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Pilomyxoid astrocytoma in the adult cerebellum
Annie S Chen1, Iddo Paldor1, Alpha E Tsui2
1Department of Neurosurgery, Royal Melbourne Hospital, 300 Grattan Street, Parkville, VIC 3050, Australia.
Abstract:
Pilomyxoid astrocytoma (PMA) is a recently recognised World Health Organization (WHO) Grade II tumour that was previously characterised as a subtype of the WHO Grade I pilocytic astrocytoma (PA). PMA has a histological appearance distinct from PA and a poorer prognosis due to its greater propensity for local recurrence and cerebrospinal dissemination. Although originally considered a paediatric tumour involving mainly the hypothalamic and chiasmatic region, reports of the lesion occurring in the adult population and other areas of the neuroaxis are emerging. We review the literature on PMA within the adult population and present the first case of PMA in the cerebellum of an adult female.
Insights
Pilomyxoid astrocytoma (PMA), a WHO Grade II tumor, is distinct from pilocytic astrocytoma (PA) and has a poorer prognosis. Emerging reports show PMA in adults, including a rare cerebellar case in a female.
Area of Science:
- Neuro-oncology
- Pathology
- Neurosurgery
Background:
- Pilomyxoid astrocytoma (PMA) is a distinct World Health Organization (WHO) Grade II tumor, previously classified as a subtype of pilocytic astrocytoma (PA).
- PMA exhibits unique histological features and a less favorable prognosis compared to PA, with a higher tendency for recurrence and spread within the cerebrospinal fluid (CSF).
Observation:
- While initially considered a pediatric tumor primarily affecting the hypothalamic and chiasmatic regions, PMA is increasingly reported in adults and across various neuroaxis locations.
- This review specifically examines the literature on adult PMA cases and introduces the first documented instance of PMA occurring in the cerebellum of an adult female.
Findings:
- Pilomyxoid astrocytoma (PMA) represents a distinct clinicopathological entity within glial tumors.
- Adult-onset PMA, particularly in non-typical locations like the cerebellum, highlights the expanding spectrum of this tumor.
- The poorer prognosis associated with PMA underscores the need for careful management and monitoring.
Implications:
- Recognition of PMA as a separate entity is crucial for accurate diagnosis and prognostication.
- Understanding the occurrence of PMA in adults and diverse locations informs clinical awareness and diagnostic approaches.
- Further research into the biology and treatment of adult pilomyxoid astrocytoma is warranted.
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