Thymoma pathophysiology: Difference between revisions
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==Pathophysiology== | ==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.<ref>{{Cite journal | |||
| author = [[C. Buckley]], [[D. Douek]], [[J. Newsom-Davis]], [[A. Vincent]] & [[N. Willcox]] | |||
| title = Mature, long-lived CD4+ and CD8+ T cells are generated by the thymoma in myasthenia gravis | |||
| journal = [[Annals of neurology]] | |||
| volume = 50 | |||
| issue = 1 | |||
| pages = 64–72 | |||
| year = 2001 | |||
| month = July | |||
| pmid = 11456312 | |||
}}</ref><ref>{{Cite journal | |||
| author = [[J. V. Souadjian]], [[P. Enriquez]], [[M. N. Silverstein]] & [[J. M. Pepin]] | |||
| title = The spectrum of diseases associated with thymoma. Coincidence or syndrome? | |||
| journal = [[Archives of internal medicine]] | |||
| volume = 134 | |||
| issue = 2 | |||
| pages = 374–379 | |||
| year = 1974 | |||
| month = August | |||
| pmid = 4602050 | |||
}}</ref> | |||
===Microscopic Pathology=== | ===Microscopic Pathology=== | ||
Revision as of 00:36, 16 August 2019
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Risk calculators and risk factors for Thymoma pathophysiology |
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]
Microscopic Pathology
On microscopic histopathological analysis, round cells, with ample vacuolated cytoplasms, and fat droplets are characteristic findings of thymoma.
Gross Pathology
On gross pathology, a well circumscribed mass that is locally invasive is a characteristic finding of thymoma.
Genetic Features
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 |
Video
{{#ev:youtube|wfyixp6JxQM}}
Associated Disorders
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 |
References
- ↑ 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) - ↑ 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) - ↑ "http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf" (PDF). Retrieved 26 February 2014. External link in
|title=(help) - ↑ "http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf" (PDF). External link in
|title=(help)