Adenoid cystic carcinoma of the breast. Data from the Connecticut Tumor Registry and a review of the literature.

The case records of the Connecticut Tumor Registry were reviewed from 1952-1982. There were 37 cases of adenoid cystic carcinoma of the breast (ACC) from a total of 40,350 invasive breast tumors. Patient survival, complications, and pathologic sections were reviewed. Only 14 of 27 surgical pathology slides available for review could be confirmed histologically as ACC. All patients were white females with a mean age of 64 years. The tumor remained localized to the breast in all cases. Nine patients had either radical or modified radical mastectomy, four patients had either simple mastectomy or lumpectomy, and one patient refused treatment. There was no evidence of axillary node involvement, metastases, or local recurrence after excision. At the time of follow-up, nine patients were alive and disease free and four died of disease unrelated to their breast cancer. The one patient who died of breast cancer had a radical mastectomy and survived 11.7 years after diagnosis. It is concluded that ACC has a favorable biologic behavior characterized by a prolonged clinical course and good prognosis. Simple mastectomy is all that is required as initial treatment, and a chest x-ray and thorough physical examination looking for local recurrence is all that is needed for follow-up.