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Primary acinar cell carcinoma of the ampulla of Vater

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Acinar cell carcinoma of the pancreatobiliary system is a relatively rare malignant neoplasm arising usually in the pancreatic parenchyma. We experienced a 68-year-old woman who presented with obstructive jaundice due to an ampullary mass 1.0 cm in diameter, detected by abdominal computed tomography and endoscopic examination. The patient underwent a curative surgical operation, and histopathological examination revealed that the tumor was confined to the ampulla of Vater with no continuity to the pancreatic parenchyma. The tumor cells showed acinar or tubular arrangement with eosinophilic to basophilic granular cytoplasm, findings identical to those of acinar cell carcinoma of the pancreas. Immunohistochemically, the tumor cells were positive for lipase. From these findings, we concluded that the tumor was primary acinar cell carcinoma arising in the ampulla of Vater, probably originating from heterotopic pancreatic tissue. This is the first reported case of primary acinar cell carcinoma in the ampulla of Vater.

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  1. H Kuerer H Shim D Pertsemlidis P Unger (1997) ArticleTitleFunctioning pancreatic acinar cell carcinoma: immunohistochemical and ultrastructural analysesx Eur J Clin Oncol 20 101–7 Occurrence Handle1:STN:280:ByiC2s3itFI%3D

    CAS  Google Scholar 

  2. GG Hartman H Ni J Pickleman (2001) ArticleTitleAcinar cell carcinoma of the pancreas Arch Pathol Lab Med 125 1127–8 Occurrence Handle11473479 Occurrence Handle1:STN:280:DC%2BD3MvislWisg%3D%3D

    PubMed  CAS  Google Scholar 

  3. DS Klimstra CS Heffess JE Oertel J Rosai (1992) ArticleTitleAcinar cell carcinoma of the pancreas. A clinicopathological study of 28 cases Am J Surg Pathol 16 815–37 Occurrence Handle1384374 Occurrence Handle10.1097/00000478-199209000-00001 Occurrence Handle1:STN:280:ByyD3snhvVU%3D

    Article  PubMed  CAS  Google Scholar 

  4. R Jaffe (1991) The pancreas JS Wigglesworth DB Singer (Eds) Textbook of fetal and perinatal pathology. Vol. 2 Blackwell Scientific Boston 1021–55

    Google Scholar 

  5. CH Chen CC Yang YH Yeh DA Chou CL Kuo (2001) ArticleTitleEctopic pancreas located in the major duodenal papilla: case report and review Gastrointest Endosc 53 121–3 Occurrence Handle11154509 Occurrence Handle10.1067/mge.2001.111396 Occurrence Handle1:CAS:528:DC%2BD3MXnsFWjsrg%3D

    Article  PubMed  CAS  Google Scholar 

  6. HR Makhlouf JL Almeida LH Sobin (1999) ArticleTitleCarcinoma of the jejunal pancreatic heterotopia Arch Pathol Lab Med 123 707–11 Occurrence Handle10420228 Occurrence Handle1:STN:280:DyaK1MzltVWitQ%3D%3D

    PubMed  CAS  Google Scholar 

  7. J Hennings U Garske J Botling P Hellman (2005) ArticleTitleMalignant insulinoma in ectopic pancreatic tissue Dig Surg 22 377–9 Occurrence Handle16432300 Occurrence Handle10.1159/000090998

    Article  PubMed  Google Scholar 

  8. JT Moncur BE Lacy DS Longnecker (2002) ArticleTitleMixed acinar–endocrine carcinoma in the ampulla of Vater Hum Pathol 33 449–51 Occurrence Handle12055683 Occurrence Handle10.1053/hupa.2002.124040

    Article  PubMed  Google Scholar 

  9. Y Sun PG Wasserman (2004) ArticleTitleAcinar cell carcinoma arising in the stomach: a case report with literature review Hum Pathol 35 263–5 Occurrence Handle14991547 Occurrence Handle10.1016/j.humpath.2003.09.020

    Article  PubMed  Google Scholar 

  10. L Guillou P Nordback C Gerber RP Schneider (1994) ArticleTitleDuctal adenocarcinoma arising in a heterotopic pancreas situated in a hiatal hernia Arch Pathol Lab Med 118 568–71 Occurrence Handle8192567 Occurrence Handle1:STN:280:ByuB2M%2Fht10%3D

    PubMed  CAS  Google Scholar 

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Kawakami, H., Kuwatani, M., Onodera, M. et al. Primary acinar cell carcinoma of the ampulla of Vater. J Gastroenterol 42, 694–697 (2007).

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