Adenoid cystic carcinoma of the temporal bone with distant metastases

Malignant tumors of the temporal bone are rare neoplasms. Adenoid cystic carcinoma (ACC) is the most common malignant tumor of minor salivary glands, while a quite rare tumor of the major. It is considered a slow-growing tumor with a course that is characterized by local recurrences and late distant metastases to lungs (80-90%), bone and liver. When metastases occur in bone especially the spine, the course of disease is usually fulminant. Intracranial involvement can occur by direct extension, hematogenous or perineural spread and represents an advanced stage of the disease. In this paper, we present a rare case of temporal bone ACC reporting for the first time simultaneous bony and pulmonary distant metastases. The origin, the pathology, the imaging techniques, the differential diagnosis, the treatment options and the prognosis of these tumors are discussed.