Chapter 33 - Pediatric oligodendroglioma

Abstract

Pediatric oligodendroglioma (OG) is a rare subtype of diffuse glioma. It contributes to <1% of brain tumor diagnoses in the pediatric population. These tumors are often molecularly distinct from their adult counterparts and therefore often lack the 1p/19q co-deletion or IDH mutation. Histologically, pediatric OG is similar to adult OG. Furthermore, there are histologically similar entities within the pediatric population, such as pilocytic astrocytoma, dysembryoplastic neuroepithelial tumor, and ganglioglioma. Given their rarity, the literature characterizing pediatric OG is sparse, and often limited to retrospective reviews of institutional experience. Initial treatment of these tumors is surgery, with chemotherapy and radiation being reserved for higher grade or recurrent disease. To date, there have been no trials dedicated specifically to the management of pediatric OG. The most commonly reported chemotherapy regimens in retrospective reviews are procarbazine, lomustine, vincristine (PCV) and “eight-drugs-in-one-day” (vincristine, hydroxyurea, procarbazine, lomustine, cisplatin, cytosine arabinoside, methylprednisolone, cyclophosphamide or dacarbazein; 8-in-1). Unfortunately, conclusions on the efficacy of these regimens for the treatment of pediatric OG is difficult and lacking given the small number of cases. In terms of overall prognosis for people diagnosed with pediatric OG, the overall 5-year survival for grade II OG in the pediatric population is estimated to be 90%, while for grade III anaplastic OG it is only 53%.