Vulvar and breast Paget's disease with synchronous underlying cancer: a unique association

We report a unique case of Paget's disease of vulva and breast. Sequentially the patient had invasive ductal carcinoma of the breast, 5 years later was diagnosed with vulvar Paget's with underlying adenocarcinoma and after another 2 years was diagnosed with Paget's disease of the breast.

A 58-year-old woman with invasive ductal cancer of the left breast was treated with lumpectomy, lymph node dissection, radiation therapy and tamoxifen. Five years later and after complaints of longstanding vulvar pruritus, the patient was diagnosed with vulvar Paget's disease and treated with simple vulvectomy, which revealed a concurrent underlying adenocarcinoma. Subsequently there was recurrence of vulvar malignancy and wide local excision was performed. Seven years after the initial diagnosis of the breast cancer, a biopsy of a left areolar red, ulcerated lesion revealed Paget's disease of the breast.

Physicians need to be cognizant of the rare occurrence of mammary and extramammary Paget's disease with underlying malignancies in both locations. A thorough physical examination including biopsy is essential for early detection and appropriate management.