Extramammary Paget disease is a cancer of the skin around the genital area. Cases of this cancer are very rare, which makes information about survival rates difficult to obtain, as the sample size is very small. Established treatment regimens can also be challenging to develop with such a limited study population. The name references the fact that the first described cases of Paget disease involved the breasts, though researchers later found that the disease could develop elsewhere in the body.
Patients with this condition develop irritation and redness that may initially resemble eczema, the result of Paget's cells growing inside their skin. It may be an independent neoplasm, or it could be related to an underlying cancer in the area, like adenocarcinoma. Unlike Paget's disease of the breast, which is almost always caused by an existing breast cancer, this condition commonly develops on its own.
As the rogue cells develop, the patient can experience more noticeable lesions that may crack and split. First line treatment for extramammary Paget disease may involve surgical resection of the growths, if possible, to prevent their further spread. This can be tricky on structures like the penis, which are difficult to operate on without causing cosmetic or functional damage. The doctor may also consider chemotherapy and radiation to control the cancer.
The superficial resemblance to eczema can delay a diagnosis of extramammary Paget disease. Care providers may not think of this condition when they notice redness and irritation around the anogenital area, and could initially recommend treatment with moisturizers and mild topical steroids to see if that resolves the problem. As the growth continues and develops, a biopsy or scraping may reveal the presence of Paget's cells and can lead to a definitive diagnosis for the patient.
Oncologists typically supervise treatment for extramammary Paget disease. They may consult with dermatologists and other care providers to provide complete care to the patient. Some patients may find it helpful to seek a referral to a specialist in order to access someone who has treated the condition before, and has some familiarity with it. This can increase the chances of a positive outcome and may mean the patient is able to have more aggressive and suitable treatment as early as possible.
This condition is prone to recurrences. People with a history of extramammary Paget disease may need regular followups to check for signs of new growth. If the cancer does return, treatment options may be more limited, depending on the location of recurrent cancer.
