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Microcystic tubulopapillary carcinoma of the pancreas: a new tumor entity?

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Abstract

An unusual pancreatic tumor with microcystic and tubulopapillary features was observed in a 53-year-old woman. The tumor presented as a large, focally cystic mass in the head of the pancreas, which compressed the surrounding structures. The histological and immunohistochemical analysis revealed a neoplasm that could not be assigned to any of the known pancreatic tumor types. At the molecular level, the tumor showed inactivation of the DPC4/SMAD4 gene, deletion of exon 1 of the p16INK4A gene and a point mutation at codon 34 (GGA>AGA) of β-catenin. Transcriptional profiling analyses and subsequent correspondence cluster analysis demonstrated that the transcriptional profile of the tumor differed distinctly from that of ductal adenocarcinomas, pancreatic cystic tumors and normal pancreatic tissues. These data suggest that the neoplasm most likely represents a new pancreatic tumor entity, which we would like to refer to as microcystic tubulopapillary tumor.

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Authors and Affiliations

  1. Department of Pathology, University of Heidelberg, Im Neuenheimer Feld 220, 69120 , Heidelberg, Germany

    Irene Esposito, Frank Bergmann, Ralf J. Rieker, Herwart F. Otto & Roland Penzel

  2. Department of Functional Genome Analysis, German Cancer Research Center, Heidelberg, Germany

    Andrea Bauer & Jörg D. Hoheisel

  3. Department of General Surgery, University of Heidelberg, Germany

    Jörg Kleeff & Helmut Friess

  4. Department of Pathology, University of Kiel, Germany

    Günter Klöppel

Authors
  1. Irene Esposito
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  2. Andrea Bauer
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  3. Jörg D. Hoheisel
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  4. Jörg Kleeff
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  5. Helmut Friess
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  6. Frank Bergmann
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  7. Ralf J. Rieker
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  8. Herwart F. Otto
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  9. Günter Klöppel
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  10. Roland Penzel
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Correspondence to Irene Esposito.

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Esposito, I., Bauer, A., Hoheisel, J.D. et al. Microcystic tubulopapillary carcinoma of the pancreas: a new tumor entity?. Virchows Arch 444, 447–453 (2004). https://doi.org/10.1007/s00428-004-0984-6

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  • DOI: https://doi.org/10.1007/s00428-004-0984-6

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