Adenoid Cystic Carcinoma Organization International => FAQ

ACC (Adenoid Cystic Carcinoma) is a rare and unique form of cancer that is known to be unpredictable in nature, with a typical growth pattern of being slow and gradual, but over time can be progressive, insidious and relentless. ACC occurs most commonly in the oral cavity with 58% of the primary tumors beginning in that area, but it can actually occur in as many as 38 different organs in the body. There are some general tendencies, such as the propensity for it to spread into surrounding nerve tissue or metastasize to other areas of the body, yet each patient can experience their own diverse patterns and issues. Metastatic spread to the lungs is a common occurrence in a large number of ACC patients and it can also spread to other organs such as liver and bone. Of the approximate 566,000 new cases of cancer diagnosed each year in the U.S., only about 1228 of them are ACC. This is why it is classified as a rare or "orphan" disease. With it's tendency for indolent but relentless course, many patients with ACC survive for 5 years, only to have tumors recur and progress in a substantial number of cases. In a recent long term study conducted by MD Anderson Cancer Center with a study of 160 ACC patients, 89% of the patients survived for 5 years, but that was reduced to 40% at 15 years. For a more in-depth, expanded explanation of ACC in a printable PDF format Click Here.

What causes ACC?

As in the case of most types of cancer, it is not known what causes ACC. Diet, environmental conditions, stress and immune system weaknesses have all been looked at as possible contributors, but there is no known single cause that can be proven at this point. Medical researchers have discovered through DNA research that there appears to be evidence of abnormal characteristics of the DNA on chromosomes 6,12, 13 and 19 for ACC cells, and they are continuing the discovery process to find the exact genes that are defective and causing the ACC cancerous growth.

I was told it is a rare disease, but how rare is ACC?

Out of the 566,000 cases of all types of cancer that occur each year in the US, only about 1224 of them are ACC. To learn more about ACC statistics Click here

I was just diagnosed with ACC, how long do I have to live?

Each ACC case is unique. The location, stage, histology, treatment protocols followed and individual biology can all factor into disease progression and longevity. There are cases of complete control of primary tumor sites with no metastases lasting many years. There are also cases of rapid progression and aggressive metastatic behavior. In a study of 160 ACC patients followed from 1977 through 1996, disease specific survival was 89% at 5 years, but less than 40% at 15 years. Because ACC is most commonly found to be a slow growing cancer, most people who are initially diagnosed have many years of life, even in cases of more advanced tumor involvement. Of course, each case is different and there are so many factors that can affect the prognosis that each case can vary greatly. In medical studies that track particular groups of ACC patients, most studies do not go beyond 10 years, but some have tracked patients for 20 years or more. In many of the longer-term studies, 60% to 70% of the study groups are still alive 10 years after initial diagnosis, a 15-year survival rate of around 40% and some patients have been reported to live over 40 years after initial diagnosis. With the advent of newer treatment protocols, earlier diagnosis, scientific research, complementary medicine, better communications provided by the INTERNET, and more sophisticated techniques and equipment, it is expected that ACC cancer patients will continue to experience longer life spans with better quality of life than before. The two issues that appear to affect longevity are metastasis and infiltration.

Where does ACC typically show up the first time?

ACC primary tumors occur in 38 different organs of the body, with approximately 58% occurring in the head and neck region. Overall, salivary gland cancers account for about 3% of all malignant head and neck cancers in North America, and out of that 3%, about 25% to 40% are ACC. Of all salivary gland tumors only about 10% to 15% are of minor salivary gland origin. ACC has a higher tendency to occur in hundreds of minor salivary glands than in the major salivary glands, which are the two large parotid glands in the cheek areas and the sub-mandibular and sub-lingual glands towards the bottom of the mouth. There have been documented cases of other primary sites besides head and neck and these include breast, bone, lung, liver and other organs, but these sites are more commonly the result of metastatic disease from tumors with the primary site being in the head and neck. ACC has a very low rate of infiltrating the lymph system, though it can occur.

Is any research being done to find a cure for ACC?

Our partner organization the ACC Research Foundation (ACCRF) is making major progress in the area of research. ACCRF has a broad Research Agenda which represents the best efforts, of the best minds, at the best cancer institutions in the world to find a cure for adenoid cystic carcinoma (ACC). As with many rare diseases, ACC poses some peculiar challenges for researchers. The lack of ACC specimens and models – the building blocks of research – has been accompanied by the lack of a logically-sequenced plan to access the most promising technological platforms of scientific discovery. These challenges are being met with ingenuity and determination in order to gain more widespread focus, greater resources and new insights. With the support of many patients and scientists, ACCRF is accelerating the development of improved treatments for ACC through this Research Agenda. To learn more about the ACCRF you can visit their website Click here

How should I approach this type of cancer?

The NCI in its published PDQ (Physician Data Query) on Salivary Gland Cancer classifies ACC as a high-grade malignancy. Some sources say that it appears as a low-grade malignancy but it is best approached as a high-grade cancer. Because of its history of being unpredictable, most knowledgeable physicians approach it with aggressive treatment protocols regardless of its history of being slow growing. There are clearly documented studies and cases of some subgroups of ACC patients having a more aggressive and faster growth pattern, so it is usually a good idea to approach it with that in mind.

Will I be able to keep working while I deal with ACC?

Every person is different and the extent of their treatments is different. The type of treatment a person gets depends on the type and stage (how far the cancer has spread) of the cancer, the age of the patient, and other medical problems and treatments the person has had. Each drug or treatment plan has different side effects. It is hard to predict what side effects will occur, even if patients receive the same treatment. Some effects can be severe and others fairly mild. Although it is true that some people have a tough time with cancer treatment, there are also many who manage quite well and are able to work throughout treatment.

What are the different types of scans and tests that are recommended for ACC?

The most common tests that are conducted for cancer patients are physical exams, X-rays, MRI scans, CT scans and blood tests. A knowledgeable doctor who conducts a thorough physical exam can feel and see areas of swelling or change that can be an indicator of tumors.

An MRI (Magnetic Resonance Imaging) machine uses large magnets to provide an image of tissue similar to an X-ray but is more definitive. This is a particularly good imaging technique for soft tissue, lymph nodes, brain and spinal cord. CT scans (Computed Tomography) are similar to an MRI, but are actually multiple, pencil-thin X-ray images that are combined into a two-dimensional cross sectional image using a computer-assisted program. CT scans are generally better for showing hard tissue such as bones, though they are often used interchangeably for soft tissue as well, such as lungs. Both MRI and CT scans may use patient injected dye material to show contrast in vascular tissue. Finally, blood tests may be appropriate for watching for various abnormalities in blood levels such as elevated liver enzymes; which can be an indicator of potential problems in the liver. All of these tools are part of the discovery process in determining as much as possible about a particular case. There are other tests such as PET (Positron Emission Tomography) but due to the tendency of slow growth these have a more limited application.

What are the most common and effective treatment choices for primary tumors?

The most common, effective, documented and widely accepted conventional treatments for ACC are surgery and radiation, with one or both used depending upon the location of the tumor. In a majority of cases surgical removal of the tumor followed with radiation is the recommended treatment by many doctors. There are different views in the medical community about the best surgical techniques and best choices for radiation, but conventional wisdom still holds that removing the primary tumor is always the first choice to consider. A standing medical theorem is “Remove the cancer from the patient.” Because of the high propensity for perineural spread and the difficulty in achieving clean surgical margins, many doctors recommend a combination of surgical removal followed up with radiation treatment to the tumor region. Because ACC can spread microscopically through a region it can be difficult to detect, and the radiation treatment is an effective way of treating the area all around the original tumor bed including lymph nodes and major nerves in the head and neck.

What treatment center and/or doctor should I see?

This is a very individual decision that often requires some research. On the web page at http://www.accoi.org there is good information and the opportunity to join an on-line support group of members who are very knowledgeable about U.S. cancer centers that are familiar with ACC. Many doctors have never treated a case of ACC because it is so rare. There are particular doctors and facilities that have a history of treating ACC and these resources can be helpful. Talking with other people, as in the Online Support Group, who have undergone treatment can be an excellent source for finding treatment centers helpful for your situation. If you are not comfortable with your existing doctor you may need to make a decision about geographical limitations in seeking a knowledgeable ACC doctor. Find out which expenses are covered by your insurance and if you can afford to travel out of your region, state or country to seek medical care from a more experienced ACC doctor. Another viable option is to encourage your local physician to consult with a doctor with more knowledge and experience with ACC.

My current doctor doesn't seem to know much about this rare cancer. What can I do?

Getting a second opinion is always a good thing to consider regardless of the outcome with an initial consult with a doctor. But in the case of a doctor who does not know much, it becomes imperative for the patient to seek additional input. In some geographical areas it may be very difficult to find a physician who has some level of experience with ACC, but even if they don't, if they are open to learning more about it, you may be able to have them guide you in your next steps and treatment.

What if my doctor tells me I can’t have surgery because of the location of my tumor?

First, consider obtaining a second opinion. Surgical techniques have continued to improve and some surgeons have developed innovative surgical techniques that may make it possible to effectively remove a tumor. However, a number of tumors that involve critical tissue may be determined to be inoperable because of the overall risk to the patient. Examples are tumors near the optic areas, spine, liver, lungs and brain. Many of these tumors may be able to be effectively treated with various methods of radiation treatment. In these cases, an experienced Radiation Oncologist should be sought out for a detailed case review and medical opinion. Depending on tumor size, type, and location decisions will be made on the type, dose and targeted scope for radiation. . Some of the radiation treatments used effectively for inoperable tumors include neutron, proton, stereotactic radiosurgery and IMRT.

What is meant by clean margins, negative margins and positive margins?

The edge or border of the tissue removed in cancer surgery is called a margin. The margin is described as negative or clean when the pathologist finds no cancer cells at the edge of the tissue, suggesting that all of the cancer has been removed. The margin is described as positive or involved when the pathologist finds cancer cells at the edge of the tissue, suggesting that all of the cancer has not been removed.

What about chemotherapy and other drugs and treatments?

No single type or combination of chemotherapy agents has shown itself to be effective in any significant number of ACC patients or over any extended period of time. There have been limited success in using chemotherapy for slowing or stopping ACC tumor progression, but these studies have been short term. In a majority of the cases reported from ACCOI members, ACC began to grow again within a year or two at the most. There has also been limited success using anti-angiogenesis drugs. There are many clinical trials being conducted with combinations of known drugs, new experimental drugs, anti-angiogenesis and other methods.

What is a clinical trial?

Clinical trials are defined as trials to evaluate the effectiveness and safety of medications or medical devices by monitoring their effects on large groups of people. Clinical research trials may be conducted by government health agencies such as NIH, researchers affiliated with a hospital or university medical program, independent researchers, or private industry.

Usually volunteers are recruited, although in some cases research subjects may be paid. Subjects are generally divided into two or more groups, including a control group that does not receive the experimental treatment, and receives a placebo (inactive substance) instead, or receives a tried-and-true therapy for comparison purposes.

Typically, government agencies approve or disapprove new treatments based on clinical trial results. While important and highly effective in preventing obviously harmful treatments from coming to market, clinical research trials are not always perfect in discovering all side effects, particularly effects associated with long-term use and interactions between experimental drugs and other medications.

For some patients, clinical research trials represent an avenue for receiving promising new therapies that would not otherwise be available. Clinical research trials are sometimes lifesaving.

There are four possible outcomes from a clinical trial:

Positive trial -- The clinical trial shows that the new treatment has a large beneficial effect and is superior to standard treatment.

Non-inferior trial -- The clinical trial shows that that the new treatment is equivalent to standard treatment. Also called a non-inferiority trial.

Inconclusive trial -- The clinical trial shows that the new treatment is neither clearly superior nor clearly inferior to standard treatment.

Negative trial -- The clinical trial shows that a new treatment is inferior to standard treatment.

What about complementary and alternative medicine (CAM), naturopathic, holistic and integrative options?

There is more and more acceptance in the medical community for complementary, alternative and integrated treatments involving non-standard forms of treatment. “Integrative” treatments would be combining conventional treatments with other naturopathic treatments such as diet, imaging, body therapy, acupuncture, supplements, etc. A good place to start is by talking with other ACC patients to see what they have experienced. You may want to visit the Quackwatch web site at: http://www.quackwatch.com to research various alternative treatments since there are some cancer treatments that are suspected of being unproven, suspect or medical scams, and good research into any form of treatment is always recommended. Many of the alternative treatment protocols have shown evidence of increasing the immune system, increasing general health, lowering side effects of conventional treatments, increasing the healing process, and aiding in quality of life issues.

What about food choices, diet, nutrition and supplements?

Nutrition is one form of alternative treatment that many have followed either by itself or in conjunction with standard and alternative treatments. Because there is a suspected link between the immune system and cancer, it makes sense to help build the immune system through proper, balanced, healthy nutrition. Quality of life and general health can be increased greatly through proper nutrition as well and it is always a good thing to research the issues. You should discuss nutrition issues with your doctor if you will be going through a type of radiation or chemo treatment as well.

What are the meanings of the words infiltrate, metastasis and recurrence?

One of the characteristics of a malignant cancer cell is that it tends to infiltrate other normal tissue. This occurs in one of the three ways: It can spread locally into adjoining tissue, spread the through the blood stream, or come back in the same area originally treated. Many types of cancer including ACC can spread by growing directly into adjoining normal tissue next to the tumor such as muscle, nerves or bone. Though it is relatively rare for ACC, it can also enter the adjoining lymph nodes and then begin the progression of growing from lymph node to lymph node. One of the more common methods ACC spreads is through metastasis using the circulatory system. Some of the cells from the primary tumor can break off and be carried to other regions of the body in the blood stream where they find a receptive location and begin to reproduce at this new site. Due to the high level of blood supply the lungs and liver are the most common sites for ACC metastatic spread. This creates new tumors that are commonly called “mets”. Metastatic spread is the greatest cause of mortality from ACC, as opposed to death caused by the primary tumor. Recurrence is the failure to achieve local control at the primary tumor site. When recurrence takes place the tumor begins to grow again in the same location or region where it was previously treated.

What is meant by perineural invasion?

ACC has a very high tendency to microscopically infiltrate the adjoining nerve tissues around the tumor site, which is called perineural invasion. Nerve tissues microscopically appear like pipes with strands of wire running through them. ACC finds a “path of least resistance” by growing along the sheathing that surrounds those strands of wires inside the pipe. This creates a real challenge for both diagnosis and treatment since the cancerous cells are very small rather than a large cluster, and don’t necessarily show up on a MRI or CT scan. For head and neck tumors the major nerves that lead back to the brain should always be inspected with scans and monitored closely for any suspect infiltration. Some medical professionals have reported that in reviewing a scan, ACC appears to “skip” areas and can infiltrate nerves in areas more removed from the tumor site.

What can I expect long term for metastasis for ACC?

If the primary site of a tumor is treated with surgery and/or radiation treatment, local control at the primary site has a relatively good chance of being successful, though not in 100% of the cases. Approximately 60% to 80% of those having surgery followed up with radiation (depending upon tumor size, location and type of radiation) gain local control without recurrence at the original tumor site. Besides the perineural spread, ACC has also shown itself to be resilient and often metastasizes (travels) to other sites in the body, most commonly to lungs and liver. When distant metastasis does occur, it most commonly occurs 5 to 10 years after the initial discovery of the primary tumor, though it can happen sooner than 5 years or later than 10. Since the lungs are the most common place where metastasis occurs, it is important to have periodic lung scans done with X-rays or CT scans. MRI scans are sometimes prescribed for lung scans but generally they are too blurry to be useful. Many retrospective studies estimate that anywhere from 40% to 67% of all ACC patients will experience lung metastasis. Since ACC is a rare cancer with few long-term studies, some physicians have stated that if more long-term studies were conducted, metastasis may occur at an even higher rate than currently documented.

What are the treatment choices for metastasis to the lungs or liver?

These two organs present some extreme challenges for both the patient and the doctor. Since there are no effective systemic treatments for ACC (chemotherapy type agents that are delivered through injection, orally or inhalation), there are a variety of treatment choices that are considered. Depending upon the tumor location, size, and involvement with the organ there may or may not be any good choices. Lungs and liver are critical organs for sustaining life and since they have a very high vascular element, treatments are complicated. Some options when considering conventional treatments are surgery, stereotactic radiosurgery (a type of pinpoint accurate radiation treatment), other types of targeted radiation and Radiofrequency Ablations (RFA). Some people have had several surgeries and/or radiation treatments to the lungs to keep the larger tumors reduced or removed. This particular issue of lung and liver mets is one of the most daunting challenges facing the ACC community.

What is meant by histology and pathology?

Histology is the more generalized study of all types of cells using a microscope. Pathologists are the primary doctors who analyze a tumor sample from a biopsy. In a biopsy, a physician removes all or a section of a tumor through surgical means or by using a large bore needle to obtain a sample. The pathologist then analyzes the sample under a microscope and makes the diagnosis for a particular type of cancer such as ACC based upon the shape, structure, framework (stroma), color and growth pattern of the cells. ACC tumors are characterized by a distinctive pattern in which abnormal “nests” or cords of certain cells (epithelial cells) surround and/or infiltrate ducts or glandular structures within the affected organ. These structures are typically filled with a mucus-like material or contain abnormal fibrous membranes (hyaline membranes). Misdiagnosis of ACC can and does occur even with a knowledgeable pathologist. ACC tends to be a “mixed cell type” tumor with complex features and endless variations which complicate the diagnosis. A common misdiagnosis for ACC is to identify it as it’s histomorphologic relative called “pleomorphic adenoma” which is a benign (non-cancerous) type of tumor which has similar features.

What are the three principle types of ACC cells and why is it helpful to know what type of cells are in an ACC patient’s tumor?

Pathologists have identified three distinct types of ACC cells. They each have a different set of characteristics and have been named cribiform, tubular and solid. One of the common characteristics of an ACC tumor is that many times it will have a combination of two or even all three of these types of cells. This can complicate the diagnosis since the erratic patterns can be misdiagnosed as being a different type. The most common type of tumor histology is cribiform. There is some evidence from some of the larger medical studies that the solid type is a more aggressive form of ACC causing an earlier mortality rate, but without more studies that is not an absolute fact yet. If a person is able to find out their histology type for their diagnosis this information may help determine the course of treatment, especially if there is a high percentage of the solid type. This information may lead to a more aggressive treatment process being recommended. In order to be considered solid a tumor needs to consist of at least 30% solid cell type.

Are there different kinds of ACC?

There appears to be some evidence that two kinds of ACC exist. A majority of the cases appear to be of the more typical, slower growing kind and the second kind involves a much faster and more aggressive form. Pathologists have identified three distinct types of ACC cells. These have been named cribiform, tubular and solid. One of the common characteristics of an ACC tumor is that it many times will have a combination of two or even all three of these types of cells with many variations. This can complicate the diagnosis since the erratic patterns can be misdiagnosed as being a different type. The most common type of tumor histology is cribiform. There is some evidence from some of the larger studies that the solid type is a more aggressive form of ACC causing an earlier mortality rate, but that is not an absolute fact. It is an area worth pursuing if an individual is diagnosed with the solid tumor type. A tumor needs to consist of at least 30% solid pattern to be considered a solid tumor.

What is meant by staging or grading?

Fundamentally, staging is a means to identify how serious a particular cancer tumor by giving it some numerical classifications as compared to other similar tumors. This is another area that has some variations when looking at various medical papers and reports. The most common staging system for ACC is the AJCC (American Joint Committee on Cancer) Staging for Salivary Gland Cancer that was first proposed in 1975. In this staging system three elements are taken into consideration: Size of tumor with or without extension, lymph node involvement and distant metastasis at time of presentation of the original tumor. Size is graded on a beginning scale of T1 for a tumor 2cm or less in greatest dimension, up to a T4 which is a tumor more than 6cm in greatest dimension. An (a) after the T number indicates no local extension, a (b) indicates local extension. Lymph nodes are rated on a scale of N0 through N3 for involvement in lymph nodes and how nodes are involved. Finally, M0 means there are no known mets and M1 means there are mets. An example of a typical ACC staging could look like T3a, N0, M1. Based upon the combinations of these three elements, a tumor is then classified as being Stage 1, 2, 3 or 4, with stage 4 being the most serious. Many ACC patients are classified as stage 4 but can live another 15 to 20 years.

An additional staging/grading system that is utilized in some medical papers is based upon tumor histology alone as seen from the standpoint of the pathologist. An example of this would be grade 1: cribiform and tubular histomorphology only, grade 2: mixture of cribiform, tubular and solid growth pattern with less than 30% being solid and finally grade 3: greater than 30% and predominantly solid tumor histologic type.

What are the issues that will affect how long I live?

Besides the size of the tumor, lymph node involvement and the presence of distant mets used in tumor staging, there are a few other factors that have been suspected of affecting mortality. Perineural invasion when it involves a major facial nerve, solid histology type in over 30% of the tumor, multiple sites of mets, metastasis to bone and spine, and failure to gain initial local control of the original tumor have all been identified by various researchers as being possible contributors to a higher mortality rate. The fewer of these elements the better.

Once I’ve been treated, what types of tests should I have done and how often?

The conventional approach to ACC is to treat it as a high-grade malignancy and watch it close. Following treatment for the primary tumor it is generally recommended to have MRI or CT scans done for the original site to check for recurrence, and annual scans of some type for the lungs and/or liver since there is a high propensity for metastasis to these organs. Each patient should discuss these with their doctor to determine what course would be best for follow up.

I feel overwhelmed by this whole issue of cancer. What can I do about it?

It has been shown that those who live longer than others with the same type of cancer diagnosis exhibit some common characteristics and approaches. These “survivor” types embrace some or all of the following approaches to their diagnosis and treatment for ACC cancer.

Find a good doctor. Find a primary physician that you trust and like who will be your primary touch point for treating your cancer. This may be an oncologist, but it could very well be an ENT, family physician, surgeon or other medical professional. Chances are pretty high that you will have an oncology team from several specialty areas that will participate in your treatment, so having a comfortable relationship with one key doctor can be very helpful in this process. It is always recommended to get a second opinion for any treatment protocol.

Gather a support system around you. Friends, family, local support groups and on-line support groups can go a long way in providing you with love, information, support, and emotional help.

Take charge and manage your treatment. Survivors realize that they alone have their disease and they alone have the most influence on the outcome. Ask questions. Be persistent in getting answers that make sense to you. Challenge the establishment. Get second opinions.

Look for qualified assistants. If you aren’t good at record keeping or computers or asking questions or reading, find someone who can help in each of the areas that you find difficult. Families and friends are generally looking for something concrete that they can do to help. Give them a job and they will usually join your “team” and provide an amazing amount of help.

Get smart. There is a lot of good information for patients who want to know more about ACC and are willing to invest the time to obtain it. Having good information is a fundamental for making good treatment decisions. Books, reports, web sites, friends, support group members, videos and your physician are all potential sources of information in this battle.

Write down and gather information. Information is power. Keep a notebook or file and write down all of your questions and answers.

Get organized. Ask for and organize all of your medical reports. It is your right to have copies of any and all paper work generated such as lab reports, pathology reports, written scanning results, surgical reports, etc. Many institutions will not give you your film from scans, but at least you can document where they are held, and you have the right to have them sent to any physician who would need to review them.

Prepare for doctor appointments. Organize your time with your doctor appointments so you are prepared with written questions. By doing this you stand a better chance of getting all the answers you need from that physician.

Two heads are better than one. Have a friend or family member accompany you to every doctor visit. Two sets of ears are always better. If you aren’t good at taking notes, ask them to do that for you. After your meeting with the doctor, have a debrief meeting to discuss what you each heard. This discussion will usually produce some more questions. You also have the option of recording the doctor visit on a portable tape deck so you can play back the discussion at a later date.