What is Adenoid Cystic Carcinoma?
Adenoid Cystic Carcinoma (ACC) is a rare type of cancer that occurs in the glands of the body. It is most commonly found in the salivary glands, but can also occur in other glands such as the lacrimal glands (tear ducts), the trachea (windpipe), and the respiratory tract. ACC is considered slow-growing cancer and often goes undiagnosed for a long period of time. It is very rare and the exact incidence of ACC is unknown, but it is estimated to occur in approximately 2-3 out of 100,000 individuals. The cancer is more common in women than in men and occurs more often in individuals over the age of 50.
Symptoms of Adenoid Cystic Carcinoma
Adenoid Cystic Carcinoma is an explicit symptomatic condition. Its symptoms may also be found in some other types of conditions, however, the signs in an integrated form are only caused by Adenoid Cystic Carcinoma. These signs are;
- Persistent or worsening sinus or nasal congestion
- Changes in voice or difficulty speaking
- Pain in the face or neck
- Unusual bleeding from the nose or mouth
- Swelling or lumps in the face or neck
- Changes in vision or double vision
- Pain when swallowing
- Numbness or tingling in the face or jaw
- Ear pain or hearing loss
- Fatigue or weakness
As early mentioned that these symptoms may also be present in other conditions, and it is important to consult a medical professional for proper diagnosis and treatment.
Causes and Risk factors of Adenoid Cystic Carcinoma
The exact cause of Adenoid Cystic Carcinoma is not clear, however, health experts are sure about some of the potential risk factors that may increase the chances of the condition.
- Age: Adenoid cystic carcinoma typically affects people over the age of 40, and the risk of developing ACC increases with age. The average age of onset is 60 years old, with a higher incidence in women than men. The risk of ACC is highest in individuals over the age of 70.
- Gender: Gender is one of these risk factors, with some studies indicating that ACC is more common in women than in men. The exact reason for this is not clear, but it may be related to hormonal differences between the sexes or to differences in exposure to environmental risk factors.
- Tobacco and alcohol use: It is slow-growing cancer, but it can spread to other parts of the body if left untreated. Tobacco and alcohol use have been identified as significant risk factors for ACC.Tobacco use: Smoking is the most significant risk factor for ACC, particularly for those who smoke for a long period of time. The chemicals in tobacco smoke damage the DNA in the cells and increase the risk of cancer. In people who smoke, the risk of developing ACC is two to three times higher than those who never smoked.Alcohol use: Alcohol consumption has also been linked to ACC. Heavy drinking, particularly for those who drink regularly, increases the risk of developing ACC. Alcohol can also interact with tobacco to increase the risk of cancer.The combination of tobacco and alcohol use has a more significant impact on ACC risk than either factor alone. People who both smoke and drink are at a much higher risk of developing ACC compared to those who only smoke or drink.It is essential to quit smoking and reduce alcohol consumption to decrease the risk of ACC. Regular check-ups and screenings can also help detect the early signs of ACC and increase the chances of successful treatment.
- Exposure to certain chemicals:Exposure to certain chemicals has been linked to an increased risk of developing ACC. Some of these chemicals include:
- Tobacco smoke: Tobacco smoke contains many harmful chemicals, including carcinogens that can increase the risk of ACC.
- Radon gas: Radon gas is a radioactive gas that is produced from the decay of uranium in the soil. Long-term exposure to radon gas has been linked to an increased risk of ACC.
- Wood dust: Workers in the wood industry who are exposed to wood dust are at a higher risk of developing ACC.
- Formaldehyde: Formaldehyde is a common chemical used in many industrial processes, and long-term exposure to it has been linked to an increased risk of ACC.
- Benzene: Benzene is a toxic chemical used in many industrial processes, and long-term exposure to it has been linked to an increased risk of ACC.
- Family history: Family history is considered a risk factor for ACC, as it is believed that a genetic predisposition may contribute to the development of this type of cancer.Studies have shown that people with a family history of ACC have a higher chance of developing the disease compared to those without a family history. People with a family history of ACC are advised to discuss their risk with their healthcare provider and consider genetic counselling and testing. Regular check-ups and screenings can help detect ACC early, allowing for more effective treatment options.
- Inherited genetic mutations: Inherited genetic mutations play a significant role in increasing the risk of developing ACC. Certain genetic mutations, such as those in the MYB, NF1, and CYLD genes, have been linked to a higher likelihood of ACC. These mutations are passed down from parents to their children through their DNA.
It is essential to note that many people with adenoid cystic carcinoma have no known risk factors. This means that the cause of this type of cancer is still largely unknown.
When to See a Doctor?
If you experience any of the following symptoms, you should see a doctor:
- Persistent sinusitis or nasal congestion that does not respond to antibiotics or other treatments
- Pain or pressure in the face or sinuses, especially in the nose or cheek area
- A lump or mass in the nose, mouth, or neck
- Changes in the voice or difficulty speaking
- Recurrent nosebleeds or drainage from the nose
- Hearing loss or ringing in the ears
- Pain in the jaw or teeth
Complications of Adenoid Cystic Carcinoma
Adenoid Cystic Carcinoma is a slow-growing cancer and can be treated easily, but if not treated, it can lead to serious complications like;
- Invasion of surrounding tissues: Adenoid cystic carcinoma can invade surrounding tissues and spread to other parts of the body, including the lymph nodes, bones, and lungs.
- Cranial nerve involvement: This type of cancer can affect the cranial nerves and cause symptoms such as facial numbness, drooling, and difficulty swallowing.
- Recurrence: Adenoid cystic carcinoma has a high rate of recurrence, even after surgical removal. This can lead to the need for additional treatments, such as radiation or chemotherapy.
- Disfigurement: Depending on the location of the cancer, surgical removal may result in disfigurements, such as a change in the shape of the face or a loss of function in the jaw.
- Pain: Adenoid cystic carcinoma can cause pain in the affected area, especially as the tumour grows and begins to put pressure on surrounding tissues.
- Breathing difficulties: Tumors located in the sinuses or near the airways can cause breathing difficulties and lead to sleep apnea.
It is advised to seek prompt medical attention if you experience any symptoms associated with adenoid cystic carcinoma, as early treatment can improve the prognosis and reduce the risk of complications.
Diagnosis of Adenoid Cystic Carcinoma
It is pretty difficult to diagnose ACC by its symptoms, because of its slow growth and due the signs found in other medical conditions. However, if your healthcare suspects you have ACC, they can recommend you some diagnostic tests to confirm the presence of the condition.
Adenoid cystic carcinoma is a type of rare cancer that affects the glands of the head and neck. Diagnosis is usually made through the following steps:
- Medical History: The doctor will ask questions about the patient’s medical history, symptoms, and any risk factors.
- Physical Exam: The doctor will examine the head and neck area for any lumps, swelling, or abnormalities.
- Imaging Tests: The doctor may request a CT scan, MRI, or PET scan to get a detailed view of the affected area.
- Biopsy: A small sample of tissue is taken from the affected area and examined under a microscope. This will confirm the diagnosis of adenoid cystic carcinoma.
- Staging: Once the diagnosis is confirmed, the doctor will determine the stage of cancer. This will help determine the best course of treatment.
- PET scan: Before having the PET scan you will be injected with a small amount of radioactive solutions. You will be asked to sit for 30-90 minutes so the solution can mover around your body. Many cancer cells will show up brighter on the scan. The scan takes around 30 minutes.
- Ultrasound: Soundwaves are used to create pictures of the inside of your body. You will be asked to lie down and a gel will be spread over the affected part of your body and then a small device (transducer) is moved over the area. The ultrasound takes about 15 minutes and is painless.
- Other Tests: The doctor may request additional tests such as blood tests, X-rays, or bone scans to determine if cancer has spread.
It is essential to accurately diagnose the condition and seek prompt treatment as adenoid cystic carcinoma can be aggressive and may spread to other parts of the body if left untreated.
Treatment of Adenoid Cystic Carcinoma
The treatment options for ACC vary depending on the stage and location of cancer, as well as the patient’s overall health. Here are some of the most common treatments for ACC:
- Surgery: The first line of treatment for ACC is often surgical removal of the cancerous tissue. Depending on the size and location of the tumour, this may involve a simple excision or a more complex procedure that involves removing lymph nodes and/or reconstructing the affected area.
- Radiation therapy: Radiation therapy may be used to shrink the tumour before surgery or to treat any remaining cancer cells after surgery. This treatment uses high-energy X-rays to kill cancer cells.
- Chemotherapy: Chemotherapy may be used to shrink the tumour or to treat any remaining cancer cells after surgery. Chemotherapy is a systemic treatment that circulates throughout the body and attacks cancer cells.
- Targeted therapy: Targeted therapy is a newer type of treatment that uses drugs to target specific proteins or pathways that drive the growth of cancer cells. This type of therapy is often used in conjunction with other treatments, such as surgery or radiation therapy.
- Observation: In some cases, ACC may grow slowly and may not cause symptoms for a long time. In these cases, the doctor may choose to observe the patient and monitor cancer rather than treat it right away.
One must discuss all of your treatment options with your doctor and to make a decision that is best for your individual situation.