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Pilomyxoid astrocytoma with high proliferation index
Sonam Kumar Pruthi1, Shrijeet Chakraborti1, Ramadas Naik1
1Department of Pathology, Kasturba Medical College, Lighthouse Hill Road, Mangalore, India.
Insights
Pilomyxoid astrocytomas are rare, aggressive brain tumors. This case highlights a 12-year-old with a temporal lobe tumor, showing high Ki-67 proliferation and recurrence after treatment.
Area of Science:
- Neuro-oncology
- Pediatric Pathology
Background:
- Pilomyxoid astrocytomas (PMAs) are uncommon, aggressive piloid neoplasms.
- They are closely related to pilocytic astrocytomas and typically affect very young children, but can occur in older children and adults.
Observation:
- A 12-year-old male presented with focal seizures, headache, and vomiting.
- Imaging revealed a left temporal lobe lesion with solid-cystic components and peripheral enhancement.
- Histopathology showed a characteristic tumor with bipolar cells, myxoid background, brisk mitoses, and necrosis.
Findings:
- The tumor exhibited strong GFAP and scattered p53 positivity, with EMA negativity.
- Ki-67 proliferation index was notably high, ranging from 30-40%, the highest reported.
- No Rosenthal fibers or eosinophilic granular bodies were observed.
Implications:
- This case underscores the aggressive nature of pilomyxoid astrocytomas, even in older pediatric patients.
- The high Ki-67 index may correlate with tumor aggressiveness and recurrence risk.
- Despite radiotherapy and temozolamide, the tumor recurred within two years, suggesting the need for further therapeutic strategies.
Abstract:
Pilomyxoid astrocytomas is an uncommon aggressive piloid neoplasm, closely related to pilocytic astrocytomas and typically presents in the very young but can occur in older children and rarely in adults. A 12-years-old male presented with focal seizures, headache and vomiting of 10 days duration. Computed tomogram showed a hypo- to hyperdense and peripherally enhancing, solid-cystic lesion in the left temporal lobe. Histopathological examination revealed a characteristic tumor composed of bipolar cells arranged in dyscohesive sheets, angiocentric pattern in a loose myxoid background, with brisk mitotic activity and foci of necrosis. No Rosenthal fibers or eosinophilic granular bodies were seen. The tumor cells showed strong GFAP and scattered p53 positivity, but were negative for EMA. Ki-67 positivity ranged from 30 to 40%, highest reported till date. The patient was treated with radiotherapy and concurrent temozolamide and the tumor recurred after two years.
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