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[Multifocal gliomas in cerebral hemisphere associated with von Recklinghausen's disease: case report]
1Department of Neurological Surgery, School of Medicine, University of Tokushima.
Abstract:
A case of intracerebral multifocal gliomas with von Recklinghausen's disease is reported. A 12-year-old boy was admitted to our hospital with an episode of convulsive attacks which were uncontrolled by anticonvulsants. CT scan and MRI revealed small well circumscribed tumors in the left frontal cortex and left parietal subcortex. Both of the tumors revealed low density in plain CT scan and low intensity in T1 weighted MRI. The vascularity of these tumors was poor in cerebral angiography. In other words these tumors were of a similar nature. The histology of the frontal tumor, which was totally removed surgically, showed typical pilocytic astrocytoma. The other tumor in the left parietal subcortex must also be included in the category of low grade glioma. In the sixth month after the operation, we could find neither recurrence of the frontal glioma nor enlargement of the parietal tumor, on CT and MRI findings. Immunohistochemically, the outer portion of the Rosenthal fiber in this tumor was positive for GFAP and S-100 protein, but the inner portion was negative, because the GFAP and S-100 protein there had degenerated. The cytoplasm of this tumor's cell was abundant with mitochondria and Golgi's bodies compared to the fibrillary astrocytoma. This case may be the first case of multifocal gliomas in the same cerebral hemisphere. We suggest that multifocal gliomas grow naturally, and over the years, tumors combine with each other and finally constitute a large type diffuse glioma.
Insights
This report details a rare case of multifocal gliomas in a single brain hemisphere in a patient with von Recklinghausen's disease. The study highlights the potential for these low-grade gliomas to grow and merge over time.
Area of Science:
- Neuro-oncology
- Pediatric Neurology
- Genetics and Oncology
Background:
- Reports a unique case of multifocal gliomas within the same cerebral hemisphere.
- Co-occurrence with von Recklinghausen's disease (neurofibromatosis type 1).
Observation:
- A 12-year-old boy presented with intractable seizures.
- CT and MRI revealed two distinct, low-grade gliomas in the left frontal and parietal regions.
- Cerebral angiography showed poor vascularity in both tumors.
Findings:
- Histopathology confirmed pilocytic astrocytoma in the frontal lobe, suggesting a low-grade glioma classification for both lesions.
- No recurrence or enlargement was observed six months post-surgery.
- Immunohistochemistry revealed specific GFAP and S-100 protein patterns in Rosenthal fibers, with abundant mitochondria in tumor cells.
Implications:
- This case may represent the first documented instance of multifocal gliomas in the same cerebral hemisphere.
- Suggests a natural growth pattern where multifocal gliomas may coalesce into diffuse gliomas over time.
- Contributes to understanding glioma development and progression in pediatric patients.