Adenoid Cystic Carcinoma is a rare or orphan disease that affects roughly 14,000 Americans alive today. While the condition is typically slow growing, it is also somewhat unpredictable and relentless. At any rate, it is a unique type of cancer that is typically considered a salivary gland tumor and is grouped with other oral cancers in statistical studies since it occurs predominantly in that area.
Early symptoms depend on the tumor’s size and location, and treatment depends on a variety of factors but may include one of several different options. In this post, we will discuss this disease in a detailed and comprehensive manner.
What Is Adenoid Cystic Carcinoma?
Adenoid cystic carcinoma (ACC) is a rare type of adenocarcinoma or cancer that begins in the glandular tissues. This specific type of cancer typically develops in the head and neck in one of the many parts of the major and minor salivary glands, such as the palate, nasopharynx, mouth lining, voice box, or windpipe. Even so, it can also occur in other locations in the body, including the breast and uterus.
ACC is typically characterized as having slow tumor growth. In fact, many times, this condition will show no growth for an extended period followed by an unexpected growth spurt. However, it can be more aggressive in some individuals. Metastatic adenoid cystic carcinoma typically spreads along the nerves or through the bloodstream. It generally only metastasizes to the lymph nodes in roughly 5 to 10 percent of cases.
Potential Causes of the Adenoid Cystic Carcinoma
The underlying cause of ACC is not yet known, but as of yet, no strong genetic or environmental risk factors have been detected. Researchers tend to agree, though, that a combination of genetic and environmental factors likely interact to cause any individual to develop cancer of any form.
Additionally, another potential cause of the cancer is represented by damage or changes to the DNA of specific cells. For example, several studies have shown that chromosomal abnormalities and genetic deletions are common across samples of adenoid cystic carcinoma.
While symptoms vary depending on the severity and location of the initial tumor, common indicators of the disease include lumps under the lining of the mouth or facial skin, numbness in the face or mouth, difficulty swallowing, hoarseness, and pain. In some cases, paralysis of the facial nerves may even present as an early symptom.
Quickcare or ER?
Because adenoid cystic carcinoma is typically a slow growing cancer, it does not often warrant immediate treatment at a quickcare or emergency room facility. Rather treatment typically centers on detection of the tumor and as effective a treatment plan as possible. With its unpredictable nature, regular monitoring is a necessary component of treatment to try to detect any new develops as early as possible.
Diagnosing Adenoid Cystic Carcinoma
Doctors use a variety of tests to locate and diagnose cancer. Likewise, one can use a number of tests to determine if the cancer has spread to another part of the body from where it originally started.
In most cases, a biopsy is the only sure way to determine whether a specific part of the body has cancer. During a biopsy, the doctor will take a small sample of tissue from the body to test in a medical laboratory. If a biopsy is not possible, the medical specialist may suggest additional tests to render a diagnosis.
When selecting a diagnostic test, your doctor will use several factors, including:
- The type of cancer suspected.
- Current signs and symptoms of the disease.
- The patient’s age and medical condition.
- The results of prior medical tests.
In addition to a physical examination, doctors utilize two groups of diagnostic tests to determine the presence and severity of this type of cancer.
Other tests may suggest that cancer is present, but a biopsy is the only definitive test for diagnosis. The doctor conducts the biopsy by using a fine needle or by surgically removing part or all of the tumor. Once the pathologist has removed the damaged tissue, he/she analyzes the cells to diagnose the disease. The pathologist will look for basaloid cells.
In addition to a biopsy, a doctor may order one of several imaging tests to determine where the cancer is located and if it has spread.
#1. An MRI
This test uses magnetic fields to produce detailed body images. This imaging test can also be used to measure the size of the tumor. In many cases, the patient will be given a special dye or contrast medium to create a clearer picture during the scan.
#2. A CT Scan
This assessment generates a 3-dimensional representation of the inside of the body utilizing x-rays taken from different angles. Once taken, a computer then combines the images to provide a detailed, cross-sectional view the will make any abnormalities or tumors show up.
#3. A PET Scan
A PET scan is another imaging test that serves to create pictures of the inner organs and tissues of the body. The medical specialist typically injects the patient with a small amount of a radioactive sugar substance.
The cells will absorb this substance; not just any cells, though. Only the ones that consume more body energy. Because cancer tends to use more energy, it absorbs more of the radioactive substance, which a scanner detects and creates a visual representation of.
After the doctor completes the diagnostic tests, he/she analyzes the results and shares the findings with the patient. From these tests, a care plan is created to fight the disease and restore the health of the patient.
What Is the Treatment for Adenoid Cystic Carcinoma?
The most common treatment approach for ACC is surgically removing tumors followed by radiation. In many cases, these “gold standard” of treatment stops the cancer, and the patient has no recurrence of the disease in his or her lifetime.
First, the doctor surgically removes the tumor. Then, the pathologist analyzes the tumor. Further on, he/she will report back that “negative” or clean margins of the investigation. The term “negative” means that the specialist removed all of the identifiable cancer. Or if cancer is still present at the surgical site, the pathologist will report “positive” margins.
Because of the number of cases that arise in the head and neck region, it is not uncommon for a tumor to be inoperable. In this case, the only form of treatment available is radiation treatment. Additionally, doctors might use chemo or other drug treatments in combination with radiation therapy. But this will happen in cases in which the doctor cannot surgically remove the tumor.
How Long Will You Have Adenoid Cystic Carcinoma?
While each case of ACC is unique, studies indicate the disease specific survival is 89 percent at five years. However, the survival rate at 15 years is less than 40 percent. Nowadays, we benefit from advanced treatments and medical care. Some of these examples are newer treatment protocols, as well as earlier diagnosis, developing research, complementary medicine, and other resources. Therefore, we can estimate that ACC cancer patients will only continue to experience extended life spans with a higher quality of life than before.
This is a rare disease. Still, nowadays, pathologists know a lot more about adenoid cystic carcinoma than ever before. While it typically occurs in the head and neck region, it can impact other parts of the body. Treatment typically focuses on removal of the tumor followed by radiation; however, depending on the location and size of the tumor, specialists might utilize other treatments such as adenoid cystic carcinoma chemotherapy. With the advent of new medical technologies, adenoid cystic carcinoma prognosis for individuals with any form of cancer is more positive than it once was.