ACCRF - Frequently Asked Questions

Adenoid Cystic Carcinoma (ACC) is a rare malignancy of the head and neck, typically originating in the salivary glands though it does appear in other primary sites, including the palate, nasopharynx, tongue base, larynx, trachea , lacrimal gland, breast and uterus. There are approximately 600 new cases of ACC diagnosed each year in the United States with a median age at diagnosis of 43. This cancer has a slow, and sometimes relentless, progression characterized by frequent recurrences and metastases to sites such as the lungs, liver and bones. This peculiar progression leads to relatively high 5-year cancer survival rates, but less optimistic outcomes as time from diagnosis proceeds. While infiltration to the lymph nodes is not common, ACC is one of the very few cancers that infiltrate and spread along nerves (perineural invasion). ACC has no known causes or links to smoking and alcohol consumption. The disease is slightly more prevalent in women than men. Standard treatment for ACC involves surgery followed by radiation. Currently, there are no drug therapies that have been proven to be effective in a large portion of ACC patients.

What causes ACC?

Currently, there is no known cause for adenoid cystic carcinoma. Cancer researchers point to genetic mutations as the underlying cause of all cancers. Only a small portion (perhaps 10%) of cancers is believed to be inherited, and ACC is not one of these inheritable cancers. Rather, as with the vast majority of cancers, ACC appears to develop from genetic mutations caused by a person´s environment, such as through exposure to radiation or carcinogens. Unlike some other cancers of the head and neck, ACC is not linked to tobacco or alcohol use or infection by the human papilloma virus.

Once researchers are able to identify the underlying genetic mutations that lead to a particular cancer, they may try to develop therapies that target the pathways used by the cancerous cells. ACCRF´s Research Agenda seeks to improve our understanding of the disease in order to accelerate the development of effective therapies.

Are there different types of ACC?

Traditionally, pathologists categorize cancers based on their histology, or how the tumor cells look under a microscope. Pathologists have identified three distinct growth patterns of ACC cells: cribiform, tubular and solid. One of the common characteristics of an ACC tumor is that it can have a combination of two or even all three of these growth patterns within the same tumor, with many variations. There is also some evidence that a tumor can progress from cribiform to solid over time, and that the solid type indicates a more aggressive form of ACC causing a higher relative rate of mortality. A tumor needs to consist of at least 30% solid pattern to be considered a solid tumor.

Over the past few years, researchers have been studying the genes and proteins that are peculiar to each type of cancer. In addition to categorizing tumors by histology, they are also categorizing cancers based on genomic or proteomic profiles. Researchers are finding that cancers that appear the same based on histology may actually have subsets with different genomic or proteomic profiles, suggesting that somewhat different treatments may be appropriate for different subsets of ACC patients (i.e. "personalized medicine"). ACCRF has initiated studies to identify the many profiles of ACC patients.

What is historical life expectancy following initial diagnosis?

Each ACC case is unique. The location, stage, histology, treatment protocols followed and individual biology 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. (Fordice) Improved diagnoses and treatments since that study may have improved patient outcomes, though not substantially.

What institutions and/or physicians have experience treating ACC?

Given the rare nature of ACC, many practicing oncologists see few, if any, ACC patients in the course of their careers. Accordingly, many ACC patients choose to visit major academic medical centers that are more likely to be aware of new treatments and might be spearheading clinical trials. These institutions also are instrumental in advancing research as well as determining the normal treatment guidelines ("standard of care ") for ACC patients.

Based on publicly available information and ACC patient feedback, the following institutions and physicians have experience treating ACC. Please note that ACCRF does not make recommendations about or endorse particular doctors or medical centers; nor does ACCRF make any representations about the qualifications or skills of such doctors and centers. Additionally, please note that this list is only inclusive of North American Institutions.

Surgeons	Location	Institution
Daniel Deschler	Boston, MA	Massachusetts Eye & Ear Infirmary
James Rocco	Boston, MA	Massachusetts Eye & Ear Infirmary
Douglas Mathisen	Boston, MA	Massachusetts General Hospital
Joseph Califano	Baltimore, MD	Johns Hopkins
Patrick Ha	Baltimore, MD	Johns Hopkins
Jeffrey Myers	Houston, TX	MD Anderson
Randy Weber	Houston, TX	MD Anderson
Frank Ondrey	Minneapolis, MN	U of Minnesota
Willard (Bill) Fee	Palo Alto, CA	Stanford
David Eisele	San Francisco, CA	UCSF
Jatin Shah	New York, NY	Memorial Sloan-Kettering

Radiation Oncologists	Location	Institution
Paul Busse	Boston, MA	Massachusetts General Hospital
Roy Tischler	Boston, MA	Dana-Farber Cancer Institute
George Laramore	Seattle, WA	U of Washington
Robert Douglas	Seattle, WA	Swedish Hospital (Cyberknife)
Allan Thornton	Bloomington, IN	Midwest Proton Radiotherapy Institute
Paul Read	Charlottesville, VA	UVA
Claudia Kirsch	Los Angeles, CA	UCLA
Elliot Abemayor	Los Angeles, CA	UCLA
Quynh Le	Palo Alto, CA	Stanford
Jeanne Quivey	San Francisco, CA	UCSF
Louis Harrison	New York, NY	Beth Israel NY

Medical Oncologists	Location	Institution
Robert Haddad	Boston, MA	Dana-Farber Cancer Institute
John Clark	Boston, MA	Massachusetts General Hospital
Neil Hayes	Chapel Hill, NC	U of North Carolina
Everett Vokes	Chicago, IL	U of Chicago
Ezra Cohen	Chicago, IL	U of Chicago
Victoria Villaflor	Chicago, IL	U of Chicago
Ann Mellott	Chicago, IL	Northwestern
Mark Agulnick	Chicago, IL	Northwestern
Merrill Kies	Houston, TX	MD Anderson
George Blumenschein	Houston, TX	MD Anderson
Lillian Siu	Toronto, Canada	Princess Margaret Hospital
Renato Martins	Seattle, WA	U of Washington
David Pfister	New York, NY	Memorial Sloan-Kettering
Arlene Forastiere	Baltimore, MD	Johns Hopkins

These additional resources may be helpful in understanding which institutions have more experience with cancer in general.

The National Cancer Institute designates a total of 63 Centers in the United States as either Cancer Centers (24) or Comprehensive Cancer Centers (39). NCI-designated Centers are characterized by scientific excellence and the capability to integrate a diversity of research approaches to focus on the problem of cancer. The list may be accessed at this link:

The National Comprehensive Cancer Network (NCCN) is a not-for-profit alliance of 21 of the worldís leading cancer centers. NCCN´s resources include its Clinical Practice Guidelines in Oncology™ and its Drugs and Biologics Compendium™. The list of NCCN member hospitals may be accessed at this link:

While there are many other hospitals that might provide excellent care, these resources are a good starting point for patients seeking treatment in the United States.

Finally, the online support groups below include members who are knowledgeable about U.S. cancer centers that are more familiar with ACC. Patients may post a message asking for guidance from these groups.

How do physicians decide upon treatment protocols?

The National Comprehensive Cancer Network (NCCN) publishes its Clinical Practice Guidelines in Oncology™. These guidelines generally define the "standard of care" for different types of cancer. The preponderance of ACC cases occur in the salivary glands and so guidelines may be found within the Head & Neck Cancer section of the NCCN guidelines.

The NCCN practice guidelines for ACC call for surgery, (if possible), followed by radiation. When surgery is not possible, some patients have radiation therapy alone. Neutron beam radiation therapy has been used for some ACC patients – especially those with unresectable tumors. To date, no drugs (chemotherapy regimens) have been found to be effective in preventing the recurrence of ACC and thus chemotherapy is not typically recommended. If complete removal of the tumor is not an option for the primary site; if radiation alone has proved ineffective; or if the cancer has metastasized; then medical oncologists may suggest experimental chemotherapy or targeted drugs for the patient. It is important to note that none of these drug therapies are approved by the Food and Drug Administration (FDA) for the treatment of ACC. Finally, some recurrences of ACC at the primary site or metastases may be treated with other procedures to remove and control tumors. These include additional traditional surgeries; Radio Frequency Ablation; Cyberknife Radiation; Laser procedures; Cryosurgery, and Photodynamic Therapy.

Pages 12 through 16 of the above document deal with salivary gland cancers. There are also sections that deal with unresectable tumors (page 60) and recurrent cancer (page 61).

These guidelines should not be considered as definitive for all patients. Experienced physicians will be able to explain the treatment choices clearly to patients, and will be able to identify situations in which the guidelines may not apply. Each patient should consult with his or her physician before making any decision about treatment or the appropriateness of the NCCN guidelines.

What if my doctor tells me I cannot have surgery because of the location, and/or size 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 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. Read here for more information on types of Radiation Therapy.

What are the types of radiation therapy that might be used in my treatment?

Several types of the radiation therapy treatment methods may be considered for initial treatment of ACC.

External Beam Radiation Therapy
This type of radiation therapy is given through an external radiation source directed at the tumor. Types of external beam radiation include Photon (x-ray and gamma ray), Neutron and Proton. Photon radiation therapy has historically been the most common approach used in treating cancer. Many ACC patients receive this type of radiation post surgery. Neutron beam has been used in a significant number of ACC cases and has been published to be particularly effective in inoperable tumors; it is offered at the University of Washington in Seattle, WA, the NIU Institute for Neutron Therapy in Batavia, IL, the Gershenson Radiation Oncology Center in Detroit, MI, and iThemba LABS in Cape Town, South Africa. Proton radiation is expanding in popularity for cancer treatment in the U.S. Each of these forms of radiation has different strengths and weaknesses that need to be assessed by each patient with their healthcare provider.

Internal Radiation Therapy
Also known as Brachytherapy, this approach involves placing a radiation therapy source very close to or inside a tumor. The radiation source is placed inside an implant, seed or catheter and then inserted surgically or through catheters. This type of radiation may involve an inpatient hospital stay. Some ACC patients have been treated with both external and internal radiation therapy in the course of their initial treatment plans.

There have been recent advancements in technologies for "targeting" various external radiation sources in order to be most effective at destroying tumor cells, while salvaging surrounding healthy tissue. Two examples are:

Three-dimensional (3–D) Conformal Radiation Therapy
Three-dimensional (3–D) conformal radiation therapy uses computer technology to allow doctors to more precisely target a tumor with radiation beams (using width, height, and depth). Many radiation oncologists use this technique. A 3–D image of a tumor can be obtained using computed tomography (CT), magnetic resonance imaging (MRI), positron emission tomography (PET), or single photon emission computed tomography (SPECT). Using information from the image, special computer programs design radiation beams that "conform" to the shape of the tumor. Because the healthy tissue surrounding the tumor is largely spared by this technique, higher doses of radiation can be used to treat the cancer . Improved outcomes with 3–D conformal radiation therapy have been reported for nasopharyngeal, prostate, lung, liver, and brain cancers.

Intensity-Modulated Radiation Therapy (IMRT)
IMRT is a new type of 3–D conformal radiation therapy that uses radiation beams (usually x–rays) of varying intensities to deliver different doses of radiation to small areas of tissue at the same time. The technology allows for the delivery of higher doses of radiation within the tumor and lower doses to nearby healthy tissue. Some techniques deliver a higher dose of radiation to the patient each day, potentially shortening the overall treatment time and improving the success of the treatment. IMRT may also lead to fewer side effects during treatment.

Much of the information above is sourced from the National Cancer Institute. Further detailed information is available at the link below:

Are there chemotherapy and other drug treatments available?

Currently, there are no drug therapies that have been proven to be effective in a large portion of ACC patients. Accordingly, the Food and Drug Administration (FDA) has not approved any drugs specifically for ACC patients. Typically, chemotherapy and other drugs are not prescribed for ACC patients whose tumors were fully removed during surgery. Medical oncologists may offer cancer drugs as an option to ACC patients with extensive metastases or whose tumors could not be removed by surgery.

There are many online resources that describe clinically relevant drugs. If your physician discusses certain drugs with you, you may find these sources useful.

What are the different types of imaging tests used in the diagnosis, treatment and follow-up cycle?

The most common imaging tests ordered for ACC patients are X-rays, MRI scans, CT scans, and PET scans. Imaging “scans” are typically used in the initial diagnosis to detect the presence, size and location of tumor tissue. These technologies are also used in the treatment cycle of ACC. Images from these “scanners ” help surgeons and radiation oncologists in their “treatment planning” process. The images guide the extent of surgical resection required as well as the targeting and dose of any radiation therapy. Finally, these scans are typically done on 3, 6, or 12 month cycles after treatment to track tumor regression, progression or stability of tumor(s). Your physician(s) will pay close attention to comparative studies over time to gauge potential recurrence or metastases. The most common imaging technologies are described below.

CT scan
Computed tomography (CT) is a diagnostic procedure that uses special X-ray equipment to create cross-sectional pictures of your body. CT images are produced using X-ray technology and powerful computers.

During a CT scan, you lie still on a table. The table slowly passes through the center of a large X-ray machine. The test is painless. During some tests you receive a contrast dye, which makes parts of your body show up better in the image. Physicians may prefer to limit CT scans to annual or biennial scans due to the relatively high level of radiation exposure.

MRI scan
Magnetic resonance imaging (MRI) uses a large magnet and radio waves to look at organs and structures inside your body. Health care professionals use MRI scans to diagnose a variety of conditions, from torn ligaments to tumors. MRIs are very useful for examining the brain, spinal cord and other soft tissues.

During the scan, you lie on a table that slides inside a tunnel-shaped machine. Doing the scan can take a long time, and you must stay still. The scan is painless. The MRI machine makes a lot of noise. The technician may offer you earplugs.

Positron emission tomography:
PET. Positron emission tomography (PET) is an imaging test that uses a radioactive substance (called a tracer) to look for disease in the body. Unlike magnetic resonance imaging (MRI) and computed tomography (CT) scans, which reveal the structure of organs, a PET scan shows how the organs and tissues are functioning.

Your physician will typically ask you to come in for follow-up appointments every 3 to 6 months. These exams will typically involve a physical exam and an imaging study. Your physician(s) will pay close attention to comparative imaging studies over time to gauge potential recurrence or metastases. The most common imaging technologies are described here:

The approach to treating a recurrence to the original site or metastasis to another area of the body is dependent on location, size of the newly discovered tumor tissue as well as the impact of any previous surgery or radiation therapy to that part of the body. Beyond recurrence to the original tumor site, two common locations for metastasis are the lungs and liver.

Physicians will typically attempt to follow the NCCN clinical practice guidelines of surgery followed by radiation for recurrence or metastases. However, previous surgery or radiation to the recurrence site can make these options high risk. If surgery is not an option, radiation alone will be evaluated. If both surgery and radiation are ruled out due to the high risk of side effects, experimental drug therapies might be pursued.

There have been recent advancements in specialized radiation and other technologies to treat recurrence and metastases. Tumor size, location and previous treatments will play a key role in determining if these are viable treatments to pursue. Stereotactic radiosurgery, stereotactic radiotherapy, gamma knife and cyberknife -- all types or names for pinpoint accurate radiation treatment – are options you can discuss with your physician. Radiofrequency Ablation (RFA) and Photodynamic Therapy are other approaches that you may want to discuss with your physician(s).

Currently, there are no drug therapies that have been proven to be effective in a large portion of ACC patients. Accordingly, the Food and Drug Administration (FDA) has not approved any drugs specifically for ACC patients. However, that does not mean that there have not been good responses to particular drugs in particular ACC patients. Medical oncologists are able to prescribe drugs for ACC patients even if the FDA has not explicitly approved the drug for use in ACC patients (“off-label” prescriptions).

Increasingly, medical oncologists are attempting to customize therapies to the genomic or proteomic profile of each patient’s individual tumor (i.e. “personalized medicine”) in the hope that the treatments will become more effective and have fewer side effects. Rather than recommend merely one drug to all advanced ACC patients, a medical oncologist might add another drug based on the presence (or absence) of a particular protein in a patient’s tumor. For example, an ACC patient with a tumor that over-expresses the EGF gene (i.e. produces more of the epidermal growth factor protein than normal cells) might take a drug that targets (inhibits) that gene.

ACC patients with recurrences or metastases ought to consult with their medical oncologist to determine whether it is appropriate to undertake chemo-sensitivity or biomarker tests to guide their treatment. Such tests might inform the decision of which drug therapies to try or in which clinical trials to enroll.

There are many online resources that describe clinically relevant drugs. If your physician discusses certain drugs with you, you may find these sources useful.

Clinical Trials
Clinical trials are studies 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 the 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, receives a placebo (inactive substance) instead, or receives a tried-and-true therapy for comparison purposes.

Typically, the Food and Drug Administration (FDA) approves or rejects new treatments based on clinical trial results. The clinical trial system is highly effective in preventing obviously harmful treatments from reaching patients. However, it is often difficult for patients to understand and assess the nature and extent of all side effects, particularly those 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. Patients with difficult to treat or currently "incurable" diseases, such as AIDS or certain types of cancer, may want to pursue participation in clinical research trials if standard therapies are not effective. Clinical research trials are sometimes lifesaving.
There are four possible outcomes from a clinical trial:

(Clinical Trials definition above is based on information found at MedicineNet.com)

If you are considering participating in clinical trials, the overall process is described in this link:
CancerNet – Clinical Trials: Medicine’s Method for Answering Questions:

If you’ve decided to learn about existing trials that may be appropriate for ACC patients, these links may be useful:

ACC patients may find the following approaches to their diagnosis and treatment helpful. This list is a “toolkit ” of sorts based on the collective experience of some ACC patients.

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; good doctors are not offended by this reasonable step.

Gather a support system around you.
Friends, family, local support groups and online 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. Respectfully challenge your doctor’s recommendations. 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 are willing and able to invest the time to obtain it. Having good information is fundamental to making good treatment decisions. Books, reports, web sites, friends, support group members, videos, your local librarian and your physician are all potential sources of information in this journey. Find and use a library to help you answer your questions. Locate a library near you through this MedlinePlus map: http://www.nlm.nih.gov/medlineplus/libraries.html. You can save time by tapping into your librarian. Librarians search as part of their professional duties – put them on your healthcare team. Always discuss information you locate with your healthcare team.

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.

The Yahoo Email Information group moderated by ACCOI ( http://www.orgsites.com/ca/acco/) is an available resource for ACC patients who would like to connect with other patients. This community of more than 600 people offers tips on treatment plans, treatment side effects and much more. Patients can search through prior postings for information or start a discussion on a topic of interest.

The Rare Cancer Alliance Adenoid Cystic Carcinoma Forum is useful for ACC patients and caregivers at any time during their treatment and healing. The Forum currently has nearly 2000 posts on over 225 topics. There is a separate forum for ACC of the breast.

This advice should not substitute for the counsel of an experienced physician, but can provide additional perspectives.

There are several sources for support in dealing with the financial burden of cancer treatment. The following links may be helpful if you find yourself in need of financial assistance.

Some patients may choose to make complementary and alternative medicine treatments some part of their approach to living with ACC. "Integrative” treatments combine conventional treatments with other naturopathic treatments such as diet, imaging, body therapy, acupuncture, supplements, etc. Other ACC patients can be a valuable resource when considering these options. To ensure that the potential interactions between conventional and CAM therapies are monitored, it makes sense to inform your physician about all your treatments.

Most cancer patients assume that significant research is being carried out on their particular cancer. For those patients with the most common 20 or so cancers, that is true. For example, the National Cancer Institute, the American Cancer Society and other organizations spend billions of dollars each year to support basic research into breast, prostate, colon and lung cancer. Additionally, private pharmaceutical companies spend even more seeking new therapies for those types of cancer.

However, for those patients with one of the more than 100 rare cancers, there is little consolation. Although rare cancers (defined by the National Institutes of Health as those affecting fewer than 200,000 persons in the United States) make up one-quarter of all cancer cases and mortalities, the vast majority of research funding goes to the common cancers while rare cancers are ignored. Currently, the National Cancer Institute and the American Cancer Society do not fund any ACC-specific research.

Rare cancer patients might hope that treatments developed for common cancers will eventually help cure their disease; however, the trend in cancer research is to understand the genomic peculiarities of each tumor type in order to customize treatments for patients (i.e. “personalized medicine”). If researchers are not studying the rare cancers, they are not likely to find the correct, customized treatments for rare cancer patients. Rare cancer patients cannot wait patiently in the hope that a new breast cancer drug will “spill over” and help them. They must strive for their own cure.

Fortunately, rare cancer patients have the ability to overcome these challenges. The Internet has made it far simpler for dispersed patients to pool their knowledge and resources to raise awareness, researcher focus and funds. Technological advances have reduced the cost of conducting sophisticated scientific research. And pharmaceutical companies are being enticed into rare disease research in their search for new drug targets and orphan drug benefits. The tools exist to make major advances in rare cancer research, but success requires ingenuity, determination and a well-organized patient organization. Through its Research Agenda, ACCRF is leading the way for ACC patients.

A key element for accelerating ACC research is the availability of high quality, ACC tumor tissue. Researchers use this tissue to study the mechanisms of action responsible for tumor development. This understanding is critical to identifying targets for drugs and other therapies. You may want to consider donating part of your tumor for research in order to speed the discovery of improved therapies. It is free, won’t interfere with your treatment, maintains your privacy and is reversible if you should change your mind.

The tissue is most valuable if cooled or frozen immediately after surgery, so ask your surgeon about tumor donation and complete the appropriate forms prior to surgery In the absence of this planning, your tumor is likely to be fixed in paraffin, limiting the types of analyses that may be performed on it in the future.

There are two established alternatives for getting your tumor from the operating room to researchers working on ACC.

1) The following institutions are already, or soon will be, part of a collaborative Salivary Gland Tumor Biorepository. If you are having your surgery at one of these centers, they will automatically ensure that your tumor and relevant clinical information become “banked” in the proper form and available later for researchers. Simply confirm with your surgeon that the appropriate paperwork is in place prior to surgery.

MD Anderson
Johns Hopkins
University of Virginia (UVA)
University of Pittsburgh Medical Center
University of California San Francisco (UCSF)
Rhode Island Hospital

Your tumor donation will bring closer the day when new therapies will benefit ACC patients.

Histology
Histology is the study of cells using a microscope. Pathologists are the primary doctors who analyze a tumor sample from a biopsy. In completing 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. The diagnosis is 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).

Metastases
One of the characteristics of a malignant cancer cell is that it tends to infiltrate other normal tissue - metastasize. This occurs in one of three ways: It can spread locally by growing directly into adjoining normal tissue next to the tumor such as muscle, nerves or bone. Second, it can enter the lymph system and then begin the progression of growing from lymph node to lymph node. The final way cancer 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. This creates new tumors that are known as metastases, or “mets”. Metastatic spread of ACC is a more common cause of mortality than effects from the primary tumor.

Perineural Invasion
ACC has a unique tendency to microscopically infiltrate the 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 tissue that surrounds those strands of wires inside the pipe. This creates a real challenge for both diagnosis and treatment. Physicians of patients with primary tumor sites in the head and neck area may order regular imaging studies of the major nerves that lead back to the brain. Some medical professionals have reported that in reviewing imaging scans, ACC appears to “skip” areas and can infiltrate areas more removed from the primary tumor site.

Recurrence
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.

Staging (or grading)
Staging is a means to “label” the level of advancement of a particular cancer tumor. This categorization by a numerical scheme allows comparison with similar tumors. There is variation in the methods of staging reported in 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 - 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 metastases and M1 means there are metastases. 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 may well live for another 15 to 20 years.

An additional staging/grading system that is referenced in some medical papers is based upon tumor histology. 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.

Frequently Asked Questions

General

Treatment Approaches and Options - Initial Diagnosis

Treatment Approaches and Options - Recurrence and Metastases

Clinical Trials Information

Living With ACC

Supporting Research

Glossary of Selected Terms

What is Adenoid Cystic Carcinoma?

What causes ACC?

Are there different types of ACC?

What is historical life expectancy following initial diagnosis?

What institutions and/or physicians have experience treating ACC?

How do physicians decide upon treatment protocols?

What if my doctor tells me I cannot have surgery because of the location, and/or size of my tumor?

What are the types of radiation therapy that might be used in my treatment?

Are there chemotherapy and other drug treatments available?

What are the different types of imaging tests used in the diagnosis, treatment and follow-up cycle?

How will I know if my treatment has been effective?

What are the treatment options for recurrence and/or metastasis?

What if I am told I cannot have additional surgery for recurrence and/or metastasis?

Are there chemotherapy and other drug therapy options available for recurrence and/or metastasis?

What are clinical trials and should I consider taking part?

Where can I find out about specific clinical trials?

What advice do experienced ACC patients have for managing this disease?

Where can I get advice from experienced ACC patients?

Are there sources of financial assistance for cancer patients?

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

What kind of research is going on in rare cancers like ACC?

Why Support ACCRF?

How can I help?

Should I donate my tumor?

Glossary of selected terms