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 investigated whether this disease causes an increased risk of undiagnosed visceral malignancy. MATERIALS AND METHODS: We reviewed the databases at our institution from 1996 to 2000 and identified 6 men 67 to 87 years old (mean age 76). In addition, 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 penile extramammary Paget's disease was present in 33%. Each man underwent wide local excision and large skin defects were immediately reconstructed with split-thickness skin grafts. In 1 case extramammary Paget's disease had spread to the superficial inguinal nodes. At a mean followup of 29 months there has been no local recurrence and internal malignancy has not been diagnosed. Our literature review revealed 13 patients with penoscrotal extramammary Paget's disease and visceral malignancy, including 12 (92%) with malignancy of the genitourinary system. CONCLUSIONS: Extramammary Paget's disease of the penis and scrotum is a rare disease that can be managed by excision and immediate reconstruction with skin grafting or a local skin flap. Disease may spread to the regional lymph nodes. Although genitourinary cancer may accompany penoscrotal extramammary Paget's disease, an extensive search for cancer of the thorax or abdomen may be unnecessary because only 1 reported case of colon cancer has been associated with penile or scrotal extramammary Paget's disease.