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

Irene Esposito; Andrea Bauer; Jörg D Hoheisel; Jörg Kleeff; Helmut Friess; Frank Bergmann; Ralf J Rieker; Herwart F Otto; Günter Klöppel; Roland Penzel

doi:10.1007/s00428-004-0984-6

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

Virchows Arch. 2004 May;444(5):447-53. doi: 10.1007/s00428-004-0984-6. Epub 2004 Mar 11.

Authors

Irene Esposito¹, Andrea Bauer, Jörg D Hoheisel, Jörg Kleeff, Helmut Friess, Frank Bergmann, Ralf J Rieker, Herwart F Otto, Günter Klöppel, Roland Penzel

Affiliation

¹ Department of Pathology, University of Heidelberg, Im Neuenheimer Feld 220, 69120, Heidelberg, Germany. [email protected]

PMID: 15014986
DOI: 10.1007/s00428-004-0984-6

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 p16(INK4A) gene and a point mutation at codon 34 (GGA>AGA) of beta-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.

Publication types

Case Reports

MeSH terms

Adenocarcinoma / chemistry
Adenocarcinoma / genetics
Adenocarcinoma / pathology*
Biomarkers, Tumor / analysis
Carcinoma, Papillary / chemistry
Carcinoma, Papillary / genetics
Carcinoma, Papillary / pathology*
Cell Nucleus / ultrastructure
Cyclin-Dependent Kinase Inhibitor p16 / analysis
Cyclin-Dependent Kinase Inhibitor p16 / genetics
Cytoskeletal Proteins / analysis
Cytoskeletal Proteins / genetics
DNA, Neoplasm / analysis
DNA-Binding Proteins / analysis
DNA-Binding Proteins / genetics
Female
Gene Deletion
Gene Silencing
Humans
Immunohistochemistry
Middle Aged
Oligonucleotide Array Sequence Analysis
Pancreas / surgery
Pancreatic Cyst / pathology*
Pancreatic Neoplasms / chemistry
Pancreatic Neoplasms / genetics
Pancreatic Neoplasms / pathology*
Pancreaticoduodenectomy
Point Mutation
Smad4 Protein
Trans-Activators / analysis
Trans-Activators / genetics
beta Catenin

Substances

Biomarkers, Tumor
CTNNB1 protein, human
Cyclin-Dependent Kinase Inhibitor p16
Cytoskeletal Proteins
DNA, Neoplasm
DNA-Binding Proteins
SMAD4 protein, human
Smad4 Protein
Trans-Activators
beta Catenin