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A 3-year- old girl with altered mental status, gait difficulty, and vomiting

Ilya Lim, Irina Mikolaenko, Mark Cohen

    Brain Pathology (Zurich, Switzerland)
    |December 7, 2010
    PubMed

    Insights

    A rare pediatric brain tumor in a 3-year-old girl presented with neurological deficits and increased intracranial pressure. Microscopic analysis revealed a synaptophysin-positive tumor with high proliferation, suggesting a primitive neuroectodermal tumor.

    Area of Science:

    • Pediatric neuro-oncology
    • Neuropathology

    Background:

    • Brain tumors are a significant cause of morbidity and mortality in children.
    • Accurate diagnosis is crucial for effective treatment planning.

    Observation:

    • A 3-year-old girl presented with a 9-month history of focal neurological deficits and signs of increased intracranial pressure.
    • Radiological imaging revealed a large, heterogeneous tumor in the left parietal region, extending into the left lateral ventricle.

    Findings:

    • Microscopic examination showed a tumor with small true rosettes and a neuropil-like matrix.
    • Immunohistochemistry revealed positivity for synaptophysin, negativity for GFAP and neurofilament protein.
    • A high MIB-1 proliferation index (focally approaching 80%) indicated aggressive tumor behavior.

    Implications:

    • The findings suggest a diagnosis of a primitive neuroectodermal tumor (PNET) or a related embryonal tumor.
    • This case highlights the importance of detailed histopathological and immunohistochemical analysis in diagnosing pediatric brain tumors.
    • Further molecular studies may be warranted to refine the diagnosis and guide targeted therapy.

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