Primitive Neuroectodermal Tumor of the Pancreas
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概要
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The primitive neuroectodermal tumor (PNET) of the pancreas, a member of Ewings 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.
著者
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Iguchi Haruo
Department Of Gastroenterology National Kyushu Cancer Center
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KAWASAKI Hideki
Department of Agricultural Chemistry, the University of Tokyo
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Hasebe Aki
Department Of Gastroenterology Ehime Prefectural Central Hospital
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Doi Hirokazu
Department Of Gastroenterology Ehime Prefectural Central Hospital
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Horiike Norio
Department Of Gastroenterology And Metabology Ehime University Graduate School Of Medicine
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Maeda Toshiharu
Department Of Medical Laboratory Ehime Prefectural Central Hospital
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SOGA YOSHIKO
Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine
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Hiraoka Atsushi
Department Of Gastroenterology Ehime Prefectural Central Hospital
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Miyamoto Yasunao
Department Of Gastroenterology Ehime Prefectural Central Hospital
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Ninomiya Tomoyuki
Department Of Gastroenterology Ehime Prefectural Central Hospital
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Takamura Kazuhito
Department Of Surgery Ehime Prefectural Central Hospital
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Ichikawa Soichi
Department Of Gastroenterology Ehime Prefectural Central Hospital
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Kodama Akihiro
Department Of Internal Medicine Seiyo Municipal Nomura Hospital
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OCHI Hironori
Department of Gastroenterology, Ehime Prefectural Central Hospital
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Nakahara Hiromasa
Department Of Gastroenterology Ehime Prefectural Central Hospital
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Ichiryu Misa
Department Of Gastroenterology Ehime Prefectural Central Hospital
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Ochi Hironori
Department Of Gastroenterology Ehime Prefectural Central Hospital
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Tamura Hiromi
Department Of Pediatric Surgery Fujiwara Memorial Hospital
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Hasegawa Tadashi
Department Of Cardiovascular And Thoracic Surgery Hakodate Municipal Hospital
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TANABE Atsushi
Department of Bioproduction Science, Faculty of Horticulture
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Kameoka Chieko
Department of Medical Laboratory, Ehime Prefectural Central Hospital
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Kan Miki
Department of Medical Laboratory, Ehime Prefectural Central Hospital
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Doi Shigekazu
Department of Medical Laboratory, Ehime Prefectural Central Hospital
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Asaki Akinori
Department of Gastroenterology, National Hospital Organization Shikoku Cancer Center
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Seno Seijin
Department of Gastroenterology, National Hospital Organization Shikoku Cancer Center
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Iguchi Haruo
Department of Gastroenterology, National Hospital Organization Shikoku Cancer Center
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Soga Yoshiko
Department of Medical Laboratory, Ehime Prefectural Central Hospital
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Kodama Akihiro
Department of Gastroenterology, Ehime Prefectural Central Hospital
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