Serotonin-producing enterochromaffin cell tumors of the pancreas: Clinicopathologic study of 15 cases and comparison with intestinal enterochromaffin cell tumors

Stefano La Rosa, Francesca Franzi, Luca Albarello, Anja Schmitt, Barbara Bernasconi, Maria Grazia Tibiletti, Giovanna Finzi, Claudia Placidi, Aurel Perren, Carlo Capella

Research output: Contribution to journalArticlepeer-review

Abstract

Objectives: Serotonin-producing tumors of the pancreas are rare endocrine neoplasms composed of enterochromaffin (EC) cells that have been mainly described in the literature as case reports. This study analyzes the clinicopathologic features of a series of pancreatic EC cell neoplasms and their similarities to and differences from intestinal EC cell tumors. Methods: The morphological, immunohistochemical, ultrastructural, and fluorescent in situ hybridization features of 15 pancreatic and 20 intestinal serotonin-producing neoplasms were compared. In addition, we reviewed the literature on pancreatic serotonin-producing tumors to better understand the clinicopathologic features of this rare tumor type. Results: The lack of substance P and acidic fibroblast growth factor immunoreactivity; the low immunohistochemical expression of CDX2, vesicular monoamine transporter 1, connective tissue growth factor, and prostatic acid phosphatase; the lack of S100-positive sustentacular cells; the strong expression of vesicular monoamine transporter 2; and peculiar ultrastructural features characterize pancreatic EC cell tumors and differentiate them from intestinal ones, although both categories show similar chromosome 18 cytogenetic alterations. The review of the literature indicates that pancreatic functioning tumors associated with the carcinoid syndrome arise in younger patients and are larger, more frequently malignant, and more aggressive neoplasms than pancreatic nonfunctioning ones. Conclusions: Pancreatic EC cell tumors show several different morphological features compared with related intestinal tumors despite similar cytogenetic alterations on chromosome 18.

Original languageEnglish
Pages (from-to)883-895
Number of pages13
JournalPancreas
Volume40
Issue number6
DOIs
Publication statusPublished - Aug 2011

Keywords

  • carcinoid
  • EC cell
  • endocrine tumor
  • pancreas
  • serotonin

ASJC Scopus subject areas

  • Hepatology
  • Internal Medicine
  • Endocrinology
  • Endocrinology, Diabetes and Metabolism

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