Background: Adenoid cystic carcinoma (ACC) is a rare neoplasm of the exocrine glands. Because of its tendency for skull base involvement and intracranial spread, ACC should be diagnosed promptly by the neurosurgeon, although discrimination from meningioma is often difficult. Radical resection of such tumors requires familiarity with complex craniofacial approaches.
Case description: An unusual case of dumbbell-shaped ACC centered over the planum sphenoidale is presented. Regular margins and neuroimaging features suggested a preoperative diagnosis of meningioma. The lesion was gross, totally resected in a two-staged procedure through frontobasal and transfacial approaches, with good functional and aesthetic result.
Conclusion: The epidemiologic, histologic, and clinical features of ACC are reviewed. ACC is rarely encountered by the neurosurgeon; however it should always be considered in the differential diagnosis of skull base tumors. Interdisciplinary surgical approaches represent the major advance in the treatment of these complex neoplasms.