Rare Endocrinology News

Disease Profile

Myoepithelial 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.


US Estimated

Europe Estimated

Age of onset





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.


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.


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)

Malignant myoepithelioma; Myoepithelioma carcinoma


Rare Cancers


Myoepithelial carcinoma is a rare malignant (cancerous) tumor that usually occurs in the salivary glands in the mouth, but can also occur in skin and soft tissues. Approximately 66% of these tumors occur in a part of the salivary gland, known as the parotid gland. The average age of diagnosis is 55 years. Symptoms of myoepithelial carcinoma usually begin with a painless mass.[1] Other symptoms vary depending on the site of the tumor and may include: hoarseness, nasal blockage, bleeding from the nose, pain, headaches, and facial weakness and paralysis.[2] Treatment depends on the location and stage of the tumor. Treatment options may include surgery to remove the affected tissues, radiation therapy, and chemotherapy.[1]


The treatment for metastatic myoepithelial carcinoma usually begins with surgery to remove the main tumorRadiation therapy can be used to reduce the chance that the tumor could return in the same location. There is limited evidence about the usefulness of chemotherapy in treating myoepithelial carcinoma, and there are no standard treatment guidelines.[1]


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 Providing General Support

    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 American Cancer Society has developed an information page on salivary gland cancer. Click on the link to view the information page.
    • The National Cancer Institute provides the most current information on cancer for patients, health professionals, and the general public.

      In-Depth Information

      • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
      • 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 Myoepithelial carcinoma. Click on the link to view a sample search on this topic.


        1. Salivary Gland Cancer Treatment: Myoepithelial carcinoma. National Cancer Institute Web site. September 15, 2015; https://www.cancer.gov/types/head-and-neck/hp/salivary-gland-treatment-pdq#link/_311. Accessed 11/18/2016.
        2. M. Sherif Said. Myoepithelial Carcinoma. Medscape. September 27, 2015; https://emedicine.medscape.com/article/1720030. Accessed 11/18/2016.
        3. Yu G; Ma D; Sun K; Li T; Zhang Y. Myoepithelial carcinoma of the salivary glands: behavior and management.. Chin Med J. 2003; 116(2):163-165. https://www.ncbi.nlm.nih.gov/pubmed/?term=12775221.
        4. Hornick JL, Fletcher CD. Myoepithelial tumors of soft tissue: a clinicopathologic and immunohistochemical study of 101 cases with evaluation of prognostic parameters. Am J Surg Pathol. 2003 Sep;27(9):1183-96..

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