Fourth Annual Chicago Supportive Oncology Conference

Extramammary Paget’s disease

Venkatesh R. Rao, MD, and David H. Henry, MD

Joan Karnell Cancer Center, Pennsylvania Hospital, Philadelphia, PA

Extramammary Paget’s disease is an uncommon cutaneous adenocarcinoma, characterized by glandular differentiation and an insidious course. The condition may or may not be associated with an underlying adnexal or visceral malignancy. Because of its rarity, the pathogenesis is unclear, and standardization of treatment is lacking. The diagnosis is made histopathologically, and management is based on wide surgical excision. Long-term follow-up is essential to exclude a recurrence of the disease and monitor the patient for an underlying or associated cancer. Here, we report a case of this rare disorder involving the perianal region and briefly review the available literature. This case report draws attention to extramammary Paget’s disease, which should be considered in the differential diagnosis of perianal disorders.

Commun Oncol 2004;1:109–115   print e-mail full text 157 kb