Pathological Findings in Myasthenia Gravis Patients with Thymic Hyperplasia and Thymoma

Pathol Oncol Res. 2018 Jan;24(1):67-74. doi: 10.1007/s12253-017-0213-7. Epub 2017 Mar 15.

Abstract

Thymectomy is routinely carried out in patients with myasthenia gravis (MG) and thymomas. However, there is still a dispute as to whether MG patients with thymic hyperplasia should undergo thymectomy. We aimed to investigate the pathological findings in the thymus in patients with co-existing MG and thymic hyperplasia or thymomas treated with thymectomy, as well as effects of immunosuppression. Thirty-three patients with MG were selected and grouped accordingly: patients with no thymic abnormalities, patients with thymic hyperplasia, and patients with thymomas. All patients were treated with methylprednisolone alongside immunosuppression. A separate cohort of 24 MG patients with thymic hyperplasia or thymomas and treated with thymectomy were selected. As controls, 5 patients with thymomas or thymic carcinoma without MG were selected. Expression of CD5, extracellular regulated protein kinases1/2 mitogen activated protein kinase (ERK1/2MAPKs) and CD95 ligand (FasL) in the thymus was examined. Methylprednisolone and immunosuppressive therapy are highly effective in MG patients with normal thymus tissue and MG patients with thymic hyperplasia compared to MG patients with thymomas alone. CD5 expression was highest in MG patients with thymic hyperplasia, correlating with expression of ERK1/2MAPKs. FasL expression was similar across all groups. Thymomas may be distinguished from thymic hyperplasia by expression of CD5 and ERK1/2MAPKs. Thymectomy is the preferred treatment for MG patients with thymomas but may not be necessary in MG patients with thymic hyperplasia who are treated with immunosuppressive therapy.

Keywords: Myasthenia gravis; Thymectomy; Thymic hyperplasia; Thymoma.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Biomarkers, Tumor / metabolism*
  • Case-Control Studies
  • Child
  • Fas Ligand Protein / metabolism
  • Female
  • Follow-Up Studies
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Intercellular Adhesion Molecule-3 / metabolism
  • Male
  • Middle Aged
  • Mitogen-Activated Protein Kinase 1 / metabolism
  • Mitogen-Activated Protein Kinase 3 / metabolism
  • Myasthenia Gravis / drug therapy
  • Myasthenia Gravis / metabolism
  • Myasthenia Gravis / pathology*
  • Prognosis
  • Retrospective Studies
  • Thymoma / drug therapy
  • Thymoma / metabolism
  • Thymoma / pathology*
  • Thymus Hyperplasia / drug therapy
  • Thymus Hyperplasia / metabolism
  • Thymus Hyperplasia / pathology*
  • Thymus Neoplasms / drug therapy
  • Thymus Neoplasms / metabolism
  • Thymus Neoplasms / pathology*
  • Young Adult

Substances

  • Biomarkers, Tumor
  • FASLG protein, human
  • Fas Ligand Protein
  • Immunosuppressive Agents
  • Intercellular Adhesion Molecule-3
  • MAPK1 protein, human
  • Mitogen-Activated Protein Kinase 1
  • Mitogen-Activated Protein Kinase 3