title: A peripheral primitive neuroectodermal tumor originating from the pancreas: a case report and review of the literature.
abstract: A peripheral primitive neuroectodermal tumor (pPNET) is a small round cell tumor occurring mostly in children or young adults and categorized into the Ewing sarcoma family of tumors. pPNETs originating from the pancreas are especially rare, and only 25 cases have been reported in the literature. We report a case of a 22-year-old man who had a giant expansive tumor located in the uncinate process of the pancreas, 80 mm in diameter resulting in obstruction in the duodenum. The patient underwent a pancreaticoduodenectomy. The histological examination showed that the pancreatic tumor was composed of atypical small round cells. Immunohistochemical findings were positive for CD99. An Ewing sarcoma breakpoint region 1 gene 22q12 rearrangement was proven by a two-color fluorescence in situ hybridization assay. We diagnosed the tumor as a pPNET of the pancreas, which, according to the literature, is highly aggressive with poor prognosis. A multidisciplinary approach to treat these neoplasms should improve the prognoses.
authors.affiliation: Department of Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374 Japan.
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Nishizawa
authors.affiliation: Department of Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374 Japan.
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Kumamoto
authors.affiliation: Department of Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374 Japan.
authors.forename: Kazuharu
authors.initials: K
authors.lastname: Igarashi
authors.affiliation: Department of Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374 Japan.
authors.forename: Ryo
authors.initials: R
authors.lastname: Nishiyama
authors.affiliation: Department of Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374 Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Tajima
authors.affiliation: Department of Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374 Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kawamata
authors.affiliation: Department of Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374 Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Kaizu
authors.affiliation: Department of Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374 Japan.
authors.forename: Masahiko
authors.initials: M
authors.lastname: Watanabe
keywords: Ewing’s sarcoma; FISH; Pancreas; Peripheral primitive neuroectodermal tumor; Small round cell tumor; Young adult