[Malignant progression of an anaplastic ganglioglioma into a glioblastoma multiforme--report on two cases and review of the literature].

BACKGROUND

Ganglioglioma is an uncommon type of primary brain tumors. In most of the cases the tumor demonstrates benign clinical behaviour with long-term patients' survival. We present two cases ofhistologically confirmed anaplastic ganglioglioma in which malignant progression into a glioblastoma multiforme was seen. CASE 1: A 36 year-old female with known Turner syndrome and recent history of single generalized seizure. The contrast-enhanced magnetic resonance imaging (MRI) showed an infiltrating lesion of the left frontal lobe. The tumor was excised partially and the histological result was anaplastic ganglioglioma (World Health Organization - WHO. gr. III). No additional neurological deterioration occurred after the procedure. A postoperative radiotherapy was performed with total dose of 60 Gy. The patient was symptom-free for one year taking her anticonvulsant medications. Control neuroimaging studies (computer tomography - CT, and MRI) were made because of progressive headache, right limbs weakness and speech disturbances. A local tumor recurrence was found and the patient underwent second operative intervention with gross total tumor resection. The histological result was glioblastoma multiforme (WHO gr. IV glioma). The patient improved after the procedure. An involvement of the contralateral cerebral hemisphere was found on control CT-scan ten months later. The patient died after one month, 23 months after her initial diagnosis. CASE 2: A 17 year-old female with recent history of progressive headache and three generalized seizures preceded by involuntary movements of the right limbs. MRI data for large, heterointense tumor lesion in the left frontal lobe was found. A subtotal tumor removal was made. The histological result was anaplastic ganglioglioma (WHO gr. ILL). The patients' headache and right side hemiparesis improved after the intervention. She was seizure-free taking her anticonvulsant medications. Thirty-day-long fractionated radiotherapy was performed with total dose of 60 Gy. The patient remained symptom-free for thirteen months after initial surgical procedure. During the next month the patients, partial motor seizures relapsed. Progressive headache, diplopia, and visual acuity impairment also developed. The ventriculoperitoneal shunt was implanted with neuroimaging data for internal hydrocephalus development. The patients' headache and visual disturbances improved after the procedure. Data for additional local tumor growth was found on control CT-scan one month later. The patient underwent subtotal excision of the lesion and the actual histological result was glioblastoma multiforme. The patient deteriorated after the intervention according to her right limbs paresis and died one month and half later. 20 months after the initial diagnosis.

CONCLUSIONS

The gangliogliomas are uncommon in clinical practice. The tumor behaviour may vary between the patients in spite of the similar histological characteristics which indicates the possible presence of different tumor subtypes.