title: Desmoplastic small round cell tumors in a young man.
abstract: A previously healthy 18-year-old man was admitted to our hospital with abdominal pain in September 2010. We performed a percutaneous biopsy of multiple intrahepatic masses. A diagnosis of desmoplastic small round cell tumors was confirmed based on the presence of a polyphenotypic immunoprofile (positivity for EMA, vimentin, cytokeratin, desmin and WT1) and characteristic EWS-WT1 gene fusion. Because the mass had invaded the mesentery and the disease had disseminated to liver, the patient received palliative chemotherapy with carboplatin, paclitaxel, vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide and irinotecan. The maximal response to the chemotherapy was a partial remission. The patient died 20 months after diagnosis.
authors.affiliation: Department of Gastroenterology and Medicine, Fukuoka University Faculty of Medicine, Japan. g.hirano@minf.med.fukuoka-u.ac.jp
authors.forename: Genryu
authors.initials: G
authors.lastname: Hirano
authors.forename: Makoto
authors.initials: M
authors.lastname: Irie
authors.forename: Yuta
authors.initials: Y
authors.lastname: Nakashima
authors.forename: Satoshi
authors.initials: S
authors.lastname: Shakado
authors.forename: Tetsuro
authors.initials: T
authors.lastname: Sohda
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Tanaka
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Takamatsu
authors.forename: Mikiko
authors.initials: M
authors.lastname: Aoki
authors.forename: Kazuo
authors.initials: K
authors.lastname: Tamura
authors.forename: Kazuki
authors.initials: K
authors.lastname: Nabeshima
authors.forename: Shotaro
authors.initials: S
authors.lastname: Sakisaka
mesh_terms: D000008:Abdominal Neoplasms / Q000175:diagnosis* / Q000188:drug therapy*; D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D058405:Desmoplastic Small Round Cell Tumor / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans; D008297:Male