Adenoid Cystic Carcinoma

What is Adenoid Cystic Carcinoma?

Adenoid Cystic Carcinoma (AdCC) is a very rare form of cancer which can occur in various parts of the body. The neck and head are the most common sites of occurrence of this type of cancer. AdCC usually affects the major as well as the minor salivary glands. The distinctive histologic appearance is the main characteristic of this type of neoplasm.

The disease is often referred to by the following names:

• Adenocyst
• ACC
• Malignant Cylindroma
• Adenocystic
• Adenoidcystic

Adenoid Cystic Carcinoma Locations

These tumors mainly arise in the salivary glands of the head and neck. However, they can also develop on numerous other sites of the body. These include:

• Lung
• Brain
• Lacrimal glands or tear glands of the eyes
• Breast
• Skin
• Bartholin gland
• Vulva
• Paranasal sinuses
• Hard palate
• Nasopharynx
• Mucosal lining of mouth
• Tongue base
• Larynx or voicebox
• Trachea or windpipe
• Submandibular gland (located under the jawbone)
• Parotid gland (the largest salivary gland located on both sides of the face at the front of one’s ears)
• Sublingual glands (located under the tongue at the floor of the mouth)
• Bartholin glands

The cancer often tends to spread to other organs and body parts suck as liver, bones and kidney.

Adenoid Cystic Carcinoma Causes

The underlying causes of AdCC are still unknown. Researchers have proved that this disorder is not hereditary and is not inherited by a child from the mother. It has also been proved that the common risk factors for cancer, such as drinking alcohol and smoking, are not responsible for this type of cancer.

Naturally, researchers have assumed a working hypothesis regarding its causes. According to this theory, AdCC is caused by various environmental factors responsible for some genetic changes in the normal cells of the body that results in uncontrolled growth. Recently, researchers have found a common genetic change in a number of AdCC tumors. This new fused gene (named MYB-NFIB) is formed as a result of the fusion of the broken number 6 and number 9 chromosomes. This “translocation” is believed to be a key factor in the development of the tumor in many cases of the condition.

Adenoid Cystic Carcinoma Symptoms

The symptoms of the condition vary greatly depending on the location of the tumor. In some cases, AdCC patients may not have any evident symptoms. This makes it difficult to diagnose the cancer. Sometimes, patients experience signs of some associated disorder rather than those resulting from ACC. In most cases, however, the disease leads to the following symptoms:

• Painless lesions on the salivary glands which grows slowly underneath the facial skin or mouth lining
• Lumps in the salivary glands located under the throat, sinuses and the mucosal lining of one’s mouth
• Lumps under the tongue, at the base of the mouth and on the palate
• Some abnormality in a specific area of the mouth lining
• Difficulty swallowing
• Numbness of the palate, upper jaw, face and/or tongue
• Dull pain
• Hoarseness
• Change in voice
• Paralysis of one or more facial nerves
• A nodule or bump underneath the jaw or at the front side of the ear
• Breathing difficulty
• Proptosis and vision changes (in case of lacrimal gland tumors)

Adenoid Cystic Carcinoma Diagnosis

Doctors use various diagnostic tests to detect the presence of AdCC and determine its metastatic stage in a patient. Some examinations can even help in deciding the best treatment option. Like many other types of cancer, biopsy is the most effective test for making a diagnosis. Various imaging tests are also used for this purpose.

The following factors are considered before choosing the best diagnostic tool for AdCC:

• Age of patient
• Medical condition
• Type of cancer supposed to be present
• Severity of symptoms
• Results of previous tests

A thorough physical exam is always performed by the doctor to understand the patient’s medical condition. Additionally, the following tests are performed:

Biopsy

Biopsy is required for understanding the growth pattern of the tumor as it is important to determine the severity of the disease. ACC has three main variants of histologic growth patterns: cribriform, solid and tubular. The solid pattern is believed to have a relatively aggressive disease course.

MRI (Magnetic Resonance Imaging)

This imaging test is very useful for detecting any perineural spread of ACC.

CT scan (Computed Tomography Scan)

It is used for creating a three dimentional image of the patient’s internal organs in order to identify and observe the tumor.

PET scan (Positron Emission Tomography Scan)

It is another imaging test which uses a radioactive substance to produce images of different tissues and organs inside the body. It helps to determine the nature and severity of the condition.

Other tests, including radiology and ultrasound, are also used for this diagnosis.

Adenoid Cystic Carcinoma Differential Diagnosis

The differential diagnoses of AdCC located in different organs and tissues generally vary from one another. The differentials for the salivary and the lacrimal glands are mentioned below.

It is important to rule out the possibility of the following disorders when diagnosing Malignant Cylindroma of the salivary gland:

• Mucoepidermoid carcinoma
• Benign mixed salivary gland tumor
• Polymorphous low-grade adenocarcinoma or PLGA

The following conditions are characterized by symptoms similar to those of ACC of the lacrimal gland:

• Lymphoma
• Chronic Dacryoadenitis
• Reactive Lymphoid Hyperplasia
• Sarcoidosis
• Sjögren’s Syndrome
• Dacryops

Adenoid Cystic Carcinoma Treatment

The treatment for the various types of this cancer may vary, depending on various factors like the severity of the symptoms and the stage of the condition. Surgery and radiation therapy are two of the most commonly used treatment procedures.

Surgery

Surgical removal of the tumor is considered to be the best treatment option for AdCC. The surgery is performed by an oncologist, a surgeon specializing in removing this type of tumors. During the operation, the tumor is removed along with around 2mm of cancer-free surrounding tissue.

External-Beam Radiation Therapy

In this therapy, high-energy x-rays or some other similar particles are used for destroying the cancer cells. The therapy is generally used after the surgical removal of the tumor to clear any remaining microscopic cancer cells in the affected area. It is usually not applied prior to the surgery. According to many surgeons, using radiation therapy before surgery prolongs the healing time of the surgical wound.

Neutron and Proton Radiation Therapy

It is another type of radiation therapy that uses neutron particles to kill the cancer cells. The neutron and proton therapy is capable of causing greater damage to the cancer cells as the neutrons produce twenty to hundred times more energy compared to the other particles used for this therapy.

Chemotherapy

In this treatment measure, appropriate drugs are used for killing the malignant tumor cells. However, chemotherapy has a relatively limited scope in the treatment of ACC. Naturally, it is advisable for patients to inquire about their clinical traits before beginning the treatment.

Metastatic ACC

The condition is referred to as Metastatic AdCC if it spreads to another part of the body from its initial location. In these cases, the first object of the treatment is to slow or stop the spreading and growth rate of the tumor. The removal of the cancer is generally done by surgery.

Adenoid Cystic Carcinoma Prognosis

The outcome of AdCC often depends upon the site where the tumor is located. Surgical removal of this tumor generally has a positive outcome and patients can have a reasonably long and normal life. But it is not possible to completely cure the condition. Proper follow-up treatment is necessary to prevent any recurrence of the tumors.

Adenoid Cystic Carcinoma Life Expectancy

Individuals suffering from AdCC have varied life expectancy depending upon factors like the time of diagnosis of the condition and success in removal of the tumor. Generally, the life expectancy is not more than 7 to 10 years. In some cases, however, patients have been known to live for more than 20 years from the time they were first diagnosed with the cancer.

Adenoid Cystic Carcinoma Survival Rate

The 5 year survival rate of AdCC is around 70% while the 10 year survival rate is approximately 65%. In about 40% of the total cases, patients are known to survive for up to 15 years.

Adenoid Cystic Carcinoma Support Groups

A number of forums and foundations help AdCC patients cope with the disorder by providing proper facts and guideline. These include:

National Cancer Institute

6116 Executive Blvd

MSC 8322

Room 3036A

Bethesda, Maryland 20892-8322

United States of America

Tel: (301)435-3848

Email: cancergovstaff@mail.nih.gov

Website: http://www.cancer.gov

Support for People with Oral and Head and Neck Cancer, Inc.

P.O. Box 53

Locust Valley, New York 11560-0053

United States

Fax: (516)671-8794

Tel: (516)759-5333

Email: info@spohnc.org

Website: http://www.spohnc.org

Cancer Hope Network

2 North Road

Suite A

Chester, New Jersey 07930

Fax: (908)879-6518

Tel: (908)879-4039

Email: info@cancerhopenetwork.org

Website: http://www.cancerhopenetwork.org

Adenoid Cystic Carcinoma Prevention

The prevention of the disorder is not possible due to its unknown etiology. However, to avoid the development of these tumors, one can take certain general measures such as:

• Following a proper diet
• Keeping the stress level low
• Regular exercise
• Maintaining a healthy lifestyle

Adenoid Cystic Carcinoma Pictures

Here are some images displaying tissues affected by ACC.

Picture 1 – Adenoid Cystic Carcinoma

Picture 2 – Adenoid Cystic Carcinoma Image

As aforesaid, Adenoid Cystic Carcinoma is a potentially fatal disease which can cause death of the patients. Early diagnosis and timely treatment are very important to help the patient live as long as possible. At present, various clinical studies and researches are still on to find more effective treatment options for this cancer.