Difference between revisions of "Thymoma pathophysiology"

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__NOTOC__
 
__NOTOC__
 
{{Thymoma}}
 
{{Thymoma}}
{{CMG}}; {{AE}} {{AM}} {{AAM}}
+
{{CMG}}; {{AE}} {{AM}} {{AAM}}{{Sab}}
  
 
==Overview==
 
==Overview==
* 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. On [[microscopic]] [[Histopathology|histopathological]] [[analysis]], round [[Cell (biology)|cells]] with ample vacuolated [[Cytoplasm|cytoplasms]] and [[fat]] droplets are characteristic findings of thymoma.
* On microscopic histopathological analysis, round cells, with ample vacuolated 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 of adaptive immunity.
+
* This makes [[thymus]] the primary center responsible for [[adaptive immunity]].
  
 
=== Pathogenesis ===
 
=== Pathogenesis ===
  
* The exact pathogenesis of the primary tumor development is not well understood.
+
* 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 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 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"
 
|-
 
|-
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| width="200pt" |'''Chromosomal Losses'''
 
| width="200pt" |'''Chromosomal Losses'''
 
|-
 
|-
|Type A || none || -6p
+
!Type A  
 +
|
 +
* None
 +
|
 +
* -6p
 
|-
 
|-
|Type AB || none || -5q21-22,-6q,-12p,-16q
+
!Type AB  
 +
|
 +
* None
 +
|
 +
* -5q21 - 22
 +
* -6q
 +
* -12p
 +
* -16q
 
|-
 
|-
|Type B3 || +1q || -6,-13q
+
!Type B3  
 +
|
 +
* +1q
 +
|
 +
* -6
 +
* -13q
 
|}
 
|}
 
==Associated Conditions==
 
==Associated Conditions==
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 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 Diseases
+
![[Neuromuscular disease|Neuromuscular diseases]]
|[[Myasthenia gravis]], [[neuromyotonia]], rippling muscle disease, [[polymyositis]]/[[dermatomyositis]], [[encephalitis]] (limbic, cortical and brain stem), [[intestinal pseudoobstruction]]
+
|
 +
* [[Myasthenia gravis]]  
 +
* [[Neuromyotonia]]  
 +
* Rippling [[muscle]] [[disease]]
 +
* [[Polymyositis]]/[[dermatomyositis]]  
 +
* [[Encephalitis]] ([[Limbic encephalitis|limbic]], cortical and [[brain stem]])  
 +
* [[Intestinal pseudoobstruction]]
 
|-
 
|-
| Haematologic Autoimmune Diseases
+
![[Hematology|Hematologic]] [[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]])
+
|
 +
* [[Anemia]]: [[Pure red cell aplasia]], [[pernicious anemia]], [[hemolytic anemia]], [[aplastic anemia]].  
 +
* Other isolated [[cytopenia]]: [[Eosinophils]], [[basophils]], [[neutrophils]]  
 +
* [[Immunodeficiency|Immunodeficiencies]]: [[Hypogammaglobulinaemia]], [[Good syndrome]]
 
|-
 
|-
| Dermatologies Diseases
+
![[Dermatologic disorders]]
|[[Pemphigus]] (foliaceus or paraneoplastic), [[lichen planus]], [[alopecia areata]]
+
|
 +
* [[Pemphigus]] (foliaceus or [[paraneoplastic]])  
 +
* [[Lichen planus]]  
 +
* [[Alopecia areata]]
 
|-
 
|-
| Endocrine Disorders
+
![[Endocrine disorders]]
|[[Addison disease]], [[graves disease]], [[Cushing's disease]]
+
|
 +
* [[Addison disease]]  
 +
* [[Graves disease]]
 +
* [[Cushing's disease]]
 
|-
 
|-
| Renal and Hepatic Diseases
+
![[Kidney|Renal]] and [[Liver|hepatic]] [[Disease|diseases]]
|[[Glomerulonephritis]], [[autoimmune hepatitis]]
+
|
 +
* [[Glomerulonephritis]]  
 +
* [[Autoimmune hepatitis]]
 
|-
 
|-
| Systemic Autoimmune Diseases
+
![[Systemic]] [[autoimmune diseases]]
|[[SLE]], [[Sjögren's syndrome]], [[systemic sclerosis]], [[graft-versus-host disease]]
+
|
 +
* [[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==

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

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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