Adenoid Cystic Carcinoma Frequently Asked Questions

FREQUENTLY ASKED QUESTIONS

Where can I find support for Adenoid Cystic Carcinoma?
Where can I find support for Adenoid Cystic Carcinoma of the Breast?
What is Adenoid Cystic Carcinoma (AdCC)?
How did I get AdCC? Is it because I smoked or was exposed to chemicals?
Is there any difference between adenocarcinoma and adenoid cystic carcinoma?
Who treats AdCC?
Why is it my doctor doesn't seem to have much knowledge of AdCC?
Should I be scared? How long will I live?
What are the initial treatment options for AdCC?
Is there research being done on AdCC?
What tests should be done to detect AdCC?
What follow up tests should be done after my initial treatments?
What are the chances of recurrence if the initial tumor is removed?
What is the difference between "recurrence" and "metastasis"?
What does "clean (free) margins" mean?
What does "perineural invasion" mean?
What is encapsulated vs. infiltrative?
What are the stages of AdCC?
What are the different types of AdCC tumors?
What is the prognosis for each stage and tumor type?
What types of treatment have been tried and succeeded?
What if I am not comfortable with my doctor?
Will I be able to continue working?
Should I get counseling?
What can I do immediately?

Where can I find support for Adenoid Cystic Carcinoma?

You can go to the Rare Cancer Alliance Forums for Adenoid Cystic Carcinoma and register for a free account to this organization and forum. Click on the register link at the top. If you have problems, use our Contact Form to ask for help.

Where can I find support for Adenoid Cystic Carcinoma of the Breast?

You can go to the Rare Cancer Alliance Forums for Adenoid Cystic Carcinoma of the Breast and register for a free account to this organization and forum. If you have problems, use our Contact Form to ask for help.

What is Adenoid Cystic Carcinoma (AdCC)?

Simply put, Adenoid Cystic Carcinoma (AdCC) is a rare form of adenocarcinoma, found mostly in the head and neck, and occasionally in the breast and other body sites. You will sometimes hear the words cylindroma, cribriform, adenocystic, and other terms used to refer to AdCC; depending on the geographic location of the pathology report. It is usually grouped with cancers of the minor salivary glands, but it can arise in a number of other sites. This cancer is known for having long indolent periods of no growth, followed by growth spurts, although it has also been known to act very aggressively in certain cases. The best word to use to describe this cancer is 'unpredictable'. Because scant statistics have been kept on this disease, the numbers are really too small to be described as conclusive of any disease pattern.

How did I get AdCC? Is it because I smoked or was exposed to chemicals?

Roll of the dice, shuffle of the cards -- we have not found anything in common that gave us this disease. There is no known cause, whether it be genetics or environmental causes. There may be one cause or several or a combination of several causes. The only thing clear is that it’s not your fault. Since no one knows what causes it, there is nothing you could have done to avoid it. There is no medical evidence relating this disease to any particular vices.

Is there any difference between adenocarcinoma and adenoid cystic carcinoma?

AdCC is a subcategory of adenocarcinoma, as are many cancers. Adenocarcinoma is the generic term for any cancer arising from glandular tissues. (For example, all the types of breast cancer are adenocarcinomas, as are gastric cancers or bowel cancers, with several different types, which usually have the word adenocarcinoma in their name.) There are several distinct types of salivary gland cancers, all of which are subtypes of adenocarcinoma. Among the less common salivary gland cancers is AdCC. Different types of adenocarcinomas behave differently in terms of overall aggressiveness, patterns of spread, etc.

Who treats AdCC?

First, you need a medical oncologist (cancer specialist) and you may need a surgeon and radiologist. You may also be referred to a specialty dentist or an Ear, Nose, and Throat (ENT) specialist.

Why is it my doctor doesn't seem to have much knowledge of AdCC?

The relative rarity of the disease makes it unlikely that your primary care physician has ever encountered it. Oncologists, pathologists and other specialists seldom see AdCC more than a few times in their careers, although, there are a small group of specialists who have seen many cases.

Should I be scared? How long will I live?

Chances are you are going to live for a long time, since AdCC is generally slow growing (although sometimes it can be very aggressive). You can make AdCC the primary factor in life, and you can live your life in fear. Alternatively, you can make yourself aware of the care you need to take. Make sure that you follow up with an oncologist or specialist on a regular basis. Keep abreast of developments. Plan for your future and then go live a full life.

What are the initial treatment options for AdCC?

Treatment for AdCC of the minor salivary gland is usually surgery in conjunction with radiotherapy, but there are situations where surgery alone or radiotherapy alone may be used. Various combinations of chemotherapeutic drugs have been tried, with varying degrees of success, but AdCC seems less responsive to chemotherapy than other cancers.

Is there research being done on AdCC?

Antiangiogenesis, research projects through NORD and private research projects through various facilities, etc., are ongoing.

What tests should be done to detect AdCC?

Regular screenings that would be used to detect any other form of cancer should be used. Current research has not proven any familial connection with AdCC.

What follow up tests should be done after my initial treatments?

In the case of AdCC of the breast there should be regular screenings of the breast area, bone scans and, if necessary, screening of other organs (i.e., lung CT scans and sonograms of the liver and kidneys, etc.). In the case of AdCC of the head and neck, a suggested follow up would include head and neck scans, bone scans, and sometimes brain scans. For ALL forms of AdCC, regular CT scans of the lungs should be done, since this is the most common site for metastasis.

What are the chances of recurrence if the initial tumor is removed with clean margins?

AdCC may recur at the primary site shortly after removal or many years after removal. Additionally, one source says that about 40% or more of individuals may experience metastasis to other sites, particularly the lungs. This is reportedly up to two times more likely with tumors of the submandibular salivary gland than with tumors of the parotid gland.

What is the difference between "recurrence" and "metastasis"?

A recurrence is the reemergence of tumors in the primary (original) body site. Metastasis is when the cancer travels to distant sites in the body.

What does "clean (free) margins" mean?

This term relates to the surgical excision of your tumor. The margin is the outside perimeter of the tumor removed. A margin is considered "clean" if it has a minimum of 2 mm. of tissue that shows no cancer cells.

What does "perineural invasion" mean?

It means that the AdCC has invaded the surrounding nerves in the vicinity of the original tumor. This is the most common way for AdCC to spread to other body sites (metastasis).

What is encapsulated vs. infiltrative?

"Encapsulated" refers to a tumor that is wholly confined to a specific area, surrounded by a capsule or localized. "Infiltrative" means a tumor that has grown through the boundaries of the area in which it originated and into surrounding tissues.

What are the stages of AdCC?

What are the different types of AdCC tumors?

What is the prognosis for each stage and tumor type?

What types of treatment have been tried and succeeded?

As of this time, the primary treatment used for AdCC is surgery with clean margins. This most certainly does not guarantee you freedom from recurrence or metastasis, although that does happen in some cases.

What if I am not comfortable with my doctor?

It is extremely important that you have confidence in your doctor and that you are able to communicate with him/her. With AdCC, as with other rare cancers, it is always best to get a second opinion on initial diagnosis. Hopefully, this will give you the choice of two specialists from the very beginning of your AdCC journey. If you are truly uncomfortable with your primary oncologist, then you should consider a change.

Will I be able to continue working?

Many people have been able to continue working for many years with AdCC. It is totally dependent on how the disease has affected your overall ability to function, either physically or emotionally.

Should I get counseling?

Counseling is a very private decision. Keep in mind that you have been diagnosed with a relatively rare cancer that often acts quite differently from more common cancers. This may at times, make you feel 'different' when talking with other cancer patients. Many of us have experienced this. Some of us believe that counseling has helped us to understand ourselves, our reactions, the reactions others, and our social interactions as we go through the many facets of this disease. However, counseling is not a mandatory element of your treatment.

What can I do immediately?

Learn how to research. Knowledge is power! Make sure you follow up on all treatments. Don’t be afraid to be your own advocate. Get a second opinion on original diagnosis and treatment options. Remember this is a rare disease. Communicate with others who have lived with the disease.