title: Peripheral primitive neuroectodermal tumors arising in the pancreas: the first case report in Asia and a review of the 14 total reported cases in the world.
abstract: OBJECTIVE
  To discuss the diagnosis and treatment of peripheral primitive neuroectodermal tumors of the pancreas based on our case and all the cases in the world.
  
  
  METHODS
  The first case of peripheral primitive neuroectodermal tumors of the pancreas in Asia was preliminarily reported by our group in 2006. The patient underwent three operations for the primary tumor and recurrences over 41 months prior to the patient's death in November 2007. All 14 reported cases of pancreatic PNETs in the world were analyzed. The corresponding literatures on its diagnosis and treatment of were reviewed.
  
  
  RESULTS
  A 13 year-old female patient was diagnosed with pancreatic PNETs by the clinical, microscopic, immunohistochemical features, and cytogenetic analysis after the resection of the tumor located in the uncinate process of the pancreas at PUMC Hospital. During the follow-up course, radiotherapy and chemotherapy were given after the first operation. Two additional operations were performed 10 months and 25 months after the first one, respectively, because of tumor recurrence. The patient died 41 months after the initial diagnosis with the recurrence and metastasis that were not suitable for a further surgery. Primitive neuroectodermal tumors of the pancreas are extremely rare. A review of the world's literature on this tumor identified fourteen cases with a mean survival time of 12 months (ranging from 6 to 50 months). These patients often have no specific clinical symptoms, but most do present with abdominal pain and/or jaundice. The diagnosis is established by small round tumor cells seen on light microscopy, immunohistochemical features of positive P30/32(MIC2) with at least two positive neuronal markers., and cytogenetic analysis showing characteristic translocation of t[11;22][q24;q12]. Since pancreatic PNETs are highly aggressive, early diagnosis, immediate surgical resection and re-resection if possible, early radiotherapy and chemotherapy and close follow-up are required.
  
  
  CONCLUSIONS
  Peripheral primitive neuroectodermal tumors can arise in pancreas. The diagnosis and treatment should be made as early as possible, aggressive surgeries for the primary and recurrences may help to improve the prognosis.
authors.affiliation: Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China;
authors.forename: Yilei
authors.initials: Y
authors.lastname: Mao
authors.affiliation: Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China;
authors.forename: Xinting
authors.initials: X
authors.lastname: Sang
authors.affiliation: Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China;
authors.forename: Naixin
authors.initials: N
authors.lastname: Liang
authors.affiliation: Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China;
authors.forename: Huayu
authors.initials: H
authors.lastname: Yang
authors.affiliation: Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China;
authors.forename: Xin
authors.initials: X
authors.lastname: Lu
authors.affiliation: Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China;
authors.forename: Zhiying
authors.initials: Z
authors.lastname: Yang
authors.affiliation: Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China;
authors.forename: Shunda
authors.initials: S
authors.lastname: Du
authors.affiliation: Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China;
authors.forename: Yiyao
authors.initials: Y
authors.lastname: Xu
authors.affiliation: Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China;
authors.forename: Haitao
authors.initials: H
authors.lastname: Zhao
authors.affiliation: Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China;
authors.forename: Shouxian
authors.initials: S
authors.lastname: Zhong
authors.affiliation: Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China;
authors.forename: Jiefu
authors.initials: J
authors.lastname: Huang
authors.affiliation: Department of Surgery, University of Chicago, Chicago Il, USA.
authors.forename: J Michael
authors.initials: JM
authors.lastname: Millis
keywords: Ewing’s sarcoma; Peripheral primitive neuroectodermal tumors; pancreatic tumor; small round cell tumors