Recent developments in gastroesophageal mesenchymal tumours

Histopathology. 2021 Jan;78(1):171-186. doi: 10.1111/his.14164.

Abstract

The pathologist's approach to gastroesophageal mesenchymal tumours has changed dramatically during the last 25 years. In particular, gastrointestinal stromal tumour (GIST) has evolved from a wastebasket mesenchymal tumour category to a precisely defined entity with an increasingly detailed genetic subclassification. This subclassification has brought gastrointestinal mesenchymal neoplasia into the realm of precision medicine, with specific treatments optimised for particular genetic subtypes. Molecular genetic data have also greatly improved our understanding of oesophageal mesenchymal tumours, including the discovery that so-called 'giant fibrovascular polyps' in fact represent a clinically distinctive presentation of well-differentiated liposarcoma. Here, we will focus on gastroesophageal mesenchymal tumours for which there have been recent developments in classification, molecular genetics or tumour biology: granular cell tumour, 'giant fibrovascular polyp'/well-differentiated liposarcoma, plexiform fibromyxoma, gastroblastoma and, of course, GIST.

Keywords: gastrointestinal stromal tumour; granular cell tumour; soft tissue tumours; succinate dehydrogenase; well-differentiated liposarcoma.

Publication types

  • Review

MeSH terms

  • Esophageal Neoplasms / pathology*
  • Esophagogastric Junction / pathology*
  • Gastrointestinal Stromal Tumors / pathology*
  • Gastrointestinal Tract / pathology
  • Humans
  • Stomach Neoplasms / pathology*