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Oligodendrogliomas are rare primary brain tumors. Significant attention has recently been focused on these interesting neoplasms because of their unique chemosensitivity and the durability of some of these responses. Surgery and radiation continue to play important roles in the treatment of oligodendrogliomas. Molecular genetic analyses have given new insight into the allelic deletions that distinguish these tumors and their progression from indolent to more aggressive forms. In the future, molecular genetic analysis may guide therapeutic decisions concerning patients with oligodendroglioma and may help us learn more about how to best treat other malignant brain neoplasms.