title: Metastatic extramammary Paget's disease responding to weekly paclitaxel.
abstract: Metastatic extramammary Paget's disease (EMPD) is a rare cancer with no validated systemic treatment. Regimens including FECOM 5-fluorouracil (5-FU, epirubicin, carboplatin, vincristine and mitomycin C), 5-FU/cisplatin and single agent docetaxel exhibited varying levels of efficacy in case reports. A 58-year-old man with EMPD diffusely metastatic to bone presented with worsening shortness of breath, significant pancytopenia and disseminated intravascular coagulation (DIC). He was started on low-dose heparin for the DIC and weekly paclitaxel. Initially requiring almost daily transfusions, his shortness of breath improved after two doses of paclitaxel, and he became transfusion-independent after only three doses. Correlating with his disease course, the patient's prepaclitaxel carcinoembryonic antigen level of 62.1 ng/mL decreased to 7.4 ng/mL on 3-month follow-up, and he showed no progression of disease on imaging. With no validated chemotherapy regimen currently, this case can help guide consideration of paclitaxel in future treatment of metastatic EMPD.
authors.affiliation: Department of Oncology, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Vania
authors.initials: V
authors.lastname: Phuoc
authors.affiliation: Department of Oncology, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Axel
authors.initials: A
authors.lastname: Grothey
mesh_terms: D000972:Antineoplastic Agents, Phytogenic / Q000627:therapeutic use*; D001859:Bone Neoplasms / Q000188:drug therapy* / Q000556:secondary; D006801:Humans; D008297:Male; D008875:Middle Aged; D017239:Paclitaxel / Q000627:therapeutic use*; D010145:Paget Disease, Extramammary / Q000188:drug therapy* / Q000556:secondary; D010502:Perineum; D012878:Skin Neoplasms / Q000473:pathology* / Q000601:surgery
chemical_list: D000972:Antineoplastic Agents, Phytogenic; D017239:Paclitaxel