Primitive Neuroectodermal Tumor of the Pancreas

Hirokazu Doi; Soichi Ichikawa; Atsushi Hiraoka; Misa Ichiryu; Hiromasa Nakahara; Hironori Ochi; Atsushi Tanabe; Akihiro Kodama; Aki Hasebe; Yasunao Miyamoto; Tomoyuki Ninomiya; Norio Horiike; Kazuhito Takamura; Hideki Kawasaki; Chieko Kameoka; Miki Kan; Shigekazu Doi; Yoshiko Soga; Hiromi Tamura; Toshiharu Maeda; Akinori Asaki; Seijin Seno; Haruo Iguchi; Tadashi Hasegawa

doi:10.2169/internalmedicine.48.1484

CASE REPORTS

Primitive Neuroectodermal Tumor of the Pancreas

Hirokazu Doi, Soichi Ichikawa, Atsushi Hiraoka, Misa Ichiryu, Hiromasa Nakahara, Hironori Ochi, Atsushi Tanabe, Akihiro Kodama, Aki Hasebe, Yasunao Miyamoto, Tomoyuki Ninomiya, Norio Horiike, Kazuhito Takamura, Hideki Kawasaki, Chieko Kameoka, Miki Kan, Shigekazu Doi, Yoshiko Soga, Hiromi Tamura, Toshiharu Maeda, Akinori Asaki, Seijin Seno, Haruo Iguchi, Tadashi Hasegawa

Author information

Hirokazu Doi
Department of Gastroenterology, Ehime Prefectural Central Hospital
Soichi Ichikawa
Department of Gastroenterology, Ehime Prefectural Central Hospital
Atsushi Hiraoka
Department of Gastroenterology, Ehime Prefectural Central Hospital
Misa Ichiryu
Department of Gastroenterology, Ehime Prefectural Central Hospital
Hiromasa Nakahara
Department of Gastroenterology, Ehime Prefectural Central Hospital
Hironori Ochi
Department of Gastroenterology, Ehime Prefectural Central Hospital
Atsushi Tanabe
Department of Gastroenterology, Ehime Prefectural Central Hospital
Akihiro Kodama
Department of Gastroenterology, Ehime Prefectural Central Hospital
Aki Hasebe
Department of Gastroenterology, Ehime Prefectural Central Hospital
Yasunao Miyamoto
Department of Gastroenterology, Ehime Prefectural Central Hospital
Tomoyuki Ninomiya
Department of Gastroenterology, Ehime Prefectural Central Hospital
Norio Horiike
Department of Gastroenterology, Ehime Prefectural Central Hospital
Kazuhito Takamura
Department of Surgery, Ehime Prefectural Central Hospital
Hideki Kawasaki
Department of Surgery, Ehime Prefectural Central Hospital
Chieko Kameoka
Department of Medical Laboratory, Ehime Prefectural Central Hospital
Miki Kan
Department of Medical Laboratory, Ehime Prefectural Central Hospital
Shigekazu Doi
Department of Medical Laboratory, Ehime Prefectural Central Hospital
Yoshiko Soga
Department of Medical Laboratory, Ehime Prefectural Central Hospital
Hiromi Tamura
Department of Medical Laboratory, Ehime Prefectural Central Hospital
Toshiharu Maeda
Department of Medical Laboratory, Ehime Prefectural Central Hospital
Akinori Asaki
Department of Gastroenterology, National Hospital Organization Shikoku Cancer Center
Seijin Seno
Department of Gastroenterology, National Hospital Organization Shikoku Cancer Center
Haruo Iguchi
Department of Gastroenterology, National Hospital Organization Shikoku Cancer Center
Tadashi Hasegawa
Department of Surgical Pathology, Sapporo Medical University School of Medicine

Keywords: primitive neuroectodermal tumor, pancreas, FDG-PET

JOURNAL OPEN ACCESS

2009 Volume 48 Issue 5 Pages 329-333

DOI https://doi.org/10.2169/internalmedicine.48.1484

Details

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.

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