Paget's disease primarily involving the scrotum

Three cases of Paget's disease primarily involving the scrotum are reported. One case exhibited positive staining for prostate specific antigen (PSA) and was associated with an underlying, invasive poorly differentiated adenocarcinoma of unknown primary. The other cases were not associated with any underlying or visceral malignancy. The literature on Paget's disease primarily involving the scrotum including clinical presentation, differential diagnosis, treatment, prognosis, and possible histogenetic mechanisms of this disease is discussed.