Low grade ganglioglioma rapidly progressing to a WHO grade IV tumor showing malignant transformation in both astroglial and neuronal cell components.

Gangliogliomas are rare CNS neoplasms mostly occuring in young adults and are usually assigned to WHO grade I. The few cases of WHO grade IV gangliogliomas were either primarily malignant glio-neuronal tumors or underwent malignant progression from other WHO grades after radiotherapy. Herein, we present the case of a now 47-year-old female patient presenting with a benign ganglioglioma and showing a tumor recurrence 2 years later with an anaplastic ganglioglioma, assigned to WHO grade IV, with malignant transformation in both glial and neuronal components. The presented case is the first reported low-grade ganglioglioma rapidly progressing to a WHO grade IV glio-neuronal tumor not being associated with radiotherapy and showing malignant transformation in both astroglial and neuronal tumor cell components.