Adenoid cystic carcinoma of the salivary glands. A study of ninety-three cases.

The present study reviews ninety-three cases of adenoid cystic carcinoma of the major (80 cases) and minor (13 cases) salivary glands occuring in ninety-one patients. This tumor is insidious in its presentation and subsequent clinical course with early perineural lymphatic invasion and late metastases. Many patients remain alive but with recurrent disease. The high incidence of recurrent disease (parotid gland 20 of 47, submaxillary gland 20 of 33, and minor salivary glands 7 of 11) suggests inadequate initial surgery at all anatomic sites. Misdiagnosis (16 cases) on frozen or permanent section has led to less than optimum primary treatment. The frequency of perineural lymphatic involvement with recurrence (11 of 19 cases) suggests that the tumor may be extending along the nerve roots in an anatomic area with extensive nerve distribution (the head and neck). Radical neck dissection, in our studies, has contributed to improved survival rates when the primary site has been the submaxillary gland. The treatment of recurrent disease has been primarily surgical (3 operations per patient), and palliation with prolonged survival has been achieved with radical excision of recurrence. The fifteen year determinate cure rate of 19 per cent in comparable to that reported in the world literature. Radiation therapy has been helpful in palliation but has not been curative. Radiation treatment was used for recurrence in twenty-seven patients, and the average time from radiation treatment to death was 3.8 years.