. IQ varies from 70 to 90 in 58% of the children; it is below 70 in 31%. Behavioural disturbances are found in 93% of cases. 82% have defective spatial orientation, dysphasia, or dysgraphia. In order to evaluate the responsibility of X-ray therapy for the development of these sequelae, the results have been compared to those of a series of cerebellar astrocytomas surgically removed, but not irradiated. The comparison shows that radiotherapy is at least partially responsible for the mental and behavioural disturbances. No relation was found between these disturbances and a persistent ventricular dilatation or an endocrine dysfunction. However the endocrine assessment showed growth hromone deficiency in 65% of the children, short stature in 55%, and compensated hypothyroidism in 58%.