Thymoma pathophysiology: Difference between revisions
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==Overview== | ==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. | * 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== | ==Pathophysiology== | ||
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{| border="1" cellpadding="2" | {| border="1" cellpadding="2" | ||
|- | |- | ||
|width="200pt"|'''WHO Type''' | | width="200pt" |'''WHO Type''' | ||
|width="200pt"|'''Chromosomal Gains''' | | width="200pt" |'''Chromosomal Gains''' | ||
|width="200pt"|'''Chromosomal Losses''' | | width="200pt" |'''Chromosomal Losses''' | ||
|- | |- | ||
|Type A || none || -6p | |Type A || none || -6p | ||
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==Associated Disorders== | ==Associated Disorders== | ||
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> | ||
{| {{table}} | {| {{table}} cellpadding="4" cellspacing="0" style="border:#c9c9c9 1px solid; margin: 1em 1em 1em 0; border-collapse: collapse;" | ||
| style="width: 25%;"| '''Type''' | | style="width: 25%;" | '''Type''' | ||
| style="width: 75%;"| '''Diseases''' | | style="width: 75%;" | '''Diseases''' | ||
|- | |- | ||
| Neuromuscular Diseases | | Neuromuscular Diseases | ||
|[[Myasthenia gravis]], [[neuromyotonia]], rippling muscle disease, [[polymyositis]]/[[dermatomyositis]], [[encephalitis]] (limbic, cortical and brain stem), [[intestinal pseudoobstruction]] | |||
|- | |- | ||
| Haematologic Autoimmune Diseases | | 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 | | Dermatologies Diseases | ||
|[[Pemphigus]] (foliaceus or paraneoplastic), [[lichen planus]], [[alopecia areata]] | |||
|- | |- | ||
| Endocrine Disorders | | Endocrine Disorders | ||
|[[Addison disease]], [[graves disease]], [[Cushing's disease]] | |||
|- | |- | ||
| Renal and Hepatic Diseases | | Renal and Hepatic Diseases | ||
|[[Glomerulonephritis]], [[autoimmune hepatitis]] | |||
|- | |- | ||
| Systemic Autoimmune Diseases | | Systemic Autoimmune Diseases | ||
|[[SLE]], [[Sjögren's syndrome]], [[systemic sclerosis]], [[graft-versus-host disease]] | |||
|} | |} | ||
Revision as of 20:22, 20 November 2018
Thymoma Microchapters |
Diagnosis |
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Thymoma pathophysiology On the Web |
American Roentgen Ray Society Images of Thymoma pathophysiology |
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
Origin
- It has been believed that thymic epithelium is derived from both ectodermal and endodermal stem cells.
- Recent evidence indicates that epithelial populations originate from a common progenitor of endodermal origin.
- Occurrence of more differentiated “committed stem cells” with medullary, cortical or other phenotypes is possible.[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
- ↑ Blackburn, CC.; Manley, NR.; Palmer, DB.; Boyd, RL.; Anderson, G.; Ritter, MA. (2002). "One for all and all for one: thymic epithelial stem cells and regeneration". Trends Immunol. 23 (8): 391–5. PMID 12133801. Unknown parameter
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ignored (help) - ↑ Gill, J.; Malin, M.; Holländer, GA.; Boyd, R. (2002). "Generation of a complete thymic microenvironment by MTS24(+) thymic epithelial cells". Nat Immunol. 3 (7): 635–42. doi:10.1038/ni812. PMID 12068292. Unknown parameter
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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)