Thymoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Amr Marawan, M.D. [2] Ahmad Al Maradni, M.D. [3]

Overview

  • On gross pathology, a well circumscribed mass that is locally invasive is a characteristic finding of thymoma.
  • On microscopic histopathological analysis, round cells, with ample vacuolated cytoplasms, and fat droplets are characteristic findings of thymoma.

Pathophysiology

Physiology

  • Thymus is the site of maturation of T cells.
  • This makes thymus the primary center of adaptive immunity.

Pathogenesis

  • The exact pathogenesis of the primary tumor development is not well understood.
  • Primary tumors of thymus are relatively rare.
  • Thymoma is the most common type of primary tumor of thymus.
  • Thymoma is histologically comprised of abnormally conditioned T cells.
  • The mingling of these abnormal T cells into the circulation is believed to be involved in the associated autoimmune disorders.[1][2]

Genetics

Genetic alterations reported for the different WHO histological thymomasubtypes[3]

WHO Type Chromosomal Gains Chromosomal Losses
Type A none -6p
Type AB none -5q21-22,-6q,-12p,-16q
Type B3 +1q -6,-13q

Associated Conditions

Approximately 30% of patients have their thymomas discovered because they have an associated autoimmune disorder. These disorders include:[4]

Type Diseases
Neuromuscular Diseases Myasthenia gravis, neuromyotonia, rippling muscle disease, polymyositis/dermatomyositis, encephalitis (limbic, cortical and brain stem), intestinal pseudoobstruction
Haematologic Autoimmune Diseases Anemia: pure red cell aplasia, pernicious anemia, hemolytic anemia, aplastic anemia. Other isolated cytopenia: eosinophils,basophils, neutrophils, immunodeficiencies: hypogammaglobulinaemia +/- T-cell deficiencies (Good syndrome)
Dermatologies Diseases Pemphigus (foliaceus or paraneoplastic), lichen planus, alopecia areata
Endocrine Disorders Addison disease, graves disease, Cushing's disease
Renal and Hepatic Diseases Glomerulonephritis, autoimmune hepatitis
Systemic Autoimmune Diseases SLE, Sjögren's syndrome, systemic sclerosis, graft-versus-host disease

Gross Pathology

On gross pathology, a well circumscribed mass that is locally invasive is a characteristic finding of thymoma.

Microscopic Pathology

On microscopic histopathological analysis, round cells, with ample vacuolated cytoplasms, and fat droplets are characteristic findings of thymoma.

Video

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References

  1. C. Buckley, D. Douek, J. Newsom-Davis, A. Vincent & N. Willcox (2001). "Mature, long-lived CD4+ and CD8+ T cells are generated by the thymoma in myasthenia gravis". Annals of neurology. 50 (1): 64–72. PMID 11456312. Unknown parameter |month= ignored (help)
  2. J. V. Souadjian, P. Enriquez, M. N. Silverstein & J. M. Pepin (1974). "The spectrum of diseases associated with thymoma. Coincidence or syndrome?". Archives of internal medicine. 134 (2): 374–379. PMID 4602050. Unknown parameter |month= ignored (help)
  3. "http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf" (PDF). Retrieved 26 February 2014. External link in |title= (help)
  4. "http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf" (PDF). External link in |title= (help)

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