Thymoma pathophysiology: Difference between revisions
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== | ==Genetics== | ||
'''Genetic alterations reported for the different WHO histological thymomasubtypes'''<ref>{{Cite web | last = | first = | title = http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf | url = http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf | publisher = | date = | accessdate = 26 February 2014 }}</ref> | '''Genetic alterations reported for the different WHO histological thymomasubtypes'''<ref>{{Cite web | last = | first = | title = http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf | url = http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf | publisher = | date = | accessdate = 26 February 2014 }}</ref> | ||
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|Type B3 || +1q || -6,-13q | |Type B3 || +1q || -6,-13q | ||
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==Associated Conditions== | |||
==Associated | |||
Approximately 30% of patients have their thymomas discovered because they have an associated autoimmune disorder. These disorders include:<ref>{{Cite web | last = | first = | title = http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf | url = http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf | publisher = | date = | accessdate = }}</ref> | Approximately 30% of patients have their thymomas discovered because they have an associated autoimmune disorder. These disorders include:<ref>{{Cite web | last = | first = | title = http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf | url = http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf | publisher = | date = | accessdate = }}</ref> | ||
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|[[SLE]], [[Sjögren's syndrome]], [[systemic sclerosis]], [[graft-versus-host disease]] | |[[SLE]], [[Sjögren's syndrome]], [[systemic sclerosis]], [[graft-versus-host disease]] | ||
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==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. | |||
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==Video== | |||
{{#ev:youtube|wfyixp6JxQM}} | |||
==References== | ==References== |
Revision as of 00:46, 16 August 2019
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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
{{#ev:youtube|wfyixp6JxQM}}
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
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(help) - ↑ "http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf" (PDF). External link in
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(help)