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Cerebellar pilocytic astrocytoma. Retrospective cohort study assessing postoperative functional outcome, cerebellar
Mauro Ruella1, Sebastian Giovannini1, Christian Pirozzi Chiusa2
1Department of Neurosurgery, Fleni, Montañeses 2325, CP 1428, Buenos Aires, Argentina.
Insights
Pediatric craniopharyngiomas (cPAs) show favorable outcomes with appropriate management of recurrence and complications. Early gross total resection (GTR) is advised, prioritizing neurological function over complete tumor removal if necessary.
Area of Science:
- Pediatric Neurosurgery
- Neuro-oncology
- Pediatric Oncology
Background:
- Craniopharyngiomas (cPAs) are rare pediatric brain tumors.
- Understanding factors influencing outcomes in pediatric cPAs is crucial for effective treatment strategies.
Purpose of the Study:
- To analyze pediatric patients with cPAs.
- To identify factors influencing neurological status, clinical manifestations (CMS), and hydrocephalus outcomes.
Main Methods:
- Retrospective cohort study of 100 pediatric cPA patients with at least 12 months follow-up.
- Assessment of clinical, imaging, surgical, and histopathological features.
- Clinical outcomes evaluated using FSS and modified Bloom-Scale; recurrence, progression-free survival (PFS), and mortality analyzed.
Main Results:
- Mean follow-up was 53.9 months in 100 patients (mean age 7.6 years).
- Hydrocephalus occurred in 79% of patients; 24% had brainstem invasion.
- Cerebellar mutism syndrome (CMS) in 15 patients was linked to tumor location, pontine invasion, and postoperative complications. Recurrence observed in 26% of cases.
Conclusions:
- Treatment of pediatric cPAs yields favorable results when recurrence and surgical complications are managed.
- Early gross total resection (GTR) is recommended, balancing tumor removal with neurological preservation.
- Individualized preoperative management of hydrocephalus is essential.
Objective:
Analyze a series of pediatric patients with cPAs evaluating factors that may modify or determine the final outcome in terms of neurological status, CMS and hydrocephalus.
Materials And Methods:
Single Center, retrospective cohort study of surgical patients with cPA and at least 12 months of follow-up. Clinical, imaging and surgical features, histopathology and adjuvancy were assessed. Clinical outcome was categorized using the FSS and modified Bloom-Scale in the short and long term. Recurrency, PFS and mortality were also analyzed.
Results:
A total of 100 patients with a mean follow-up of 53.9 months, with no gender predilection, and a mean age of 7.6 years were included. The most frequent location was central and 24% of the tumors presented brainstem invasion. 79 patients had hydrocephalus at diagnosis and 48% required preoperative treatment. RT was achieved in 67% of cases. CMS was observed in 15 patients, statistically associated with location, pontine invasion, preoperative Bloom-score, and postoperative meningitis/ventriculitis. The functional "outcome" was correlated with complications such as meningitis/ventriculitis and cerebellar hematoma, mutism and recurrence. Recurrence was observed in 26% of cases and surgical reintervention was the chosen therapy.
Conclusion:
The treatment of cPAs in pediatric patients is associated with relatively favorable results. Recurrence, CMS and surgical complications play a prognostic value in the outcome and therefore must be managed appropriately. Early treatment with the intention of GTR should be considered, opting, if this is not possible, to leave a tumor residue over neurological damage. The treatment of hydrocephalus in the preoperative instance requires individualized management.
