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Pediatric cerebellar pilomyxoid-spectrum astrocytomas
Jonathan A Forbes1, Bret C Mobley, Thomas M O'Lynnger
1Department of Neurosurgery, Vanderbilt University Medical Center, Nashville, Tennessee, USA. jonathan.forbes@vanderbilt.edu
Journal of Neurosurgery. Pediatrics
|July 5, 2011
Summary
Pediatric cerebellar pilomyxoid astrocytomas have higher recurrence and leptomeningeal dissemination rates than pilocytic astrocytomas. This study presents two cases, highlighting the aggressive nature of these rare pediatric brain tumors.
Area of Science:
- Pediatric neuro-oncology
- Neurosurgery
- Pediatric neuropathology
Background:
- Pilomyxoid astrocytomas (PMAs) are rare pediatric brain tumors, often occurring in the hypothalamic/chiasmatic region.
- Pilomyxoid features can also present as intermediate pilomyxoid tumors (IPTs), with features of both pilocytic astrocytoma (PA) and PMA.
- Cerebellar PMAs and IPTs are less commonly described in neurosurgical literature compared to hypothalamic/chiasmatic counterparts.
Observation:
- This study details two pediatric cases of cerebellar pilomyxoid-spectrum astrocytomas: one classic PMA and one IPT.
- Both patients presented with cerebellar symptoms, including gait disturbance, ataxia, and weakness.
- Tumor resection was limited by brainstem invasion, precluding gross-total resection in both cases.
Findings:
- One patient developed leptomeningeal dissemination (LD) three years post-surgery, while the other experienced local recurrence within twelve months.
- Both patients required chemotherapy following surgical intervention.
- Pilomyxoid-spectrum astrocytomas constituted 4% of cerebellar neoplasms and 10% of cerebellar glial neoplasms in the retrospective review.
Implications:
- Cerebellar pilomyxoid-spectrum astrocytomas demonstrate a higher propensity for local recurrence and leptomeningeal dissemination compared to cerebellar PAs.
- These findings underscore the aggressive behavior of these tumors in pediatric populations.
- Further research into optimal treatment strategies for cerebellar pilomyxoid-spectrum astrocytomas is warranted.
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