Thymic carcinoma pathophysiology: Difference between revisions

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Latest revision as of 21:51, 25 July 2019

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]

Overview

The thymic carcinoma is a tumor of the thymic epithelium, but it is associated with a lack of immature lymphocytes and often presents with a more invasive or metastatic disease.^[1] The pathophysiology of thymic carcinoma depends on the histological subtype. On microscopic histopathological analysis, thymic carcinoma is divided into squamous cell carcinoma, basaloid carcinoma, mucoepidermoid carcinoma, lymphoepithelioma-like carcinoma, sarcomatoid carcinoma (carcinosarcoma), clear cell carcinoma, papillary adenocarcinoma, carcinoma with t(15;19) translocation, neuroendrocrine carcinomas, undifferentiated carcinoma, and combined thymic epithelial tumors.^[2] Thymic carcinoma has not been reported in association with myasthenia gravis, in contrast with thymoma^[3]

Pathophysiology

The thymic carcinoma is a tumor of the thymic epithelium, but it is associated with a lack of immature lymphocytes and often presents with a more invasive or metastatic disease.^[1]
In contrast to thymoma, thymic carcinoma has not been reported in association with myasthenia gravis.

Genetics

Development of thymic carcinoma is the result of multiple genetic mutations.^[3]
Genetic mutations involved in the pathogenesis of thymic carcinoma include:

Gain of chromosome 1q, 17q, and 18
Deletion of chromosome 3p, 6, 13q, 16q, and 17p

Associated Conditions

Paraneoplasic syndromes has been reported among patients with thymic cancer (neuroendocrine subtype), however the syndrome is not as common as with thymoma patients.

Microscopic Pathology

On microscopic histopathological analysis, characteristic findings of thymic carcinoma are:

Cytologically malignant - variable morphology
+/-Squamous differentiation

Immunohistochemistry

CD5 +ve (90% of cases)
CD117 +ve (87% of cases)
CD7 +ve
TTF-1 -ve

References

↑ ^1.0 ^1.1 Thomas CR, Wright CD, Loehrer PJ (1999). "Thymoma: state of the art". Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology. 17 (7): 2280–9. PMID 10561285. Retrieved 2012-01-18. Unknown parameter |month= ignored (help)
↑ Suster, S.; Rosai, J. (1991). "Thymic carcinoma. A clinicopathologic study of 60 cases". Cancer. 67 (4): 1025–32. PMID 1991250. Unknown parameter |month= ignored (help)
↑ ^3.0 ^3.1 Harris, C.C (2004), Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart (PDF), Lyon: IARC, retrieved 01/03/2016 Check date values in: |accessdate= (help)

[pmid10561285-1] 1.0 ^1.1 Thomas CR, Wright CD, Loehrer PJ (1999). "Thymoma: state of the art". Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology. 17 (7): 2280–9. PMID 10561285. Retrieved 2012-01-18. Unknown parameter |month= ignored (help)

[Suster-1991-2] Suster, S.; Rosai, J. (1991). "Thymic carcinoma. A clinicopathologic study of 60 cases". Cancer. 67 (4): 1025–32. PMID 1991250. Unknown parameter |month= ignored (help)

[dhingramc-3] 3.0 ^3.1 Harris, C.C (2004), Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart (PDF), Lyon: IARC, retrieved 01/03/2016 Check date values in: |accessdate= (help)

[1]

[2]

[3]

@@ Line 1: / Line 1: @@
 __NOTOC__
 {{Thymic carcinoma}}
-{{CMG}}; {{AE}} {{AL}}{{PSD}}
+{{CMG}}; {{AE}} {{Marjan}}
 ==Overview==
+The thymic carcinoma is a tumor of the thymic [[epithelium]], but it is associated with a lack of immature [[lymphocytes]] and often presents with a more invasive or [[metastasis|metastatic disease]].<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> The pathophysiology of thymic carcinoma depends on the histological subtype. On microscopic histopathological analysis, thymic carcinoma is divided into squamous cell carcinoma, basaloid carcinoma, mucoepidermoid carcinoma, lymphoepithelioma-like carcinoma, sarcomatoid carcinoma (carcinosarcoma),  clear cell carcinoma, papillary adenocarcinoma, carcinoma with t(15;19) translocation, neuroendrocrine carcinomas, undifferentiated carcinoma, and combined thymic epithelial tumors.<ref name="Suster-1991">{{Cite journal  | last1 = Suster | first1 = S. | last2 = Rosai | first2 = J. | title = Thymic carcinoma. A clinicopathologic study of 60 cases. | journal = Cancer | volume = 67 | issue = 4 | pages = 1025-32 | month = Feb | year = 1991 | doi =  | PMID = 1991250 }}</ref> Thymic carcinoma has not been reported in association with [[myasthenia gravis]], in contrast with [[thymoma]]<ref name=dhingramc>{{Citation |last=Travis |first=W.D ||last=Brambilla |first=E |last=Müller-Hermelink |first=H.K |last=Harris |first=C.C |year=2004 |title=Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart |publisher=IARC |publication-place=Lyon |page= |url=http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf |accessdate=01/03/2016 }}</ref>
 ==Pathophysiology==
-The thymic carcinoma is a tumor of the thymic [[epithelium]], but it is associated with lack of immature [[lymphocytes]] and often presents with more invasive or [[metastasis|metastatic disease]].<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>
+*The thymic carcinoma is a tumor of the thymic [[epithelium]], but it is associated with a lack of immature [[lymphocytes]] and often presents with a more invasive or [[metastasis|metastatic disease]].<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>
+* In contrast to [[thymoma]], thymic carcinoma has not been reported in association with [[myasthenia gravis]].
 ==Genetics==
+* Development of thymic carcinoma is the result of multiple genetic mutations.<ref name=dhingramc>{{Citation |last=Travis |first=W.D ||last=Brambilla |first=E |last=Müller-Hermelink |first=H.K |last=Harris |first=C.C |year=2004 |title=Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart |publisher=IARC |publication-place=Lyon |page= |url=http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf |accessdate=01/03/2016 }}</ref>
+* Genetic mutations involved in the pathogenesis of thymic carcinoma include:
+:* Gain of chromosome 1q, 17q, and 18
+:* Deletion of chromosome 3p, 6, 13q, 16q, and 17p
-*Thymic carcinoma, specially the [[squamous cell]] subtype, is associated with genetic alterations.
+==Associated Conditions==
-*It has been reported chromosomal gains of 1q, 17q, and 18 and chromosomal losses of 3p, 6, 13q, 16q, and 17p to a relation with this subtype. <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>
+*Paraneoplasic syndromes has been reported among patients with thymic cancer (neuroendocrine subtype), however the syndrome is not as common as with [[thymoma]] patients.
-{|
-| [[File:Kariotype thymic carcinoma.png|x300px|thumb|G-banded metaphase spread shows a complex karyotype, including der(16)t(1;16)(q12;q12.1).]]
-|}
-==Patologic Classification==
-Thymic carcinomas are categorized in low and high grade histological subtypes, which includes squamous cell carcinoma, basaloid carcinoma, mucoepidermoid carcinoma, lymphoepithelioma-like carcinoma, sarcomatoid carcinoma (carcinosarcoma),  clear cell carcinoma, papillary adenocarcinoma, carcinoma with t(15;19) translocation, neuroendrocrine carcinomas, undifferentiated carcinoma, and combined thymic epithelial tumors.<ref name="Suster-1991">{{Cite journal  | last1 = Suster | first1 = S. | last2 = Rosai | first2 = J. | title = Thymic carcinoma. A clinicopathologic study of 60 cases. | journal = Cancer | volume = 67 | issue = 4 | pages = 1025-32 | month = Feb | year = 1991 | doi =  | PMID = 1991250 }}</ref>
-* '''Squamous cell carcinoma:'''  This subtype of thymic carcinoma is the most common and exhibits atypia with a clear-cut aspect of keratinization  with keratin pearls as seen in squamous cell carcinomas.  Squamous cell carcinoma lack of capsule and presents with necrosis and hemorrhage.
-* '''Basaloid carcinoma:''' This subtype consists of solid lobules of tumor cells with marginal palisading, without keratinization and a basophilic pattern due to an elevated nucleocytoplasmic ratio.
-* '''Mucoepidermoid carcinoma:''' The characteristic features for this rare subtype is the presence of squamous and mucus producing cells, with moderate atypia.  It has a mucinous macroscopic appearance.
-* '''Lymphoepithelioma-like carcinoma:''' The morphology of this subtype resembles the nasopharingeal lymphoepitelioma, with syncytial growth of undifferentiated malignant cells.
-* '''Sarcomatoid carcinoma (carcinosarcoma):''' It is also known as spindle cell thymic carcinoma, is an infiltrative neoplasm with large areas of coagulative necrosis and without a capsule.  It is an uncommon  tumor that affects patients between 40-80 years old.
-* '''Clear cell carcinoma:''' Consist of cells with minimal nuclear atypia with a characteristic lucent cytoplasm and a lobulated architecture without sinusoidal vasculature (in contrast with the renal clear cell carcinoma)
-* '''Papillary adenocarcinoma:''' It has a tubulopapillary pattern growth with cuboidal cells and Psammoma bodies may be present.   Type A thymoma may be the origin of this subtype of carcinoma due an expression of malignant transformation.
-* '''Carcinoma with t(15;19) translocation:''' It is an aggressive tumor with a translocation t(15;19)(q13:p13.1 )that has the characteristic presence of undifferentiated cells with high mitotic activity and squamous morphology.
-* '''Neuroendocrine Carcinomas:''' The neuroendocrine thymic carcinomas are classified in 4 categories: typical, atypical, small cell and large cell carcinomas.  The typical and atypical are categorized as well differentiated neuroendocrine carcinomas, and the small cell and large cell carcinomas are categorized as poorly differentiated.
-Classification of Neuroendocrine Carcinomas<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>
-{|class="wikitable" border="1"
-|-
-! colspan=2 | Well Differentiated || colspan=2 | Poorly Differentiated
-|-
-| Typical Carcinoid || Atypical Carcinoid  ||  Small Cell || Large Cell
-|-
-| No necrosis; <BR> <2 mitoses per 2 mm2 (10 HPF) || Necrosis present and/or <BR> 2-10 mitoses per 2 mm2 (10 HPF) ||  Small cell cytology || Non-small cell NEC with >10 <BR> mitoses per 2 mm2 (10 HPF)
-|-
-| colspan=2 | '''Morphological Variants''' <BR>  Spindle cell type <BR> Pigmented type <BR> With amyloid (extrathyroidal medullary carcinoma) <BR> Oncocytic/oxyphilic type <BR> Mucinous <BR> Angiomatoid type <BR> Combinations of the above variants || valign=top |'''Variants''' <BR> SCNEC combined with Non-NECs || valign=top | —
-|-
-|}
-HPF: High power field,  SCNEC: Small cell neuroendocrine carcinoma, NEC: Neuroendocrine carcinoma
 ==Microscopic Pathology==
@@ Line 59: / Line 22: @@
 *Cytologically malignant - variable morphology
 *+/-Squamous differentiation
+==Immunohistochemistry==
-==Associated Conditions==
+*[[CD5]] +ve (90% of cases)
-*Thymic carcinoma has not been reported in association with [[myasthenia gravis]], in contrast with [[thymoma]].
+*[[CD117]] +ve (87% of cases)
-*Paraneoplasic syndromes had been reported in the neuroendocrine subtype, but is not as common as in [[thymomas]].
+*[[CD7]] +ve
+*TTF-1 -ve
 ==References==
@@ Line 68: / Line 32: @@
 [[Category:Disease]]
+ [[Category:Up-To-Date]]
+[[Category:Oncology]]
+[[Category:Medicine]]
+[[Category:Hematology]]
+[[Category:Immunology]]
+[[Category:Surgery]]