title: Primitive neuroectodermal tumor of the pancreas.
abstract: The primitive neuroectodermal tumor (PNET) of the pancreas, a member of Ewing's sarcoma family of tumors, is extremely rare. We treated a 37-year-old Japanese man who had a solitary pancreatic tumor 40 mm in diameter and multiple hepatic tumors with surgical resection. The PNET was positive for CD99 on immunohistochemical staining. Fluorescence in situ hybridization (FISH) was also performed, which revealed a Ewing sarcoma breakpoint region 1 (EWSR1) 22q12 rearrangement. According to the Japan-Ewing protocol, chemotherapy with Ifomide (ifosfamide), etoposide, vincristine, and cyclophosphamide was given after surgery. To the best of our knowledge, to date 13 PNET cases have been reported with a mean age for all patients of 19.3 years old. Surgical resection was performed in most cases and some patients received postoperative chemotherapy. The clinicopathologic characteristics and management of this extremely rare disease are also discussed.
authors.affiliation: Department of Gastroenterology, Ehime Prefectural Central Hospital, Matsuyama.
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Doi
authors.forename: Soichi
authors.initials: S
authors.lastname: Ichikawa
authors.forename: Atsushi
authors.initials: A
authors.lastname: Hiraoka
authors.forename: Misa
authors.initials: M
authors.lastname: Ichiryu
authors.forename: Hiromasa
authors.initials: H
authors.lastname: Nakahara
authors.forename: Hironori
authors.initials: H
authors.lastname: Ochi
authors.forename: Atsushi
authors.initials: A
authors.lastname: Tanabe
authors.forename: Akihiro
authors.initials: A
authors.lastname: Kodama
authors.forename: Aki
authors.initials: A
authors.lastname: Hasebe
authors.forename: Yasunao
authors.initials: Y
authors.lastname: Miyamoto
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Ninomiya
authors.forename: Norio
authors.initials: N
authors.lastname: Horiike
authors.forename: Kazuhito
authors.initials: K
authors.lastname: Takamura
authors.forename: Hideki
authors.initials: H
authors.lastname: Kawasaki
authors.forename: Chieko
authors.initials: C
authors.lastname: Kameoka
authors.forename: Miki
authors.initials: M
authors.lastname: Kan
authors.forename: Shigekazu
authors.initials: S
authors.lastname: Doi
authors.forename: Yoshiko
authors.initials: Y
authors.lastname: Soga
authors.forename: Hiromi
authors.initials: H
authors.lastname: Tamura
authors.forename: Toshiharu
authors.initials: T
authors.lastname: Maeda
authors.forename: Akinori
authors.initials: A
authors.lastname: Asaki
authors.forename: Seijin
authors.initials: S
authors.lastname: Seno
authors.forename: Haruo
authors.initials: H
authors.lastname: Iguchi
authors.forename: Tadashi
authors.initials: T
authors.lastname: Hasegawa
mesh_terms: D000328:Adult; D002148:Calmodulin-Binding Proteins / Q000235:genetics; D004358:Drug Therapy; D006801:Humans; D008297:Male; D018242:Neuroectodermal Tumors, Primitive / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery; D049268:Positron-Emission Tomography; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins / Q000235:genetics
chemical_list: D002148:Calmodulin-Binding Proteins; C500986:EWSR1 protein, human; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins