Response to 5-fluorouracil in

disease of the scrotum presenting as pancytopenia and back pain Extramammary Paget disease is a rare intraepithelial neoplasm of the vulvar, penoscrotal, or perianal skin. No effective therapies for metastatic disease have been reported, and prognosis for metastatic disease is poor. Here, we report the case of an asian man who was initially diagnosed with extramammary Paget disease of the scrotum. Three years later, the patient presented to hospital with pancytopenia and back pain. after an extensive work-up, biopsies of liver and bone marrow revealed adenocarcinoma with signet cells and immunohistochemical staining positive for keratin 7, carcinoembryonic antigen, and prolactin-induced protein, but negative for keratin 20, S100, and prostate markers, consistent with his previous biopsy-proven Paget disease of the scrotum. The patient was treated with 5-fluorouracil–based therapy in addition to palliative radiotherapy to selected spine levels. a promising palliative response was demonstrated fol-lowing 5-fluorouracil chemotherapy. a review of the literature on the pathogenesis, diagnosis, treatment options, and outcomes for meta-static extramammary Paget disease is presented