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Genetic and Rare Diseases Information Center (GARD)

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Paget disease, extramammary

Other Names for this Disease
  • EMPD
  • Extramammary Paget disease
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Your Question

I was diagnosed with extramammary Paget disease in 2008 in the groin, axilla, and scrotum. I am still unable to find much information about this type of cancer, how I may have contracted it, or what my prognosis may be concerning any possible reoccurrence.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is extramammary Paget disease?

Extramammary Paget disease is characterized by a chronic eczema-like rash of the skin around the genital regions of males and females. Under the microscope, this condition looks very similar to a condition that occurs on the breast called mammary Paget disease. Extramammary Paget disease most often occurs in women between age 50 to 60. About 25 percent of extramammary Paget disease is associated with an underlying cancer.[1]
Last updated: 5/21/2013

What are the symptoms of extramammary Paget disease?

The most common symptom of extramammary Paget disease is mild to intense itching of a lesion found around the genital or anal area. Pain and bleeding may result from scratching the lesions. The lesions can become thick, red, scaly, and crusty. In women, the most common affected area is the vulva. The location of extramammary Paget disease is useful in predicting the risk of associated cancer. Approximately 25-35% of extramammary Paget disease around the anal region is associated with an underlying colorectal cancer.[1]
Last updated: 5/21/2013

How is extramammary Paget disease diagnosed?

Extramammary Paget disease is usually diagnosed by skin biopsy of the lesion. Under the microscope, the presence of Paget cells along with other specific findings confirms the diagnosis. Special stains may be necessary to distinguish extramammary Paget disease from early melanoma (melanoma in situ).[1]
Last updated: 5/21/2013

What causes extramammary Paget disease?

The cause of extramammary Paget disease remains unknown. Approximately 25% of cases are associated with an underlying tumor. The type of tumor that is most likely to be associated with this disease is an apocrine carcinoma. Other associated cancers include cutaneous adnexal carcinoma and carcinoma of the Bartholin glands, urethra, bladder, vagina, cervix, endometrium, or prostate. About 4-7% of patients with genital disease have an associated carcinoma. Perianal disease is associated with underlying colorectal cancer in 25-35% of cases.[2]
Last updated: 5/21/2013

How is extramammary Paget disease treated?

Extramammary Paget disease is usually treated by surgically removing the skin lesion. It is sometimes difficult to determine how much skin should be removed, particularly when lesions are spread throughout the anogenital region. Because recurrence is common, it is standard for patients to be re-examined every 3 months after surgery for the next 2 years. After 2 years, annual follow-ups are usually recommended. A promising treatment involves a cream that modifies the immune response, called imiquimod.[1][2]
Last updated: 5/21/2013

What is the prognosis for extramammary Paget disease?

The prognosis for extramammary Paget disease depends on early diagnosis and surgical treatment. The prognosis is good for cases where the disease only affects the epidermal (top) layer of skin and the areas around the surgically removed lesion (margins) remain free of the disease. One study showed a mortality rate of 18% for patients without associated carcinoma and 46% for those with underlying carcinoma. Perianal disease, invasion into the dermal skin layer, and spread of cancer to lymph nodes are more likely to be associated with a poor prognosis. The rate of recurrence rate is approximately 30%. In those people who experience a recurrence of extramammary Paget disease, the average time it takes for the disease to recur is 2.5 years.[2]
Last updated: 5/21/2013