Thymoma pathophysiology: Difference between revisions
No edit summary |
No edit summary |
||
Line 4: | Line 4: | ||
==Overview== | ==Overview== | ||
On [[gross pathology]], a well-circumscribed mass, that is locally [[Invasive (medical)|invasive]], is a characteristic finding of thymoma. On [[microscopic]] [[Histopathology|histopathological]] [[analysis]], round [[Cell (biology)|cells]] with ample vacuolated [[Cytoplasm|cytoplasms]] and [[fat]] droplets are characteristic findings of thymoma. | |||
==Pathophysiology== | ==Pathophysiology== | ||
Line 11: | Line 10: | ||
=== Physiology === | === Physiology === | ||
* Thymus is the site of maturation of T cells. | *[[Thymus]] is the site of maturation of [[T cell|T cells]]. | ||
* This makes thymus the primary center | * This makes [[thymus]] the primary center responsible for [[adaptive immunity]]. | ||
=== Pathogenesis === | === Pathogenesis === | ||
* The exact pathogenesis of the primary tumor development is not | * The exact [[pathogenesis]] of the [[primary tumor]] development is not completely understood. | ||
* Primary tumors of thymus are relatively rare. | *[[Primary tumor|Primary tumors]] of [[thymus]] are relatively rare. | ||
* Thymoma is the most common type of primary tumor of thymus. | * Thymoma is the most common type of [[primary tumor]] of [[thymus]]. | ||
* Thymoma is histologically comprised of abnormally conditioned T cells. | * Thymoma is [[Histology|histologically]] comprised of abnormally conditioned [[T cell|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 | * The mingling of these abnormal [[T cell|T cells]] into the [[Circulatory system|circulation]] is believed to be involved in the [[causality]] of the associated [[Autoimmunity|autoimmune disorders]].<ref>{{Cite journal | ||
| author = [[C. Buckley]], [[D. Douek]], [[J. Newsom-Davis]], [[A. Vincent]] & [[N. Willcox]] | | 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 | | title = Mature, long-lived CD4+ and CD8+ T cells are generated by the thymoma in myasthenia gravis | ||
Line 44: | Line 43: | ||
==Genetics== | ==Genetics== | ||
'''Genetic | '''Genetic Alterations Reported for the Different WHO Histological Thymoma sub-types'''<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> | ||
{| border="1" cellpadding="2" | {| border="1" cellpadding="2" | ||
|- | |- | ||
Line 51: | Line 50: | ||
| width="200pt" |'''Chromosomal Losses''' | | width="200pt" |'''Chromosomal Losses''' | ||
|- | |- | ||
!Type A | |||
| | |||
* None | |||
| | |||
* -6p | |||
|- | |- | ||
!Type AB | |||
| | |||
* None | |||
| | |||
* -5q21 - 22 | |||
* -6q | |||
* -12p | |||
* -16q | |||
|- | |- | ||
!Type B3 | |||
| | |||
* +1q | |||
| | |||
* -6 | |||
* -13q | |||
|} | |} | ||
==Associated Conditions== | ==Associated Conditions== | ||
Approximately 30% of patients have their thymomas discovered because | Approximately 30% of the [[Patient|patients]] have their thymomas discovered because of a symptomatic 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}} cellpadding="4" cellspacing="0" style="border:#c9c9c9 1px solid; margin: 1em 1em 1em 0; border-collapse: collapse;" | {| {{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 | ![[Neuromuscular disease|Neuromuscular diseases]] | ||
|[[Myasthenia gravis]] | | | ||
* [[Myasthenia gravis]] | |||
* [[Neuromyotonia]] | |||
* Rippling [[muscle]] [[disease]] | |||
* [[Polymyositis]]/[[dermatomyositis]] | |||
* [[Encephalitis]] ([[Limbic encephalitis|limbic]], cortical and [[brain stem]]) | |||
* [[Intestinal pseudoobstruction]] | |||
|- | |- | ||
| | ![[Hematology|Hematologic]] [[autoimmune diseases]] | ||
|[[Anemia]]: [[ | | | ||
* [[Anemia]]: [[Pure red cell aplasia]], [[pernicious anemia]], [[hemolytic anemia]], [[aplastic anemia]]. | |||
* Other isolated [[cytopenia]]: [[Eosinophils]], [[basophils]], [[neutrophils]] | |||
* [[Immunodeficiency|Immunodeficiencies]]: [[Hypogammaglobulinaemia]], [[Good syndrome]] | |||
|- | |- | ||
| | ![[Dermatologic disorders]] | ||
| | |||
* [[Pemphigus]] (foliaceus or [[paraneoplastic]]) | |||
* [[Lichen planus]] | |||
* [[Alopecia areata]] | |||
|- | |- | ||
![[Endocrine disorders]] | |||
|[[Addison disease]] | | | ||
* [[Addison disease]] | |||
* [[Graves disease]] | |||
* [[Cushing's disease]] | |||
|- | |- | ||
| Renal and | ![[Kidney|Renal]] and [[Liver|hepatic]] [[Disease|diseases]] | ||
|[[Glomerulonephritis]] | | | ||
* [[Glomerulonephritis]] | |||
* [[Autoimmune hepatitis]] | |||
|- | |- | ||
![[Systemic]] [[autoimmune diseases]] | |||
|[[SLE]] | | | ||
* [[SLE]] | |||
* [[Sjögren's syndrome]] | |||
* [[Systemic sclerosis]] | |||
* [[Graft-versus-host disease]] | |||
|} | |} | ||
==Gross Pathology== | ==Gross Pathology== | ||
On gross pathology, a well circumscribed mass that is locally invasive is a characteristic finding of thymoma. | On [[gross pathology]], a well circumscribed mass, that is locally [[Invasive (medical)|invasive]], is a characteristic finding of thymoma. | ||
==Microscopic Pathology== | ==Microscopic Pathology== | ||
On microscopic histopathological analysis, round cells, with ample vacuolated cytoplasms, and fat droplets are characteristic findings of thymoma. | On [[microscopic]] [[histopathological]] [[analysis]], round [[Cell (biology)|cells]], with ample vacuolated [[Cytoplasm|cytoplasms]], and [[fat]] droplets are characteristic findings of thymoma. | ||
{| | {| | ||
==Video== | ==Video== |
Revision as of 01:15, 16 August 2019
Thymoma Microchapters |
Diagnosis |
---|
Case Studies |
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
Physiology
- Thymus is the site of maturation of T cells.
- This makes thymus the primary center responsible for adaptive immunity.
Pathogenesis
- The exact pathogenesis of the primary tumor development is not completely 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 causality of the associated autoimmune disorders.[1][2]
Genetics
Genetic Alterations Reported for the Different WHO Histological Thymoma sub-types[3]
WHO Type | Chromosomal Gains | Chromosomal Losses |
Type A |
|
|
---|---|---|
Type AB |
|
|
Type B3 |
|
|
Associated Conditions
Approximately 30% of the patients have their thymomas discovered because of a symptomatic associated autoimmune disorder. These disorders include:[4]
Type | Diseases |
Neuromuscular diseases |
|
---|---|
Hematologic autoimmune diseases | |
Dermatologic disorders |
|
Endocrine disorders | |
Renal and hepatic diseases | |
Systemic autoimmune diseases |
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
|title=
(help) - ↑ "http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf" (PDF). External link in
|title=
(help)