RESUMO
Chordomas are uncommon tumors occurring from remnants of the notochord. They are mainly localized in the sacrococcygium, the spine and the central skull base. Here we report a rare case of clivus chordoma presenting with cerebrospinal fluid(CSF)rhinorrhea. A 41-year-old man with a 11-year history of recurrent CSF rhinorrhea was found to have a clival lesion. Bone image CT revealed lytic bone destruction at the clivus. MRI showed a cystic mass with a membrane enhanced by gadolinium protruding into the sphenoid sinus thorough the bone defect. However, there was no solid component observed. The membrane was partially resected and the CSF fistula was repaired via endoscopic endonasal approach. The pathological diagnosis was chordoma, and there has been no recurrence for three years after the surgery. It is necessary for chordoma to be considered as a differential diagnosis for cystic lesions of the clivus when the cyst membrane is enhanced by gadolinium.