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{{Thymoma}}
{{Thymoma}}
{{CMG}}
{{CMG}}; {{AE}} {{AM}} {{AAM}}{{Sab}}
 
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==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==
Thymoma originates from the [[epithelium|epithelial]] cell population in the thymus. Many subtypes are recognized, some of which have a better- or worse-than-general prognosis.<ref name="pmid10561285">{{cite journal |author=Thomas CR, Wright CD, Loehrer PJ |title=Thymoma: state of the art |journal=[[Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology]] |volume=17 |issue=7 |pages=2280–9 |year=1999 |month=July |pmid=10561285 |doi= |url=http://www.jco.org/cgi/pmidlookup?view=long&pmid=10561285 |accessdate=2012-01-18}}</ref>


===Associated Disorders===
=== Physiology ===
A third of the patients who have a thymoma detected because they have an associated [[autoimmune disorder]]. The most common condition in this group is [[myasthenia gravis]] (of which 25-50% are associated with a thymoma); patients with myasthenia are routinely screened for thymoma. Other associated autoimmune conditions are [[pure red cell aplasia]] and Good's syndrome (thymoma with [[combined immunodeficiency]] and hypoimmunoglobulinemia G). Rare associations that have been reported are: [[acute pericarditis]], [[Addison's disease]], [[agranulocytosis]], [[alopecia areata]], [[ulcerative colitis]], [[Cushing's disease]], [[hemolytic anemia]], limbic encephalopathy, [[myocarditis]], [[nephrotic syndrome]], [[panhypopituitarism]], [[pernicious anemia]], [[polymyositis]], [[rheumatoid arthritis]], [[sarcoidosis]], [[scleroderma]], sensorimotor radiculopathy, ''[[stiff person syndrome]]'', [[systemic lupus erythematosus]] and [[thyroiditis]].<ref name="pmid10561285">{{cite journal |author=Thomas CR, Wright CD, Loehrer PJ |title=Thymoma: state of the art |journal=[[Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology]] |volume=17 |issue=7 |pages=2280–9 |year=1999 |month=July |pmid=10561285 |doi= |url=http://www.jco.org/cgi/pmidlookup?view=long&pmid=10561285 |accessdate=2012-01-18}}</ref>
 
*[[Thymus]] is the site of maturation of [[T cell|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 tumor|Primary tumors]] of [[thymus]] are relatively rare.
* Thymoma is the most common type of [[primary tumor]] of [[thymus]].
* Thymoma is [[Histology|histologically]] comprised of abnormally conditioned [[T cell|T cells]].
* 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]]
| 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>
 
==Genetics==
'''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"
|-
| width="200pt" |'''WHO Type'''
| width="200pt" |'''Chromosomal Gains'''
| width="200pt" |'''Chromosomal Losses'''
|-
!Type A
|
* None
|
* -6p
|-
!Type AB
|
* None
|
* -5q21 - 22
* -6q
* -12p
* -16q
|-
!Type B3
|
* +1q
|
* -6
* -13q
|}
==Associated Conditions==
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;"
| style="width: 25%;" | '''Type'''
| style="width: 75%;" | '''Diseases'''
|-
![[Neuromuscular disease|Neuromuscular diseases]]
|
* [[Myasthenia gravis]]
* [[Neuromyotonia]]
* Rippling [[muscle]] [[disease]]
* [[Polymyositis]]/[[dermatomyositis]]
* [[Encephalitis]] ([[Limbic encephalitis|limbic]], cortical and [[brain stem]])
* [[Intestinal pseudoobstruction]]
|-
![[Hematology|Hematologic]] [[autoimmune diseases]]
|
* [[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]]
* [[Graves disease]]
* [[Cushing's disease]]
|-
![[Kidney|Renal]] and [[Liver|hepatic]] [[Disease|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 (medical)|invasive]], is a characteristic finding of thymoma.
 
==Microscopic Pathology==
On [[microscopic]] [[histopathological]] [[analysis]], round [[Cell (biology)|cells]], with ample vacuolated [[Cytoplasm|cytoplasms]], and [[fat]] droplets are characteristic findings of thymoma.
{|
==Video==
{{#ev:youtube|wfyixp6JxQM}}


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
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Latest revision as of 01:16, 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]Sabawoon Mirwais, M.B.B.S, M.D.[4]

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

Pathogenesis

Genetics

Genetic Alterations Reported for the Different WHO Histological Thymoma sub-types[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 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

  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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