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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
Insights
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.
Object:
Pediatric cerebellar astrocytomas with pilomyxoid features include classic pilomyxoid astrocytomas (PMAs) and intermediate pilomyxoid tumors (IPTs). Since the original description of PMA in 1999, most reports in the literature have described PMAs arising from the hypothalamic/chiasmatic region. To the authors' knowledge, PMAs arising from the posterior fossa have not been discussed in the neurosurgical literature. Intermediate pilomyxoid tumors, or tumors with pathological features of both pilocytic astrocytoma (PA) and PMA, have only recently been described. In this article, the authors present 2 cases that fall within the spectrum of pediatric cerebellar PMA-including a classic PMA and an intermediate pilomyxoid tumor. The authors compare the radiological presentation, surgical results, and postoperative course to findings in a cohort of 15 patients with cerebellar PAs.
Methods:
Between 2003 and 2010, 2 patients with pilomyxoid-spectrum astrocytomas underwent treatment at Vanderbilt Children's Hospital. One was a 22-month-old girl who presented with progressive gait disturbance and falls. The other was a 4-year-old girl who presented with ataxia and generalized weakness. In a retrospective review of pediatric cerebellar neoplasms resected by the senior author during this period, these tumors comprised 4% of cerebellar neoplasms and approximately 10% of cerebellar glial neoplasms.
Results:
Both patients were treated with midline suboccipital craniotomy for resection. In both cases, tumor invasion anteriorly into the brainstem prevented gross-total resection. the patient in Case 1 was placed on chemotherapy following pathological diagnosis and later developed definitive evidence of leptomeningeal dissemination (LD) 3 years after the operation. The patient in Case 2 was placed on chemotherapy after exhibiting progressive evidence of local recurrence (findings were negative for LD) 12 months following resection.
Conclusions:
Pediatric patients with cerebellar pilomyxoid-spectrum astrocytomas appear to suffer higher rates of local recurrence and LD than pediatric patients with cerebellar PAs.
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