It is the third most common malignant salivary gland tumor overall (after mucoepidermoid carcinoma and polymorphous low grade adenocarcinoma). It represents 28% of malignant submandibular gland tumors, making it the single most common malignant salivary gland tumor in this region. Patients may survive for years with metastases because this tumor is generally well-differentiated and slow growing. In a 1999 study of a cohort of 160 ACC patients, disease specific survival was 89% at 5 years but only 40% at 15 years, reflecting deaths from late-occurring metastatic disease.
Primary treatment for this cancer, regardless of body site, is surgical removal with clean margins. This surgery can prove challenging in the head and neck region due to this tumour's tendency to spread along nerve tracts. Adjuvant or palliativeradiotherapy is commonly given following surgery. For advanced major and minor salivary gland tumors that are inoperable, recurrent, or exhibit gross residual disease after surgery, fast neutron therapy is widely regarded as the most effective form of treatment.Chemotherapy is used for metastatic disease. Chemotherapy is considered on a case by case basis, as there is limited trial data on the positive effects of chemotherapy. Clinical studies are ongoing, however.
Coronal MRI showing right parotid adenoid cystic carcinoma with perineural spread of tumor. The tumor originates in the right parotid gland and spreads along the trigeminal nerve via the auricuotemporal branch extending intracranially through the foramen ovale at the skull base towards Meckel's cave
Coronal MRI showing right parotid adenoid cystic carcinoma with perineural spread of tumor along the facial nerve extending to the stylomastoid foramen
Histopathological image of adenoid cystic carcinoma of the salivary gland. Hematoxylin & eosin stain.
Histopathological image of adenoid cystic carcinoma of the salivary gland. Immunostain for S-100 protein.
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Neville, Damm, Allen, Bouquot. Oral and Maxillofacial Pathology. 2nd edition.