Extramammary Paget's disease of the scrotum: an analysis of 26 cases

Abstract

To investigate the clinical features, diagnosis and therapeutics of extramammary Paget's disease. A total of 26 cases of scrotal Paget's disease were included. According to Ray staging, 16, 7 and 3 cases had A1, A2 and B stages of Peget's diseases respectively. All cases underwent surgical resection of their diseased sites after a biopsy. The excision margin was 2 cm apart from the edge of lesion. And resection depth reached deep fascia. During operation, frozen sections of excised tissue specimens in 18 cases were pathologically examined. And the excision margins were involved by tumor cell in 3 cases (16.67%). Thus extended excision was performed immediately. Postoperatively, excision margin involvement was confirmed in 1 case and phase II operation performed. Three cases with inguinal lymph node metastasis underwent unilateral or bilateral inguinal lymph node dissection. Paget's disease was confirmed in all 26 cases. Tumor was localized in epidemics in 15 cases and skin invasion found in 11 cases. The lymph node was tumescent in 9 cases. Among them, 3 had pathologically confirmed metastasis and 6 was diagnosed with lymphadenitis. All cases received a mean follow-up period of 5 years (range: 1 - 7 years). Five locally recurrent cases healed after a second operation. Chronic skin lesions of the scrotum should be biopsied as early as possible if extramammary Paget's disease is suspected. Radical resection of diseased skin is its preferred therapy. A second operation may be performed for recurrence. The prognosis of extramammary Paget's disease is excellent after radical surgery.