Treatment of a recurrent intracranial anaplastic ependymoma – A case report

Abstract

Objective: Ependymomas represent about 10% of intracranial neoplasms in children. The proportion of cases defined as anaplastic is about 25%. Due to the survival rate after 5 years of 40–50%, as well as the tumor's high recurrence rates, further research into the efficacy of treatment strategies and adjuvant therapy is necessary. Extent of resection remains the most important determinant of survival in patients with ependymomas. Patient/Methods: We report a case of malignant ependymoma (WHO Grade III) first diagnosed in the age of 17 months in a child who has so far lived for over 9 years after onset of our interdisciplinary treatment Results: A 17 months old boy with vomiting presented with a tumor located in the fourth ventricle. The tumor was resected radically. Neuropathological examination revealed the diagnosis of anaplastic ependymoma, which was confirmed by two reference pathologies. He underwent a chemotherapy following the HIT-SKK-92 protocol and radiotherapy (54Gy). After 2 cycles MTX, Vincristin, Cyclophosphamid, Carboplatin, Etoposid and intrathecal MTX, a follow-up MRI revealed leucencephalopathy requiring immediate termination of MTX therapy. Within 27 months he developed supratentorial metastasis in both lateral ventricles and underwent multiple craniotomies for tumor resection as well as 2 stereotactic radiosurgeries for 3 targets (20Gy) and chemotherapy following the HIT-2000 and CWS-96 GPOH protocol. The last operation was in 10/2007. Currently, the patient is receiving temozolamide for 1 year. Besides a treated precocious puberty there are no major neurological and only mild neuropsychological deficits. Conclusion: Despite a poor prognosis due to the malignant ependymoma and the age of less than 3 years at onset this case demonstrates that the interdisciplinary treatment with a combination of surgery, chemotherapy and radiosurgery can achieve local control of anaplastic ependymoma without dramatic.