General Thoracic and Cardiovascular Surgery

, Volume 56, Issue 4, pp 143–150 | Cite as

Immunological function of thymoma and pathogenesis of paraneoplastic myasthenia gravis

  • Meinoshin Okumura
  • Yoshitaka Fujii
  • Hiroyuki Shiono
  • Masayoshi Inoue
  • Masato Minami
  • Tomoki Utsumi
  • Yoshihisa Kadota
  • Yoshiki Sawa
Current Topics Review Article

Abstract

Thymoma and thymic carcinoma are the representative tumors arising from the thymic epithelium. Thymoma is well known for association with autoimmune diseases including myasthenia gravis, suggesting its biological activity. Herein, recent progress in research of thymoma is reviewed with reference to its immunological function. Myasthenia gravis is frequently associated with WHO type B1 and B2 thymomas. These types of thymomas hold a significant number of CD4+CD8+ double-positive T cells, and at the same time, the neoplastic epithelial cells express HLA-DR molecules at a slightly reduced level compared with the normal thymus. The impaired expression of HLA-DR molecules in neoplastic epithelial cells of thymomas possibly affects positive selection of CD4+CD8 single-positive T cells and may result in alteration of its repertoire. The function of thymoma neoplastic cells as the cortical epithelium of the thymus and the morphological resemblance of thymomas to the cortex suggest that thymoma is of cortical epithelial origin; this might imply that thymoma lacks the functional medulla where professional antigenpresenting cells are engaged in negative selection. These findings suggest that thymoma generates autoreactive T cells causing autoimmunity. Further investigation on immunological function of thymoma is supposed to elucidate the pathogenesis of thymoma-related autoimmunity and the high affinity of thymoma with myasthenia gravis. In addition, studying the biology of thymoma is also expected to contribute to further understanding of T-cell development and immunological tolerance in the human, because thymoma can be considered an acquired thymus.

Key words

Thymoma Thymic carcinoma T-cell development Human leukocyte antigen Paraneoplastic autoimmunity 

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Copyright information

© The Japanese Association for Thoracic Surgery 2008

Authors and Affiliations

  • Meinoshin Okumura
    • 1
  • Yoshitaka Fujii
    • 2
  • Hiroyuki Shiono
    • 1
  • Masayoshi Inoue
    • 1
  • Masato Minami
    • 1
  • Tomoki Utsumi
    • 1
  • Yoshihisa Kadota
    • 1
  • Yoshiki Sawa
    • 1
  1. 1.Department of General Thoracic Surgery (L5)Osaka University Graduate School of MedicineOsakaJapan
  2. 2.Department of Surgery IINagoya City University Medical SchoolNagoyaJapan

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