This is a malignant tumour of salivary gland origin. It may occur in any of the paired salivary glands (submandibular, sublingual or parotid) but also may occur in the minor salivary glands in the upper aerodigestive tract. It is a high grade tumour that if cure  is the aim, the primary treatment is that of surgical resection. This means a parotidectomy if the site of origin is in the parotid gland a extended submandibular gland excision if the tumour is in the submandibular gland and a wide local excision if the tumour is anywhere else. The overall management of these tumours is best undertaken in a Head and Neck Cancer Center.

Radiotherapy can be used as an adjunct to surgery for microscopic disease control but is of little benefit if it is the only upfront treatment of this salivary gland tumour.

Unfortunately patients with adenoid cystic carcinoma can develop distant metastasis many years after surgery typically in the lungs but can also have local nerve spread referred to as perineural invasion.