Definition
- A rare malignancy arising from the secretory glands, most commonly seen involving the salivary glands.
Numbers
- Females (60% versus 40% in males)
- 4.5 cases per 100,000 individuals.
- Highest incidence of cases of ACC in the head and neck area seen in the fifth to sixth decade of life.
Location
- Head and neck
- Secretory cells of the salivary glands,
- Can also originate in other areas like the hard palate, nasopharynx, lacrimal glands, and tongue as well as the external auditory canal.
- Others
- Cases of ACC arising from glands in the breast, reproductive tract, skin, and trachea,
Aetiology
- Smoking and the use of alcohol have NOT been linked as potential risk factors for the development of ACC.
Genetic
- Most frequently encountered deletion of chromosome 1p35-36
- Translocation between chromosomes 6q and 9p
- Creates the MYB:NFIB gene fusion, which leads to the overexpression of the MYB oncoprotein
Clinical presentation
- A painless swelling in the head and neck region because of its high tendency to metastasize
- Facial pain and paralysis, particularly if it involves the facial nerve
- Tumors originating close to the skull base may cause ocular dysmotility, and cranial nerve palsies involving IX, X, XI, and XII
- Spread via perineural spread into cranial cavity
Prognosis
- ACCs generally carry a favourable prognosis with a 5-year survival rate of around 75%, but the 10-year survival rate drops acutely to 20%
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