Adenoid Cystic Carcinoma (ACC) Treatment

Adenoid cystic carcinoma is a rare type of cancer that usually starts in the glands that produce saliva, such as the salivary glands. That said, it can also develop in other areas of the body, including the respiratory tract and tear glands. ACC is known for its slow growth, but over time, it can spread to nearby nerves or other parts of the body. This cancer often causes symptoms such as swelling, pain, or difficulty swallowing and breathing, depending on where the tumour is located. Despite its slow growth, it can be challenging to treat because of its tendency to invade nearby nerves and its potential to recur.

The exact cause of adenoid cystic carcinoma remains unclear, but some factors may increase the risk. These include:

Genetic Mutations

Alterations or mutations in the genes responsible for regulating cell growth can lead to the development of ACC. These mutations may cause normal cells to grow uncontrollably, forming a tumour. In some cases, genetic factors may be inherited, though ACC is typically not passed down in families.

Radiation Exposure

Previous exposure to radiation, particularly in areas near the head or neck, can increase the risk of ACC. This is why individuals who have undergone radiation therapy for other conditions may have a slightly higher chance of developing ACC.

Age and Gender

ACC can occur at any age but is most commonly diagnosed in adults, typically between the ages of 40 and 60. Both men and women can develop ACC, although its prevalence is slightly higher in women.

Environmental Factors

While the link between environmental factors and ACC is not yet well understood, exposure to certain chemicals or substances, especially in occupational settings, might increase the risk of developing this cancer.

Other Medical Conditions

In some cases, ACC has been linked to other health conditions, including chronic inflammation or infections in areas like the salivary glands, though more research is needed to fully understand these connections.

Adenoid cystic carcinoma can be difficult to detect early as it grows slowly and may not show obvious symptoms at first. It is essential to be aware of the signs, especially if you experience persistent issues that don't improve with time. At Max Hospital, we are dedicated to helping patients identify and manage rare cancers like ACC through advanced diagnosis and treatment.

The symptoms of adenoid cystic carcinoma depend on its location, but some of the common signs include:

• Swelling or Lump:The presence of a painless lump or swelling in areas like the mouth, neck, or throat is a frequent sign of ACC. This may occur in the salivary glands or other areas where the cancer may develop.
• Pain or Discomfort or Nerve Weakness: Since ACC can spread to nearby nerves, it may lead to numbness or weakness in the face, mouth, or neck.
• Difficulty Swallowing (Dysphagia): Tumours in the head or neck region can obstruct the throat, leading to difficulty swallowing.
• Hoarseness or Voice Changes: Tumours affecting the vocal cords or respiratory tract can lead to changes in voice or cause hoarseness.
• Breathing Problems:  If the tumour grows in the respiratory tract, it may cause breathing difficulties or a persistent cough, particularly if it obstructs the windpipe.
• Numbness or Weakness: As ACC may invade surrounding nerves, it may cause numbness or weakness in the face, mouth, or neck.

Diagnosing adenoid cystic carcinoma requires a thorough approach to accurately identify the cancer and understand its extent. At Max Hospitals, we leverage advanced diagnostic methods to ensure a precise diagnosis, laying the foundation for appropriate treatment plans. The diagnostic process typically includes:

Physical Examination

The initial step in diagnosing ACC often begins with a comprehensive physical exam. During this examination, the doctor will look for noticeable lumps or swelling in areas such as the neck, face, mouth, or throat, where ACC commonly develops. They will also discuss any symptoms the patient might be experiencing.

Imaging Tests

Imaging studies help determine the size, location, and possible spread of the tumour. Several types of imaging tests are used to gather detailed images of the body:

• CT Scan (Computed Tomography): A CT scan takes clear, detailed images of the body, helping doctors find the exact location of the tumour and see how it’s connected to nearby areas. This provides information on whether the cancer has spread to nearby lymph nodes or organs.
• MRI (Magnetic Resonance Imaging): MRI uses radio waves and magnetic fields to create high-quality images of soft tissues, which is particularly helpful in assessing tumours in areas such as the head, neck, and salivary glands. It assesses how far the tumour has spread, especially to critical nerves and blood vessels.
• X-ray: X-rays can be used to detect abnormalities in the chest or neck area. While they are not as detailed as CT or MRI, they help spot any initial signs of the tumour or check for any signs of metastasis (spread) to the lungs or bones.
• PET Scan (Positron Emission Tomography): In some cases, a PET scan may be used in combination with CT or MRI to assess how actively the cancer cells are growing, helping to evaluate whether the cancer has spread to other parts of the body.

Biopsy

A biopsy is the most definitive way to confirm a diagnosis of ACC. In this procedure, a small sample of tissue from the suspected tumour is removed and analysed under a microscope to look for cancer cells. The biopsy sample is then sent to a pathologist who will examine the cells for cancerous changes and determine whether the tumour is adenoid cystic carcinoma or another type of cancer.

Endoscopy

Endoscopy is particularly helpful for detecting tumours in the nasal passages, throat, or respiratory tract. In this procedure, a thin, flexible tube with a camera (known as an endoscope) is inserted through the nose or mouth to inspect the area where the tumour could be located. Endoscopy allows doctors to view the inside of the body in real-time, and if an abnormal growth is seen, a biopsy may be performed during the procedure.

At Max Hospitals, treating adenoid cystic carcinoma involves a careful approach. A dedicated team of oncologists, surgeons, and medical professionals work together to provide the best care for each patient. The treatment options for ACC include but are not limited to:

Surgery

Surgery is the most common treatment for ACC. The aim of the surgery is to completely remove the tumour, if possible. The surgeons at Max Hospitals have extensive experience in removing ACC tumours, especially those in the head, neck, or salivary glands.

• Tumour Removal: The surgeon aims to remove the tumour and some healthy tissue around it to lower the risk of the cancer coming back.
• Reconstructive Surgery: If needed, surgery may also be done to repair the area affected by the tumour, such as the mouth or face. If nerves are involved by tumour then rehabilitation for nerve weakness may also be required.
• Lymph Node Removal: If the cancer has spread to nearby lymph nodes, they may be removed as well.

Radiation Therapy

Radiation therapy uses powerful high energy rays to destroy cancer cells or shrink the tumour. It can be used after surgery to treat high risk tumours or for tumours that cannot be fully removed.

• External Radiation: This is the most common type of radiation, where the radiation is directed at the cancer from outside the body.
• Brachytherapy: In some cases, radiation is placed directly inside or near the tumour to deliver high doses to the cancer cells.

Chemotherapy

Chemotherapy involves using drugs to destroy cancer cells or prevent them from growing. However, ACC typically doesn't respond well to chemotherapy alone, but it may still be used if the cancer has spread or cannot be removed through surgery. Sometimes, chemotherapy is combined with radiation to help treat ACC more effectively.

Targeted Therapy

Targeted therapy is a modern treatment that targets specific areas of cancer cells to block their growth. It’s often used for advanced cases of ACC or if the cancer comes back after other treatments.

Palliative Care

Palliative care aims to help patients manage symptoms and enhance their quality of life, particularly when the cancer is in its advanced stages. It is used alongside other treatments to make the patient more comfortable.

• Pain Management: Palliative care helps reduce pain and discomfort caused by the tumour.
• Supportive Care: Supportive care involves counselling and emotional support to assist patients and their families in managing the challenges of living with cancer.
• Symptom Relief: Palliative care also helps manage symptoms like fatigue, difficulty eating, or breathing problems.

At Max Hospitals, doctors assess the stage of the cancer to determine the most effective approach for each patient.

ACC may cause several complications, especially if not treated early. At Max Hospitals, we take care of these complications to help improve patients' health and quality of life.

• Recurrence: Sometimes, the cancer can come back after treatment. We monitor patients closely to catch it early and treat it right away.
• Metastasis: If the cancer spreads to places like the lungs or bones, we use treatments like chemotherapy or radiation to manage it.
• Nerve Damage: If the tumour is near important nerves, it can cause problems like facial weakness, difficulty speaking or swallowing. Our experienced doctors try to avoid nerve damage, and if it happens, we offer rehabilitation and support to help restore function.
• Pain: Some tumours cause pain, especially in the head or neck. Our team works to make patients feel more comfortable and manage pain.
• Breathing and Swallowing Problems: ACC can affect the throat or breathing. We provide treatments to make breathing and swallowing easier.

Currently, there is no known way to completely prevent ACC, as the exact cause of the cancer remains unclear. Although there is no guaranteed way to prevent ACC, the following tips can help reduce the risk of developing cancer:

• Avoiding Tobacco Use: Smoking and tobacco use are connected to an increased risk of many cancers. By avoiding these habits, the risk of cancers in areas like the head and neck, where ACC is commonly found, can be lowered.
• Protecting from Radiation: Limiting exposure to unnecessary radiation, such as from certain medical imaging tests or environmental sources, may reduce the risk of cancers like ACC. Always follow guidelines to ensure that radiation exposure is necessary and minimal.
• Regular Check-ups: Early detection can help manage cancers more effectively. Regular medical check-ups, especially for those with a family history of cancer or other risk factors, may help identify issues before they develop into more serious problems.
• Healthy Lifestyle Choices: Eating a well-rounded diet, keeping a healthy weight, and staying active can support overall health and may help reduce the risk of different types of cancer.

If you are concerned about your risk, it is important to consult with a doctor. At Max Hospitals, our specialists can guide you on the best practices for cancer prevention and early detection.

1. What is the prognosis for someone with Adenoid Cystic Carcinoma?

The prognosis for people with Adenoid Cystic Carcinoma depends on things like how far the cancer has spread, how early it was found, and how well the treatment works. Finding and treating the cancer early can improve the chances of success. Even if the cancer is more advanced, treatment can still help control it and manage symptoms for a long time.

2. Can Adenoid Cystic Carcinoma affect children or teenagers?

Adenoid Cystic Carcinoma is more prevalent in adults, particularly those aged 40 to 60. It is very rare in children or teenagers, but it can still happen.

3. Is surgery the only option for treating Adenoid Cystic Carcinoma?

Although surgery is the most common treatment for Adenoid Cystic Carcinoma, it is not the only option. The treatment plan may also include radiation therapy, chemotherapy, or targeted therapy based on the tumour's size, location, and stage. It is created based on the specific needs of the patient.

4. Will I need to stay in the hospital for a long time after surgery?

Hospital stays after surgery for Adenoid Cystic Carcinoma can differ for each individual. Recovery time in the hospital can vary; some patients may only need a few days, while others may require a longer stay, depending on the complexity of the surgery and their general health.

Your doctor will provide details about your expected recovery time.

5. Are there any long-term effects of treating Adenoid Cystic Carcinoma?

Most people recover well after treatment, but some may experience long-term effects such as difficulty swallowing, speech problems, or nerve issues, especially if the tumour is located near vital nerves.

6. How do I know if my Adenoid Cystic Carcinoma is spreading?

In some cases, the cancer may not show obvious symptoms when it begins to spread. Regular check-ups, imaging tests, and physical exams help doctors monitor whether the cancer has spread to other areas of the body. If you experience new or unusual symptoms, it’s important to discuss them with your doctor as soon as possible.

7. Is it possible to live a normal life after treatment for Adenoid Cystic Carcinoma?

Many people live a normal or near-normal life after treatment for Adenoid Cystic Carcinoma, especially if the cancer is detected early and treated effectively. It’s important to maintain a healthy lifestyle and follow medical advice to support your recovery.

Reviewed By Dr. Akshat Malik, Principal Consultant, Cancer Care, Surgical Oncology, Head & Neck Oncology, Robotic Surgery, 14 January 2025.