Anaplastic cortical ependymoma in 10 months girl: A case report

Ependymomas arise from the ependymal cells that line the brain ventricles, and central canal. In children most of them are benign. However, cortical anaplastic ependymomas are very rare in pediatrics.

A 10 months girl presented with vomits for one week, increased head circumference, psychomotor development delay, left facial nerve (VII) palsy, and left hemiparesis 3/5. Magnetic resonance imaging (MRI) of the brain demonstrated a large parenchymal lesion filling most of the right hemisphere. She underwent a total excision of the lesion. The tumor had no connection to the ventricular ependymal lining. No adjuvant chemotherapy or radiotherapy was considered. The final diagnosis is Anaplastic Ependymoma (WHO Grade III).

Cortical anaplastic ependymomas are extremely rare. In pediatrics they affect frontal, frontoparietal, and parietal lobes, Temporal and occipital lobes are uncommon. Migration disorders from the germinal matrix and the differentiation of primitive neuroectodermal tumors along the ependymal lineage are considered two hypotheses that explain the pathogenesis of ectopic ependymomas.

Ependymomas should be considered a differential diagnosis in children, as the successful total removal of ependymomas in that age saves the patient from the need of adjuvant therapy such as radio-therapy or chemotherapy.