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01.12.2012 | Case report | Ausgabe 1/2012 Open Access

World Journal of Surgical Oncology 1/2012

A case of pancreatic neuroendocrine tumor in a patient with neurofibromatosis-1

World Journal of Surgical Oncology > Ausgabe 1/2012
Takeshi Nishi, Yasunari Kawabata, Youko Hari, Hiroshi Imaoka, Noriyoshi Ishikawa, Seiji Yano, Riruke Maruyama, Yoshitsugu Tajima
Wichtige Hinweise

Electronic supplementary material

The online version of this article (doi:10.​1186/​1477-7819-10-153) contains supplementary material, which is available to authorized users.

Competing interests

The authors declare that they have no competing interests.

Authors’ contributions

TN carried out the surgical procedure, designed the report, analyzed all the reports, and drafted the manuscript. YK, HY, and SY carried out the surgical procedure and participated in designing the manuscript. HI performed all examinations and made the diagnosis. NI performed the histological analysis of the surgical specimens and provided histological sections as figures for the manuscript. RM performed the histological analysis of the surgical specimens and analyzed the expression of neurofibromin in the immunohistochemistry. YT participated in designing the report and revised the manuscript for submission. All authors have read and approved the final manuscript.


Patients with neurofibromatosis-1 (NF-1) sometime develop neuroendocrine tumors (NET). Although these NETs usually occur in the duodenum or peri-ampullary region, they occasionally grow in the pancreas (PNET). A 62-year-old man with NF-1 had mild liver dysfunction and was admitted to our hospital for further examination. An abdominal contrast-enhanced computed tomography scan demonstrated a 30-mm tumor in the head of the pancreas. The scan showed an invasion of the tumor into the duodenum, and biopsy under an endoscopic ultrasonography indicated that the tumor was a NET. A subtotal stomach-preserving pancreaticoduodenectomy was performed. Macroscopically, the pancreatic tumor was white and elastic hard. Microscopically, tumor cells were composed of ribbons, cords, and solid nests with an acinus-like structure. The tumor was diagnosed as NET G2 according to the WHO classification (2010). The product of theNF-1 gene, i.e., neurofibromin, was weakly positive in the tumor cells, suggesting that the tumor was induced by a mutation in the NF-1 gene. This is the seventh case of PNET arising in NF-1 patients worldwide.
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