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Frequently Asked Questions
General
What is Adenoid Cystic Carcinoma? Treatment
Which hospitals have experience in treating ACC patients?
What is Adenoid Cystic Carcinoma?Adenoid cystic carcinoma (ACC) is an uncommon malignancy typically originating in the salivary glands, though it does appear in other primary sites, including the palate, nasopharynx, tongue base, larynx, trachea, breast and uterus. This cancer has a slow, but relentless, progression with frequent recurrences and metastases to the lungs, liver and bones. ACC tumors infiltrate and spread along nerves (perineural invasion). ACC has no known causes or links to smoking and alcohol consumption. It is slightly more prevalent in women than men. Thousands of Americans now have ACC and thousands more will be diagnosed with it before the cause and a cure can be found. Standard treatment for ACC involves surgery followed by radiation. While there are some patients participating in clinical trials, currently there are no proven effective drug therapies. 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. Importantly, due to ACC´s protracted course, many current patients may benefit from research efforts launched today. For more information on ACC, you may visit these web sites: UVA: Adenoid Cystic Carcinoma Facts and Tumor Registry People Living With Cancer: Guide to Adenoid Cystic Carcinoma Oral Cancer Foundation: Adenoid Cystic Carcinoma Adenoid Cystic Carcinoma Organization International (ACCOI) How is cancer research typically funded?The level of funding for cancer research, as with many illnesses, depends upon how many people are afflicted. Typically, common diseases receive generous funding from large non-profit organizations (such as the American Cancer Society), public institutions (such as the National Cancer Institute) and private pharmaceutical and biotechnology firms. Rare diseases, on the other hand, must depend upon private philanthropy to seed research that becomes sufficiently promising to warrant further support from larger institutions. A rare disease (also called an orphan disease) is a disease or condition affecting fewer than 200,000 persons in the United States, according to the National Institutes of Health. An estimated 25 million people in the United States have a rare disease (nearly 10% of the total population). Currently, the National Cancer Institute and the American Cancer Society do not fund any ACC-specific research. Why Support ACCRF?1. Without private funding, there will be no ACC research programs. ACCRF is shepherding research projects along until such time as they will be eligible for more significant public and private funds. 2. ACCRF is pioneering a new research model that will be more effective in conducting research into rare diseases with limited funding.
3. 100% of donations will go directly to fund ACC research programs. ACCRF´s founders have committed to covering the funding of all administrative, legal and accounting expenses. How may I help?ACCRF seeks three important contributions from the general public:
Click here to donate to ACCRF. Which hospitals have experience in treating ACC patients?Given the rarity of ACC, many practicing oncologists may see few, if any, ACC patients in the course of their careers. Accordingly, many ACC patients converge upon the major academic medical centers that are more likely to be aware of new treatments or spearheading clinical trials. These institutions also are instrumental in determining the normal treatment guidelines ("standard of care") for ACC patients. 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: http://cancercenters.cancer.gov/cancer_centers/map-cancer-centers.html The National Comprehensive Cancer Network (NCCN) is a not-for-profit alliance of 21 of the world´s 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: http://www.nccn.org/members/network.asp The National Cancer Institue also provides a website with information on how to find an appropriate doctor, a second opinion or a treatment facility: http://www.cancer.gov/cancertopics/factsheet/Therapy/doctor-facility While there are many other hospitals that might provide excellent care, these lists are a good starting point for patients in the United States. For patients outside the United States, the best option is to contact the International Cancer Information Service Group (ICISG): How do surgical and medical oncologists decide upon treatments?For ACC, the standard treatment for new cases tends to involve surgery (if possible), followed by radiation. Since no drugs have been found to be effective in preventing the recurrence of ACC, none are typically prescribed thereafter. If complete removal of the tumor is not an option for the primary site, or if the cancer has metastasized, then medical oncologists may suggest chemotherapy or targeted drugs for the patient, although none have been approved by the Food and Drug Administration (FDA) for ACC. Additional surgeries or radiation treatments may also be recommended. The National Comprehensive Cancer Network (NCCN) publishes its Clinical Practice Guidelines in Oncology that generally defines a "standard of care" for different types of cancer. The preponderance of ACC cases occur in the salivary glands and so the appropriate guidelines are within the Head & Neck Cancer version: http://www.nccn.org/professionals/physician_gls/PDF/head-and-neck.pdf Pages 12 through 16 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. The National Cancer Institute provides extensive information on chemotherapy and radiation therapy for patients considering those treatments: Chemotherapy and You: Support for People with Cancer Radiation Therapy and You: Support for People with Cancer Where may I find out about clinical trials?ACC patients with recurrent or metastatic disease may wish to consider participating in clinical trials. The overall process is described well in this link: If you´ve decided to learn about existing trials that may be appropriate for ACC patients, these links may be useful: http://www.clinicaltrials.gov/ct/search?term=adenoid+cystic+carcinoma http://www.centerwatch.com/patient/studies/cat161.html http://www.trialcheck.org/cancertrialshelp/ Where may I find out about drugs?There are many online resources that describe clinically-relevant drugs, some of which include: http://www.cancer.gov/cancertopics/druginfo/alphalist http://www.nlm.nih.gov/medlineplus/druginformation.html http://www.webmd.com/drugs/index-drugs.aspx http://en.wikipedia.org/wiki/Chemotherapy http://www.fda.gov/cder/cancer/druglistframe.htm http://www.cancerbackup.org.uk/Treatments/Chemotherapy/Individualdrugs Should I have my tumor tested for chemo-sensitivity?Some ACC patients that are considering taking drugs for a recurrence or metastasis have chemo-sensitivity testing performed on their tumor. Researchers at commercial laboratories generate reports that purport to tell a patient whether a particular tumor might be sensitive to a particular therapy. The goal is to find the optimal mix of drugs to treat the particular genomic profile of each patient. Examples of such firms may be found at these links: www.molecularprofiling.com/index.cfm ACCRF cannot vouch for these laboratories or the efficacy of their services, which can be quite expensive in some cases. And it is not clear that the science is proven in this field. Any patient considering chemo-sensitivity testing ought to do so with the guidance of an experienced and trusted oncologist. Where may I get advice from experienced ACC patients?The Yahoo email user's group moderated by ACCOI (www.accoi.org) is an excellent resource for ACC patients who would like more information on their individual situation. A 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 information should not substitute for the advice of an experienced physician, but may provide additional perspectives. |
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