小児における攻撃的で再発性の神経節細胞腫、異常な遺伝子型を伴う稀な症例報告

Ganglioglioma is a glioneuronal tumor comprised of a combination of neoplastic ganglion and glial cells and associated with mutations in the MAPK signaling pathway. It is considered a central nervous system (CNS) World Health Organisation grade 1 tumor and is usually seen in a younger age group, particularly in the first two decades of life. The most common location is the temporal lobe, although they can occur anywhere in the central nervous system. They usually have an excellent prognosis, but around 5% of gangliogliomas are associated with aggressive behavior. Recurrence or malignant transformation in gangliogliomas is rare. We report an unusual case of recurrent aggressive ganglioglioma in a 12-year-old child with unexpected novel genotypic expression along with a brief review of literature.