Microcystic tubulopapillary carcinoma of the pancreas: a new tumor entity?
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.
Esposito
Irene
I
Department of Pathology, University of Heidelberg, Im Neuenheimer Feld 220, 69120, Heidelberg, Germany. irene_esposito@med.uni-heidelberg.de
Bauer
Andrea
A
Hoheisel
Jörg D
JD
Kleeff
Jörg
J
Friess
Helmut
H
Bergmann
Frank
F
Rieker
Ralf J
RJ
Otto
Herwart F
HF
Klöppel
Günter
G
Penzel
Roland
R
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
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