Abstract
BACKGROUND: Medulloepithelioma is a rare primitive neuroectodermal tumor of the central nervous system, usually developing in childhood. Due to its rarity, the optimal management is still unknown. The prognosis is poor, especially when resection is incomplete. Adjuvant radiochemotherapy is often indicated. CASE PRESENTATION: We report a rare case of infratentorial medulloepithelioma in a 3 year old girl. She presented symptoms of increased intracranial pressure. On examination, she had coordination problems, ptosis and exotropia of the right eye. Magnetic resonance imaging demonstrated a large cerebellar vermix tumor. Immuno-histochemistry revealed a diffuse positivity for Vimentin and focal positivity for the epithelial membrane antigen, but Glial Fibrillary Acidic Protein and Synaptophysin were negative, the MIB-1 antibody was very high. She received postoperative craniospinal irradiation and died 7 months later. CONCLUSION: We describe the features (epidemiological, clinical, histological, immunohistochemical and therapeutic outcomes) of our case and confront it to literature data.