Thymoma-associated multiorgan autoimmunity with exclusive gastrointestinal tract involvement: case report and review of the literature

Virchows Arch. 2018 Jul;473(1):121-125. doi: 10.1007/s00428-018-2304-6. Epub 2018 Jan 26.

Abstract

Thymoma-associated multiorgan autoimmunity (TAMA) is a recently delineated and rare paraneoplastic syndrome reported in patients with thymoma. The disorder is characterized by graft-versus-host disease-like pathology affecting the skin, gastrointestinal tract (GIT), and liver, and is usually associated with a poor outcome. We document a case of TAMA with exclusive GIT involvement which included the stomach, small and large bowel, presenting in a 66-year-old male patient 5 years after complete resection of a type B2 thymoma. A brief review is provided of this scarce syndrome, the GIT pathology described in the 21 TAMA cases reported to date, and the unique characteristics of patients with exclusive GIT involvement by this acquired autoimmune disorder.

Keywords: Graft-versus-host disease-like gastroenterocolitis; Paraneoplastic syndrome; Secondary autoimmune enteropathy; Thymoma-associated multiorgan autoimmunity.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Autoimmune Diseases / immunology*
  • Autoimmunity*
  • Gastrointestinal Tract / pathology*
  • Humans
  • Male
  • Skin / pathology
  • Thymoma / pathology*
  • Thymus Neoplasms / diagnosis
  • Thymus Neoplasms / pathology*