title: Microcystic tubulopapillary carcinoma of the pancreas: a new tumor entity?
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.
authors.affiliation: Department of Pathology, University of Heidelberg, Im Neuenheimer Feld 220, 69120, Heidelberg, Germany. irene_esposito@med.uni-heidelberg.de
authors.forename: Irene
authors.initials: I
authors.lastname: Esposito
authors.forename: Andrea
authors.initials: A
authors.lastname: Bauer
authors.forename: Jörg D
authors.initials: JD
authors.lastname: Hoheisel
authors.forename: Jörg
authors.initials: J
authors.lastname: Kleeff
authors.forename: Helmut
authors.initials: H
authors.lastname: Friess
authors.forename: Frank
authors.initials: F
authors.lastname: Bergmann
authors.forename: Ralf J
authors.initials: RJ
authors.lastname: Rieker
authors.forename: Herwart F
authors.initials: HF
authors.lastname: Otto
authors.forename: Günter
authors.initials: G
authors.lastname: Klöppel
authors.forename: Roland
authors.initials: R
authors.lastname: Penzel
mesh_terms: D000230:Adenocarcinoma / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D014408:Biomarkers, Tumor / Q000032:analysis; D002291:Carcinoma, Papillary / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D002467:Cell Nucleus / Q000648:ultrastructure; D019941:Cyclin-Dependent Kinase Inhibitor p16 / Q000032:analysis / Q000235:genetics; D003598:Cytoskeletal Proteins / Q000032:analysis / Q000235:genetics; D004273:DNA, Neoplasm / Q000032:analysis; D004268:DNA-Binding Proteins / Q000032:analysis / Q000235:genetics; D005260:Female; D017353:Gene Deletion; D020868:Gene Silencing; D006801:Humans; D007150:Immunohistochemistry; D008875:Middle Aged; D020411:Oligonucleotide Array Sequence Analysis; D010179:Pancreas / Q000601:surgery; D010181:Pancreatic Cyst / Q000473:pathology*; D010190:Pancreatic Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D016577:Pancreaticoduodenectomy; D017354:Point Mutation; D051901:Smad4 Protein; D015534:Trans-Activators / Q000032:analysis / Q000235:genetics; D051176:beta Catenin
chemical_list: D014408:Biomarkers, Tumor; C495270:CTNNB1 protein, human; D019941:Cyclin-Dependent Kinase Inhibitor p16; D003598:Cytoskeletal Proteins; D004273:DNA, Neoplasm; D004268:DNA-Binding Proteins; C497796:SMAD4 protein, human; D051901:Smad4 Protein; D015534:Trans-Activators; D051176:beta Catenin