A Case of Malignant Transformation of Juvenile Pilocytic Astrocytoma with Neurofibromatosis Type 1 / 대한소아신경학회지

ABSTRACT

Pilocytic astrocytoma (PA) is a World Health Organization (WHO) grade I tumor typically affecting children and young adults. In general, surgical resection for PA is thought to be curative, and tumor recurrence or malignant transformation is relatively rare. Herein, we report an extremely rare case of a malignant transformation of PA. Five years prior, the patient underwent subtotal resection for PA in the left suprasellar area and left side brain parenchyma of the third ventricle, followed by gamma knife radiosurgery for remnants of the tumor. Four years after surgery, recurrent PA developed, and surgery was performed through an anterior interhemispheric transcallosal approach. One year after surgery, a rapidly growing tumor in the left basal ganglia and hypothalamus was discovered, and subtotal removal was performed. The histological diagnosis was glioblastoma. To the best of our knowledge, malignant transformation of PA has not been reported in Korea. Through review of the literature, we found malignant transformation to be limited solely to patients treated with radiotherapy, which is an observation that should be validated in future studies.