Türk Nöroşirürji Dergisi 2006 , Vol 16 , Num 3
A Case of Giant Supratentorial Extraventricular Anaplastic Ependymoma Causing Macrocephaly in Children
Adem YILMAZ1, S. Meltem CAN2, Okan KAHYAOĞLU3, Murat MÜSLÜMAN4, Yüksel ŞAHİN5, Yunus AYDIN6, Canan TANIK7
1,2,3,4,5,6Şişli Etfal Eğitim ve Araştırma Hastanesi Nöroşirürji Kliniği, İstanbul
7Şişli Etfal Eğitim ve Araştırma Hastanesi Patoloji Kliniği, İstanbul
Extraventricular ependymomas are rarely seen neoplasms among neuroepithelial tumors. Ependymomas are frequently encountered tumors like medulloblastomas and astrocytomas in childhood. They are divided into four groups, ie. subependymoma, mixopapillary ependymoma and anaplastic ependymoma.

A 3 year -old child whose head circumference is abnormally larger for his age referred to the emergency service because of development of nausea, blurred conscious, and right hemiparesia. Cranial magnetic resonance imaging demonstrated a solid tumoral mass lesion (101 x 72 x 67 mm) with intense contrast uptake, which contained tubular vascular formations, and occasional cystic areas. Commonly surgery is the first treatment option for such tumors. Adjuvant theraphy such as radiotherapy and/or chomotherapy is performed according to the evaluation of age and clinical manifestations of the patients. Tumor was dissected totally from the parenchyma using microsurgical approach. During operation tumor was found to be at a safe distance from lateral ventricle. Histopathological examination revealed perivascular pseudoresette formation, mitoses and massive calcifications. Immunohistochemical examination demonstrated poorly stained areas with glial fibrillary acidic protein (GFAP), and focal areas retaining epithelial membrane antigen (EMA) and cynoptophysine chromogranin dyes. These findings were found to be consistent with anaplastic ependymoma (Grade 3 according to WHO 2000 criteria). Postoperative neuroradiological examinations confirmed total extirpation of the tumor. Later on the patient also underwent radiotherapy. Anahtar Kelimeler : Macrocephaly, Anaplastic ependymoma, Giant tumor, Supratentorial extraventricular ependymoma