Intradural Extramedullary Ependymoma with Leptomeningeal Dissemination: The First Case Report in a Child and Literature Review

Abstract

Background: Primary intradural extramedullary ependymomas are very rare tumors and have never been described in children.

Case description: We report on an 11-year-old girl presenting with a 1-month history of neck pain, left arm weakness, paresthesia in the fingers of the left hand and gait disturbances. Magnetic resonance imaging on admission revealed an intradural extramedullary cystic lesion at the cervical level with craniospinal leptomeningeal nodules causing mild hydrocephalus. The multicystic lesion was surgically removed and neuropathologic examination revealed a World Health Organization grade II ependymoma. The patient underwent adjuvant radiotherapy with progressive reduction of the metastatic nodules. At her 2-year follow-up, the patient was symptom free with no evidence of recurrence on magnetic resonance imaging.

Conclusions: Although a rare entity, intradural extramedullary ependymomas should be included in the differential diagnosis of intradural extramedullary lesions in children. Surgical treatment seems to play a pivotal role in the prognosis of these rare tumors, with a possible role for adjunctive radiotherapy in the case of recurrence, anaplastic transformation, and metastasis.

Keywords: Children; Intradural extramedullary ependymoma; Magnetic resonance imaging; Radiotherapy; Surgery.