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Abstract
Extramammary Paget's disease is a rare cutaneous adenocarcinoma, usually of epidermal
origin and glandular differentiation and frequently associated with an underlying
adnexal carcinoma and perhaps with underlying internal malignancy. One hundred ninety-seven
cases of extramammary Paget's disease (196 cases reported in the English literature
from 1962 to 1982 and one case of my own) are reviewed. It remains a rare cutaneous
malignancy that occurs primarily in elderly people. It is seen more frequently in
women than in men and occurs predominantly in vulvar and perianal locations. Twenty-six
percent of patients with this disease will ultimately die of it or an associated internal
malignancy. Twenty-four percent of patients with the disease have an associated underlying
cutaneous adnexal adenocarcinoma. These patients have a higher mortality rate--46%--than
patients with extramammary Paget's disease without underlying cutaneous adnexal adenocarcinoma.
Twelve percent of patients with extramammary Paget's disease have an associated concurrent
underlying internal malignancy. The location of the underlying internal malignancy
appears to be closely related to the location of the extramammary Paget's disease--i.e.,
a perianal location is associated with adenocarcinoma of the digestive system, a penile-scrotal-groin
location with genitourinary malignancy, etc. A directed internal malignancy search
may be of benefit in patients who are diagnosed as having extramammary Paget's disease.