Adenoid cystic carcinoma

Adenoid cystic cancer (AdCC) is a rare type of cancer that can exist in many different body sites. It most often occurs in the areas of the head and neck, in particular the salivary glands; but has also been reported in the breast, lacrimal gland of the eye, lung, brain, bartholin gland, trachea, and the paranasal sinuses. It is sometimes referred to as adenocyst, malignant cylindroma, adenocystic, adenoidcystic, ACC, AdCC.

It is the second most common malignant salivary gland tumor overall (after mucoepidermoid carcinoma). 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^[1] of a cohort of 160 ACC patients, disease specific survival was 89% at 5 years but only 40% at 15 years,^[2] reflecting deaths from late-occurring metastatic disease.

Treatment

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 spread along nerve tracts. Adjuvant or palliative radiotherapy is commonly given following surgery. In some cases surgery is not an reasonable option, however radiation used alone may provide an effective treatment. Neutron therapy has been shown to be superior for some types of cancer including adenoid cystic carcinoma of the head and neck. 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.

Images

Histopathological image of adenoid cystic carcinoma of the salivary gland. Hematoxylin & eosin stain.

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.

See also

• Primary cutaneous adenoid cystic carcinoma

References

1. ^ Fordice, J. " Adenoid cystic carcinoma of the head and neck - Predictors of morbidity and mortality." Archives of Otolaryngology - Head and Neck Surgery 125:2 (February, 1999) pp. 149-152 [1]
2. ^ Christopher Moskaluk, MD, PhD, and Henry F. Frierson, Jr., MD. "Adenoid Cystic Carcinoma." [2]

• Neville, Damm, Allen, Bouquot. Oral and Maxillofacial Pathology. 2nd edition.

External links

• Adenoid Cystic Carcinoma Research Foundation
• FAQ for patients at Adenoid Cystic Carcinoma Organization International
• Adenoid Cystic Carcinoma Information
• Adenoid Cystic Carcinoma at Oral Cancer Foundation