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Disease Profile
Adenoid cystic carcinoma
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
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Age of onset
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ICD-10
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Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.
X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Not applicable
Other names (AKA)
Adenocystic carcinoma; Cribriform carcinoma; Cylindroma
Categories
Rare Cancers
Summary
Adenoid cystic carcinoma (ACC) is a rare form of adenocarcinoma, a type of
Cause
There has been evidence that ACC
Organizations
Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.
Organizations Supporting this Disease
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Adenoid Cystic Carcinoma Research Foundation
P.O. Box 442
Needham, MA 02494
Telephone: (781) 248-9699
E-mail: https://www.accrf.org/html/contactus.php
Website: https://www.accrf.org/ -
The Oral Cancer Foundation
3419 Via Lido #205
Newport Beach, CA 92663
Telephone: 949-646-8000
E-mail: [email protected]
Website: https://oralcancerfoundation.org
Learn more
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
Where to Start
- The Oral Cancer Foundation provides information about adenoid cystic carcinoma. Visit the above link to access this information.
- Cancer.Net, a resource from the American Society of Clinical Oncology, provides information about adenoid cystic carcinoma. Click on the above link to access this information.
- The National Cancer Institute provides the most current information on cancer for patients, health professionals, and the general public.
- The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
In-Depth Information
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Adenoid cystic carcinoma. Click on the link to view a sample search on this topic.
References
- Adenoid Cystic Carcinoma. Cancer.Net (American Society of Clinical Oncology). January, 2016; https://www.cancer.net/cancer-types/adenoid-cystic-carcinoma/overview.
- Adenoid Cystic Carcinoma. Oral Cancer Foundation. https://www.oralcancerfoundation.org/facts/rare/ac/. Accessed 6/20/2017.
- Mikse OR, Tchaicha JH, Akbay EA, Chen L, Bronson RT, Hammerman PS, Wong KK. The impact of the MYB-NFIB fusion proto-oncogene in vivo. Oncotarget. May 31, 2016; 7(22):31681-31688. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5077968/.
- Understanding ACC. Adenoid Cystic Carcinoma Research Foundation. https://www.accrf.org/living-with-acc/understanding-acc/. Accessed 6/21/2017.
- Yoshioka N, Mese H, Okui T, Ibaragi S, Sasaki A. Familial adenoid cystic carcinoma of sublingual salivary glands. Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology. May, 2015; 27(3):353-356. https://www.sciencedirect.com/science/article/pii/S2212555814000477.
- Channir HI, van Overeem Hansen T, Andreasen S, Yde CW, Kiss K, Charabi BW. Genetic Characterization of Adenoid Cystic Carcinoma of the Minor Salivary Glands: A Potential Familial Occurrence in First-Degree Relatives. Head Neck Pathol. February 16, 2017; [Epub ahead of print]:https://www.ncbi.nlm.nih.gov/pubmed/28210977.
- Shen TK, Teknos TN, Toland AE, Senter L, Nagy R. Salivary Gland Cancer in BRCA-Positive Families : A Retrospective Review. JAMA Otolaryngol Head Neck Surg. September 25, 2014; [Epub ahead of print]:Accessed 12/10/2014.
- Yilmaz B, Goldberg LH, Schechter NR, Kemp BL, Ruiz H. Basal cell nevus syndrome concurrent with adenoid cystic carcinoma of salivary gland. J Am Acad Dermatol. May, 2003; 48(5 Suppl):S64-66.
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