Rare Infectious Disease News

Disease Profile

Vulvar cancer

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



Rare Cancers


Vulvar cancer is the abnormal growth of cells of the vulva, which is part of the female reproductive system and includes the vaginal lips, clitoris, and part of the vagina. Symptoms of vulvar cancer may consist of a lump (mass), itching, or unusual bleeding. Though the exact cause of this cancer is unknown, older women and those who have human papillomavirus have a higher chance of developing vulvar cancer. Treatment may include laser therapy, surgery, chemotherapy, or radiation therapy.[1]


Most vulvar cancers occur by chance due to older age or human papillomavirus infection. However, an inherited condition called Fanconi anemia is associated with a significantly increased risk for vulvar cancer.[2] This condition is very rare and may cause only a small proportion of all vulvar cancers. There is also some evidence that a small number of vulvar cancers may be related to a genetic predisposition: one research study found that relatives of individuals with vulvar cancer have a slightly increased risk to develop vulvar cancer, which suggests there may be a genetic component to this disease that could be shared by family members, but the specific gene is not yet known.[3]


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

    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

    • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.

      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.


        1. National Cancer Institute. General Information about Vulvar Cancer. Vulvar Cancer Treatment (PDQ). 2012; https://www.cancer.gov/cancertopics/pdq/treatment/vulvar/Patient. Accessed 7/20/2012.
        2. Rosenberg PS, Alter BP, Ebell W. Cancer risks in Fanconi anemia: findings from the German Fanconi Anemia Registry. Haematologica. 2008; 93:511-517. https://www.ncbi.nlm.nih.gov/pubmed/18322251. Accessed 7/20/2012.
        3. Hussain SK, Sundquist J, Hemminki K. Familial clustering of cancer at human papillomavirus-associated sites according to the Swedish Family-Cancer Database. International Journal of Cancer. 2008; 122:1873-1878. https://www.ncbi.nlm.nih.gov/pubmed/18074353. Accessed 7/20/2012.

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