Penoscrotal extramammary Paget's disease: The University of Texas M. D. Anderson Cancer Center contemporary experience

Purpose: Extramammary Paget's disease is a rare intra-epithelial malignancy that is occasionally associated with an invasive adenocarcinoma component as well as other secondary cancers. We investigated a consecutive series of patients referred for extramammary Paget's disease of the penis or scrotum at a single center in the contemporary era to determine the presence of secondary cancers and treatment outcomes. Materials and Methods: Between December 1990 and February 2009, 20 patients with extramammary Paget's disease of the scrotum and/or penis were seen. Patients were investigated with computerized tomography of the abdomen/pelvis, chest x-ray, cystoscopy, colonoscopy and serum prostate specific antigen measurement to rule out obvious secondary cancer. Clinical charts were reviewed with institutional review board approval. Mean followup of treated cases was 48 months (range 7 to 208). Results: Patients were 50 to 86 years old with a history of symptoms of between 6 months and 10 years. Eight patients had invasive adenocarcinoma, of whom 2 died of the disease and 3 who had disease at 5, 7 and 40 months, respectively, were undergoing multimodal therapy. No patient with confirmed intra-epidermal disease only died of the disease. Invasive disease predicted nodal and metastatic progression. Conclusions: Surgical resection to achieve tumor-free margins resulted in durable relapse-free survival of patients with intra-epidermal extramammary Paget's disease alone. Extramammary Paget's disease with invasion was associated with regional metastatic progression. The latter scenario as well as failure to treat localized extramammary Paget's disease alone was associated with a fatal outcome. Systemic chemotherapy should be further explored in patients with invasive adenocarcinoma or lymph node positive disease.