Extracranial relapse of an anaplastic oligodendroglioma in an adolescent: case report and review of the literature.

Oligodendroglioma is an uncommon childhood tumor and is more chemosensitive than other malignant glial neoplasms. Treatment involves gross total resection, and if anaplastic, radiation and chemotherapy. Distinct genetic alterations are associated with improved prognosis. We report a child with a low-grade oligodendroglioma that recurred as a high-grade oligodendroglioma and ultimately as extraneural systemic relapse. It was initially responsive to temozolomide, cyclophosphamide, etoposide, and carboplatin, perhaps predicted by combined loss of heterozygosity at 1p and 19q. This chemotherapy may be promising in treating malignant oligodendroglioma. However, he succumbed to progressive systemic disease. Positron emission spectroscopy scan was useful in sequentially assessing his disease.