Intracranial adenoid cystic carcinoma of suprasellar region

Adenoid cystic carcinoma (ACC) is a relatively common head and neck tumor which grows slowly but locally aggressive and prone to recurrence. ACC most commonly arises in the major and minor salivary glands. Some rare cases of intracranial ACC with an unknown primary site have been reported. The authors present the first case of primary intracranial ACC of the suprasellar region. A 34 year-old female presented with one month's duration of visual disturbance and galactorrhea. Magnetic resonance image (MRI) showed intra- and suprasellar mass mimicking a craniopharyngioma. There was no evidence of invasion from an extracranial site. The operative findings showed the mass existed under the arachnoid membrane and seemed to originate from the pituitary stalk. Pathological diagnosis was ACC. After the first operation, local recurrence and CSF dissemination to the lower clivus occurred within two months. Recurrence repeatedly treated by Gamma knife radiosurgery 10 times and 4 times by surgery during a 3 year follow-up period. But total removal of the tumor at the first operation along with radiosurgery may control the lesion and prevent further recurrence.