Publication:

Anaplastic pleomorphic xanthoastrocytoma in a 12-year-old with early leptomeningeal dissemination: a rare pediatric case report from Central India

Pleomorphic xanthoastrocytoma (PXA) is a rare glioma subtype, making up less than 1% of astrocytoma, mainly affecting young adults. It typically appears in solid, cystic, or mixed forms in the supratentorial region, especially the temporal lobe, with a peak onset around age 20. Reported cases describe cerebrospinal fluid dissemination, often occurring months after diagnosis. We report a case of 12-year-old boy presenting with headache and vomiting for over a month, with no significant medical or family history. Magnetic resonance imaging brain showed lesion in the right fronto-parietal region. He underwent surgery to excise the lesion, which was diagnosed as a PXA, classified as World Health Organization (WHO) grade 3. Immunohistochemical analysis indicated positivity for ATRX, H3K27me3, and p53 protein suggesting high-grade glial tumor. After surgery, he had recurrence of headache within a month. Follow-up magnetic resonance imaging revealed residual tumor, leading to second surgery for tumor re-exploration. He received adjuvant radiation therapy and chemotherapy with Temozolomide. Despite treatment, he re-developed symptoms, and further imaging showed metastasis in the left medial temporal lobe and lumbar spine. Unfortunately, he did not respond to chemotherapy and passed away within a month. This case highlights the complex behavior of PXA, which complicates disease management. It denotes surgical excision alone is inadequate and should be complemented by spine staging scans and radiation therapy. Poor prognosis indicators include necrosis, high mitotic activity and incomplete resection underscoring the need for comprehensive treatment approach.