PMID- 19252356 OWN - NLM STAT- MEDLINE DCOM- 20090603 LR - 20190606 IS - 1349-7235 (Electronic) IS - 0918-2918 (Linking) VI - 48 IP - 5 DP - 2009 TI - Primitive neuroectodermal tumor of the pancreas. PG - 329-33 AB - 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. FAU - Doi, Hirokazu AU - Doi H AD - Department of Gastroenterology, Ehime Prefectural Central Hospital, Matsuyama. FAU - Ichikawa, Soichi AU - Ichikawa S FAU - Hiraoka, Atsushi AU - Hiraoka A FAU - Ichiryu, Misa AU - Ichiryu M FAU - Nakahara, Hiromasa AU - Nakahara H FAU - Ochi, Hironori AU - Ochi H FAU - Tanabe, Atsushi AU - Tanabe A FAU - Kodama, Akihiro AU - Kodama A FAU - Hasebe, Aki AU - Hasebe A FAU - Miyamoto, Yasunao AU - Miyamoto Y FAU - Ninomiya, Tomoyuki AU - Ninomiya T FAU - Horiike, Norio AU - Horiike N FAU - Takamura, Kazuhito AU - Takamura K FAU - Kawasaki, Hideki AU - Kawasaki H FAU - Kameoka, Chieko AU - Kameoka C FAU - Kan, Miki AU - Kan M FAU - Doi, Shigekazu AU - Doi S FAU - Soga, Yoshiko AU - Soga Y FAU - Tamura, Hiromi AU - Tamura H FAU - Maeda, Toshiharu AU - Maeda T FAU - Asaki, Akinori AU - Asaki A FAU - Seno, Seijin AU - Seno S FAU - Iguchi, Haruo AU - Iguchi H FAU - Hasegawa, Tadashi AU - Hasegawa T LA - eng PT - Case Reports PT - Journal Article DEP - 20090302 PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 RN - 0 (Calmodulin-Binding Proteins) RN - 0 (EWSR1 protein, human) RN - 0 (RNA-Binding Protein EWS) RN - 0 (RNA-Binding Proteins) SB - IM MH - Adult MH - Calmodulin-Binding Proteins/genetics MH - Drug Therapy MH - Humans MH - Male MH - Neuroectodermal Tumors, Primitive/*diagnosis/drug therapy/surgery MH - Pancreatic Neoplasms/*diagnosis/drug therapy/surgery MH - Positron-Emission Tomography MH - RNA-Binding Protein EWS MH - RNA-Binding Proteins/genetics EDAT- 2009/03/03 09:00 MHDA- 2009/06/06 09:00 CRDT- 2009/03/03 09:00 PHST- 2009/03/03 09:00 [entrez] PHST- 2009/03/03 09:00 [pubmed] PHST- 2009/06/06 09:00 [medline] AID - JST.JSTAGE/internalmedicine/48.1484 [pii] AID - 10.2169/internalmedicine.48.1484 [doi] PST - ppublish SO - Intern Med. 2009;48(5):329-33. doi: 10.2169/internalmedicine.48.1484. Epub 2009 Mar 2.