Adenoid cystic carcinoma

Adenoid cystic carcinoma (ACC) is a rare form of cancer that primarily affects the salivary glands but can also occur in other parts of the body. It is characterized by slow growth, but it is also known for its potential to spread and recur.

Epidemiology

ACC accounts for approximately 1% of all head and neck cancers, and about 10% of all salivary gland tumors. It can occur at any age but is most commonly diagnosed in individuals between the ages of 40 and 60. There is a slight female predominance.

Pathophysiology

ACC arises from the secretory cells of the salivary glands. It is characterized by a distinctive pattern of growth, with tumor cells forming structures that resemble the normal architecture of the glands. The tumor cells produce excessive amounts of mucin, a sticky substance that can cause the tumor to adhere to surrounding tissues and make it difficult to remove surgically.

Clinical Presentation

The most common symptoms of ACC are a slowly enlarging mass, pain, and nerve involvement leading to facial weakness or numbness. Depending on the location of the tumor, other symptoms may include difficulty swallowing, changes in voice, or breathing difficulties.

Diagnosis

The diagnosis of ACC is typically made by biopsy of the tumor. Imaging studies such as CT scan or MRI may be used to determine the extent of the disease.

Treatment

Treatment for ACC typically involves surgery to remove the tumor, often followed by radiation therapy. In cases where the tumor cannot be completely removed, radiation therapy may be used as the primary treatment. Chemotherapy is generally reserved for cases where the disease has spread to other parts of the body.

Prognosis

The prognosis for ACC is variable, depending on factors such as the size and location of the tumor, the extent of disease at the time of diagnosis, and the patient's overall health. Despite its slow growth, ACC is known for its potential to spread and recur, and long-term follow-up is typically recommended.

See Also

• Salivary gland cancer
• Head and neck cancer
• Cancer