Scrotal extramammary Paget's disease associated with renal cell carcinoma

A 62-year-old male presented with a longstanding right scrotal skin lesion. After a skin biopsy, the lesion was proven to be extramammary Paget's disease. The right scrotal skin was excised and a split thickness skin graft performed. After 16-months of follow up, the patient visited again, this time complaining of abdominal pain. On abdominal computerized tomography (CT) and whole body bone scans, the presence of a solid renal mass at the upper pole of the left kidney, with multiple metastatic lesions in the liver, bone and lymph nodes, were found. Under the impression of a malignant primary renal tumor, a radical nephrectomy was performed. A histological examination showed a conventional type renal cell carcinoma, with malignant Paget cells infiltrating and residing within the tumor mass. The patient died on the 50th postoperative day due to acute respiratory distress syndrome (ARDS) caused by aspiration pneumoniae.