Multiple familial gastrointestinal autonomic nerve tumors and small intestinal neuronal dysplasia

Am J Surg Pathol. 1999 Feb;23(2):198-204. doi: 10.1097/00000478-199902000-00009.

Abstract

Multiple small intestinal stromal tumors were removed from mother and natural daughter within 15 months of each other. Both had long histories of recurrent iron deficiency anemia and upper gastrointestinal bleeding. Light microscopy revealed that the tumors had arisen in conjunction with diffuse hyperplasia/ dysplasia of Auerbach's myenteric plexus. Immunohistochemistry generally did not show myogenic or paraganglionic phenotypes; CD34 was positive in most tumors. Electron microscopy confirmed the association with the abnormal Auerbach's plexus and showed the structure of gastrointestinal autonomic nerve tumors (GANTs). These findings provide information as to the origin and evolution of GANTs, and also have implications for the clinical management of these tumors which appear to occur more frequently than previously thought.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Antigens, CD34 / analysis
  • Biomarkers, Tumor / analysis
  • Female
  • Gastrointestinal Neoplasms / chemistry
  • Gastrointestinal Neoplasms / diagnostic imaging
  • Gastrointestinal Neoplasms / pathology*
  • Gastrointestinal Neoplasms / surgery
  • Humans
  • Hyperplasia / pathology
  • Immunoenzyme Techniques
  • Intestine, Small / diagnostic imaging
  • Intestine, Small / innervation*
  • Intestine, Small / pathology*
  • Intestine, Small / surgery
  • Middle Aged
  • Myenteric Plexus / pathology
  • Organelles / ultrastructure
  • Pedigree
  • Peripheral Nervous System Neoplasms / chemistry
  • Peripheral Nervous System Neoplasms / diagnostic imaging
  • Peripheral Nervous System Neoplasms / pathology*
  • Peripheral Nervous System Neoplasms / surgery
  • Phosphopyruvate Hydratase / analysis
  • Sarcoma / chemistry
  • Sarcoma / diagnostic imaging
  • Sarcoma / pathology*
  • Sarcoma / surgery
  • Tomography, X-Ray Computed

Substances

  • Antigens, CD34
  • Biomarkers, Tumor
  • Phosphopyruvate Hydratase