Invasive extramammary Paget's disease of the bladder diagnosed 18 years after noninvasive extramammary Paget's disease of the vulva

Abstract

Paget's disease of the vulva is a rare vulvar neoplasm most commonly seen in postmenopausal women. Clinically, it presents as a pink eczematoid lesion with white islands of hyperkeratosis accompanied by pruritus. Pathologically it resembles mammary Paget's disease of the nipple and areola, first described by Sir James Paget in 1874 (Paget). The mean age at diagnosis of Paget's disease of the vulva has been reported to be between 50 and 80 years, and it is most common in Caucasian women (Preti et al., 2000). Disease is often limited to the epidermis and mucosa, without invasion. The optimal management of Paget's disease of the vulva remains unclear. Surgical excision is usually the primary therapy; however, 30% to 60% of patients develop recurrent disease. Furthermore, the lesions often extend past clinically apparent borders and surgical excision is limited by the anatomy of the vulva. In addition, the disease is often multifocal. Immunohistochemistry (IHC) can help distinguish Paget's disease from other vulvar conditions. Cytokeratin 7 (CK7), cytokeratin 20 (CK20), carcinoembryonic antigen (CEA), and gross cystic disease fluid protein-15 (GCDFP-15) are common markers for Paget's disease. Approximately 10% of patients with Paget's disease of the vulva have an underlying second malignancy. These include colorectal cancer, cervical cancer, breast cancer, as well as carcinoma of the transitional epithelium from the renal pelvis to urethra. Routine screening with colonoscopy, Pap test, and mammogram is therefore recommended. In addition, in rare instances, Paget's disease may become invasive, infiltrating the dermis and even metastasizing to lymph nodes and distant sites (Reddy et al., 2012). In this report, we describe the case of a woman with noninvasive Paget's disease of the vulva who developed invasive Paget's disease of the bladder 18 years after her original diagnosis of Paget's disease of the vulva.