Extramammary Paget's disease of the penis and scrotum: Excision, reconstruction and evaluation of occult malignancy

Purpose: We describe treatment and reconstruction in patients after surgery for extramammary Paget's disease of the penis and scrotum. We also investi gated whether this disease causes an increased risk of undiagnosed visceral malignancy. Materials and Methods: We reviewed the databases at our institution from 19 96 to 2000 and identified 6 men 67 to 87 years old (mean age 76). In additi on, we reviewed the literature on the clinical and pathological features of this disease. Results: In our 6 patients scrotal involvement was present in 83% and penil e extramammary Paget's disease was present in 33%. Each man underwent wide local excision and large skin defects were immediately reconstructed with s plit-thickness skin grafts. In 1 case extramammary Paget's disease had spre ad to the superficial inguinal nodes. At a mean followup of 29 months there has been no local recurrence and internal malignancy has not been diagnose d. Our literature review revealed 13 patients with penoscrotal extramammary Paget's disease and visceral malignancy, including 12 (92%) with malignanc y of the genitourinary system. Conclusions: Extramammary Paget's disease of the penis and scrotum is a rar e disease that can be managed by excision and immediate reconstruction with skin grafting or a local skin flap. Disease may spread to the regional lym ph nodes. Although genitourinary cancer may accompany penoscrotal extramamm ary Paget's disease, an extensive search for cancer of the thorax or abdome n may be unnecessary because only 1 reported case of colon cancer has been associated with penile or scrotal extramammary Paget's disease.