Non-cystic solid-pseudopapillary tumor of the pancreas showing nuclear accumulation and activating gene mutation of beta-catenin

Pathol Int. 2006 Nov;56(11):707-11. doi: 10.1111/j.1440-1827.2006.02034.x.

Abstract

Solid-pseudopapillary tumor (SPT) is an unusual pancreatic neoplasm that is characterized by a mixture of solid and cystic components and a fibrous capsule. Recently, the tumorigenesis of SPT has been reported to be associated with gene mutations of beta-catenin, which is a molecule participating in the Wnt signaling pathway. Reported herein is the case of a 53-year-old woman with SPT. The tumor, approximately 3 cm in diameter in the pancreas body, had a clear margin and central calcification but had neither a cystic component nor fibrous capsule. Several lines of pathological findings in the surgically resected specimen indicated SPT: (i) pseudopapillary proliferation of eosinophilic polygonal cells with oval nuclei; (ii) positive expression of several marker molecules indicating differentiation into acinar and endocrine cells; and (iii) zymogen granule-like structures in the cytoplasm on electron microscopy. Further, the tumor cells had intense nuclear accumulation of beta-catenin and an activating mutation, (34)Gly(GGA) to Arg(AGA), in exon 3 of the beta-catenin gene, as previously reported in most SPT. These findings suggest that association of the beta-catenin phenotype with development of the rare phenotype of SPT, a non-cystic and unencapsulated tumor, is unlikely.

Publication types

  • Case Reports

MeSH terms

  • Biomarkers, Tumor / metabolism
  • Cell Nucleus / pathology*
  • Cystadenoma, Papillary / genetics
  • Cystadenoma, Papillary / metabolism
  • Cystadenoma, Papillary / pathology*
  • DNA Mutational Analysis
  • Disease-Free Survival
  • Female
  • Humans
  • Middle Aged
  • Mutation*
  • Pancreatectomy
  • Pancreatic Neoplasms / genetics
  • Pancreatic Neoplasms / metabolism
  • Pancreatic Neoplasms / pathology*
  • Secretory Vesicles / ultrastructure
  • Sequence Analysis, DNA
  • beta Catenin / genetics*
  • beta Catenin / metabolism

Substances

  • Biomarkers, Tumor
  • beta Catenin