Pathological differential diagnosis of solid-pseudopapillary neoplasm and endocrine tumors of the pancreas

World J Gastroenterol. 2010 Feb 28;16(8):1025-30. doi: 10.3748/wjg.v16.i8.1025.

Abstract

Aim: To investigate differential points of solid-pseudopapillary neoplasm (SPN) of the pancreas and pancreatic endocrine tumor (PET).

Methods: Ten cases of SPN and fourteen cases of PET were studied in this retrospective study. Clinical and pathologic features, immunostaining reactions and beta-catenin gene mutations were analyzed.

Results: The mean age of SPN patients was 25.6 years and these patients had no specific symptoms. The mean diameter of the tumors was 11.0 cm, 9/10 cases were cystic or a mixture of solid and cystic structures, and there was hemorrhage and necrosis on the cut surface in 8/10 (80%) cases. Characteristic pseudopapillary structure and discohesive appearance of the neoplastic cells were observed in all 10 (100%) cases. The results of immunostaining showed that nuclear expression of beta-catenin and loss of E-cadherin in all the cases, was only seen in SPN. Molecular studies discovered that 9/10 (90%) cases harbored a point mutation of exon 3 in beta-catenin gene. On the other hand, the mean age of PET patients was 43.1 years. Eight of 14 cases presented with symptoms caused by hypoglycemia, and the other 6 cases presented with symptoms similar to those of SPN. The mean size of the tumors was 2.9 cm, most of the tumors were solid, only 3/14 (21%) were a mixture of solid and cystic structures, and macroscopic hemorrhage and necrosis were much less common (3/14, 21%). Histologically, tumor cells were arranged in trabecular, acinar or solid patterns and demonstrated no pseudopapillary structure and discohesive appearance in all 14 (100%) cases. The results of immunostaining and mutation detection were completely different with SPN that membrane and cytoplastic expression of beta-catenin without loss of E-cadherin, as well as no mutation in beta-catenin gene in all the cases.

Conclusion: Both macroscopic and microscopic features of SPN are quite characteristic. It is not difficult to distinguish it from PET. If necessary, immunostaining of beta-catenin and E-cadherin is quite helpful to make the differential diagnosis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Base Sequence
  • Cadherins / genetics
  • Cadherins / metabolism
  • DNA Mutational Analysis
  • Endocrine Gland Neoplasms / diagnosis*
  • Endocrine Gland Neoplasms / genetics
  • Endocrine Gland Neoplasms / pathology*
  • Female
  • Humans
  • Male
  • Middle Aged
  • Molecular Sequence Data
  • Mutation
  • Pancreas / anatomy & histology
  • Pancreas / metabolism
  • Pancreas / pathology
  • Pancreatic Neoplasms / diagnosis*
  • Pancreatic Neoplasms / genetics
  • Pancreatic Neoplasms / pathology*
  • Retrospective Studies
  • Young Adult
  • beta Catenin / genetics
  • beta Catenin / metabolism

Substances

  • Cadherins
  • beta Catenin