Pleomorphic xanthoastrocytoma with anaplastic features: a case report

Area of Science:

• Neuro-oncology
• Pediatric Pathology
• Neurosurgery

Background:

• Pleomorphic xanthoastrocytoma (PXA) is generally classified as a WHO Grade-II astrocytic tumor with a favorable prognosis.
• Recent observations indicate certain PXA patterns exhibit a poorer prognosis, leading to the concept of "PXA with anaplastic features".

Observation:

• A 9-year-old girl presented with recurrent headaches, seizures, and declining academic performance.
• MRI revealed a left fronto-parietal enhancing mass with callosal involvement and a concurrent arteriovenous malformation.
• Initial assessment suggested a high-grade glial neoplasm or PNET.

Findings:

• Intraoperative frozen sections showed spindle cells, pleomorphic bizarre giant cells with multilobated nuclei, atypical mitosis, and abundant eosinophilic cytoplasm.
• Permanent sections confirmed a biphasic glial neoplasm with spindle cells, multinucleated giant cells, vacuolated/lipidized cytoplasm, nuclear hyperchromasia, intranuclear inclusions, eosinophilic granular bodies, increased mitosis (7/10 HPF), microvascular proliferation, necrosis, and parenchymal invasion.
• Histomorphological findings led to a diagnosis of pleomorphic xanthoastrocytoma with anaplastic features.

Implications:

• This case underscores the importance of recognizing anaplastic features in PXA, even in pediatric patients.
• Accurate histopathological diagnosis is crucial for determining prognosis and guiding treatment strategies for aggressive glial neoplasms.
• The presence of anaplastic features necessitates a re-evaluation of the typical favorable prognosis associated with PXA.