Adenoid cystic carcinoma (AdCC) is a rare form of adenocarcinoma, which is a broad term describing any cancer that begins in glandular tissues. In general, AdCC is found mainly in the head and neck region. It can occasionally occur in other locations in the body, including the breasts or a woman’s uterus. AdCC most commonly occurs in the salivary glands, which consist of clusters of cells that secrete saliva scattered throughout the upper aerodigestive tract. The upper aerodigestive tract includes the organs and tissues of the upper respiratory tract, such as the lips, mouth, tongue, nose, throat, vocal cords, and part of the esophagus and windpipe. 

Symptoms

The initial symptoms of AdCC depend on the location of the tumor. Early lesions of the salivary glands may appear as painless, usually slow-growing masses underneath the normal lining of the mouth or skin of the face. Because there are many salivary glands under the mucosal lining of the mouth, throat, and sinuses, lumps in these locations could be from this type of tumor. Other symptoms may include:

• A lump on the roof of the mouth, under the tongue, or in the bottom of the mouth
• An abnormal area on the lining of the mouth
• Numbness of the upper jaw, palate, face, or tongue
• Difficulty swallowing
• Hoarseness
• Dull pain
• A bump or nodule in front of the ear or underneath the jaw
• Paralysis of a facial nerve

Diagnosis

• Biopsy: A biopsy is the removal of a small amount of tissue for examination under a microscope
• Imaging tests. Imaging techniques, primarily magnetic resonance imaging (MRI) or computed tomography (CT) scan, are useful to help doctors see the size and location of the tumor before surgery. A positron emission tomography (PET) scan may also be used to determine if the tumor has spread to other parts of the body. 

Treatment

• Surgery
• External-beam radiation therapy
• Neutron and proton radiation therapy
• Therapies using medication

Systemic therapy is the use of medication to destroy cancer cells. This type of medication is given through the bloodstream to reach cancer cells throughout the body. Systemic therapies are generally prescribed by a medical oncologist.

Common ways to give systemic therapies include an intravenous (IV) tube placed into a vein using a needle or in a pill or capsule that is swallowed (orally).

The types of systemic therapies used for AdCC include: 

• Chemotherapy
• Targeted therapy 

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