Metastatic extramammary Paget's disease responding to Cisplatin, 5-Fluorouracil and radiotherapy - a case report

Extramammary Pagetââ"šÂ¬Ã¢"žÂ¢s disease is a rare intraepithelial neoplasm affecting individuals aged between 50 and 80 years. We report a case of a 63-year-old Filipino man with metastatic extramammary Pagetââ"šÂ¬Ã¢"žÂ¢s disease involving intraabdominal lymph nodes, initially treated with sequential multimodality treatment of combination chemotherapy with cisplatin and 5-fluorouracil based on the squamous cell carcinoma protocol followed by radiotherapy. He achieved a partial response with progression free survival of 3 months. He was subsequently treated with concurrent chemoradiation with 5-fluorouracil followed by carboplatin, then capecitabine. Despite the sequential multimodality treatment, his overall survival was 18 months, highlighting the poor prognosis of this disease. This case also raises the potential usefulness of positron emission tomography in extramammary Pagetââ"šÂ¬Ã¢"žÂ¢s disease for staging of nodal disease, assessment of response to therapy and disease progression as determined by the glucose avidity.