Sarcomatoid carcinoma of the lung pathophysiology: Difference between revisions

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{{CMG}}; {{AE}} {{Trusha}}
{{CMG}}; {{AE}} {{Trusha}}
==Overview==
==Overview==
Sarcomatoid carcinomas are rare, aggressive, malignant cancer and can occur in different organs such as the [[Thyroid cancer|thyroid gland]], [[Osteosarcoma|bone]], [[Skin cancer|skin]], [[Breast cancer|breast]], [[Pancreatic cancer|pancreas]], [[Hepatocellular carcinoma|liver]], [[Transitional cell carcinoma|urinary tract]], and [[Sarcomatoid carcinoma of the lung|lung]]. Sarcomatoid carcinoma of the lung is a group of poorly differentiated [[Non small cell lung cancer|non-small cell lung carcinomas]]. Sarcomatoid carcinoma is defined as a biphasic tumor with morphological characteristics of both, [[Carcinoma|carcinomatous]] and [[Sarcoma|sarcomatous]] components. The exact pathogenesis of sarcomatoid carcinoma of the lung is not fully understood, but the [[EGFR]] and [[K ras|K-ras]] gene mutations have been associated with the development of sarcomatoid carcinoma of the lung.
Sarcomatoid carcinomas are rare, aggressive, malignant cancer and can occur in different organs such as the [[Thyroid cancer|thyroid gland]], [[Osteosarcoma|bone]], [[Skin cancer|skin]], [[Breast cancer|breast]], [[Pancreatic cancer|pancreas]], [[Hepatocellular carcinoma|liver]], [[Transitional cell carcinoma|urinary tract]], and [[Sarcomatoid carcinoma of the lung|lung]]. Sarcomatoid carcinoma of the lung is a group of poorly differentiated [[Non small cell lung cancer|non-small cell lung carcinomas]]. Sarcomatoid carcinoma is defined as a biphasic tumor with morphological characteristics of both, [[Carcinoma|carcinomatous]] and [[Sarcoma|sarcomatous]] components. The exact pathogenesis of sarcomatoid carcinoma of the lung is not fully understood, but the [[EGFR]] and [[KRAS|K-ras]] gene mutations have been associated with the development of sarcomatoid carcinoma of the lung.


==Pathophysiology==
==Pathophysiology==
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*The pathogenesis of sarcomatoid carcinoma of the lung is characterized by a rare epithelial origin and morphologic features suggestive of a malignant [[Mesenchyme|mesenchymal]] tumor.<ref name="pmid6978190">{{cite journal |vauthors=Sobin LH |title=The international histological classification of tumours |journal=Bull. World Health Organ. |volume=59 |issue=6 |pages=813–9 |date=1981 |pmid=6978190 |pmc=2396133 |doi= |url=}}</ref><ref name="pmid11829087">{{cite journal |vauthors=Brambilla E, Travis WD, Colby TV, Corrin B, Shimosato Y |title=The new World Health Organization classification of lung tumours |journal=Eur. Respir. J. |volume=18 |issue=6 |pages=1059–68 |date=December 2001 |pmid=11829087 |doi= |url=}}</ref><ref name="pmid20073605">{{cite journal |vauthors=Franks TJ, Galvin JR |title=Sarcomatoid carcinoma of the lung: histologic criteria and common lesions in the differential diagnosis |journal=Arch. Pathol. Lab. Med. |volume=134 |issue=1 |pages=49–54 |year=2010 |pmid=20073605 |doi=10.1043/2008-0547-RAR.1 |url=}}</ref>
*The pathogenesis of sarcomatoid carcinoma of the lung is characterized by a rare epithelial origin and morphologic features suggestive of a malignant [[Mesenchyme|mesenchymal]] tumor.<ref name="pmid6978190">{{cite journal |vauthors=Sobin LH |title=The international histological classification of tumours |journal=Bull. World Health Organ. |volume=59 |issue=6 |pages=813–9 |date=1981 |pmid=6978190 |pmc=2396133 |doi= |url=}}</ref><ref name="pmid11829087">{{cite journal |vauthors=Brambilla E, Travis WD, Colby TV, Corrin B, Shimosato Y |title=The new World Health Organization classification of lung tumours |journal=Eur. Respir. J. |volume=18 |issue=6 |pages=1059–68 |date=December 2001 |pmid=11829087 |doi= |url=}}</ref><ref name="pmid20073605">{{cite journal |vauthors=Franks TJ, Galvin JR |title=Sarcomatoid carcinoma of the lung: histologic criteria and common lesions in the differential diagnosis |journal=Arch. Pathol. Lab. Med. |volume=134 |issue=1 |pages=49–54 |year=2010 |pmid=20073605 |doi=10.1043/2008-0547-RAR.1 |url=}}</ref>
*Four major hypotheses may explain sarcomatoid neoplasms:<ref name="pmid8772780">{{cite journal |vauthors=Thompson L, Chang B, Barsky SH |title=Monoclonal origins of malignant mixed tumors (carcinosarcomas). Evidence for a divergent histogenesis |journal=Am. J. Surg. Pathol. |volume=20 |issue=3 |pages=277–85 |date=March 1996 |pmid=8772780 |doi= |url=}}</ref><ref name="pmid200736052">{{cite journal |vauthors=Franks TJ, Galvin JR |title=Sarcomatoid carcinoma of the lung: histologic criteria and common lesions in the differential diagnosis |journal=Arch. Pathol. Lab. Med. |volume=134 |issue=1 |pages=49–54 |date=January 2010 |pmid=20073605 |doi=10.1043/2008-0547-RAR.1 |url=}}</ref>
*Four major hypotheses may explain sarcomatoid neoplasms:<ref name="pmid8772780">{{cite journal |vauthors=Thompson L, Chang B, Barsky SH |title=Monoclonal origins of malignant mixed tumors (carcinosarcomas). Evidence for a divergent histogenesis |journal=Am. J. Surg. Pathol. |volume=20 |issue=3 |pages=277–85 |date=March 1996 |pmid=8772780 |doi= |url=}}</ref><ref name="pmid200736052">{{cite journal |vauthors=Franks TJ, Galvin JR |title=Sarcomatoid carcinoma of the lung: histologic criteria and common lesions in the differential diagnosis |journal=Arch. Pathol. Lab. Med. |volume=134 |issue=1 |pages=49–54 |date=January 2010 |pmid=20073605 |doi=10.1043/2008-0547-RAR.1 |url=}}</ref>
**'''The embryonic rest hypothesis:''' This theory suggests that sarcomatoid tumors are the result of misplaced “mini-organs” that has epithelial and stromal component.
**'''The embryonic rest hypothesis:''' This theory suggests that sarcomatoid tumors are the result of misplaced “mini-organs” that has [[Epithelium|epithelial]] and [[Stromal cell|stromal]] component.
**'''The collision hypothesis:''' This theory implies separate but concomitant [[Proliferation|malignant proliferation]] of [[epithelium]] and [[mesenchyme]].
**'''The collision hypothesis:''' This theory implies separate but concomitant [[Proliferation|malignant proliferation]] of [[epithelium]] and [[mesenchyme]].
**'''The stromal induction/metaplasia hypothesis:''' This theory suggests that sarcomatous elements are an atypical response to the growth of a carcinoma.
**'''The stromal induction/metaplasia hypothesis:''' This theory suggests that [[Sarcoma|sarcomatous]] elements are an atypical response to the growth of a [[carcinoma]].
**'''Totipotential hypothesis:''' This hypothesis proposes an origin from a single totipotential stem cell that differentiates into epithelial and mesenchymal components.
**'''Totipotential hypothesis:''' This hypothesis proposes an origin from a single totipotential stem cell that differentiates into [[Epithelium|epithelial]] and [[Mesenchyme|mesenchymal]] components.


==Genetics==
==Genetics==
*The development of sarcomatoid carcinoma is the result of multiple genetic mutations such as:<ref name="BillahStewart2011">{{cite journal|last1=Billah|first1=Shahreen|last2=Stewart|first2=John|last3=Staerkel|first3=Gregg|last4=Chen|first4=Su|last5=Gong|first5=Yun|last6=Guo|first6=Ming|title=EGFR and KRAS mutations in lung carcinoma|journal=Cancer Cytopathology|volume=119|issue=2|year=2011|pages=111–117|issn=1934662X|doi=10.1002/cncy.20151}}</ref>
*The development of sarcomatoid carcinoma is the result of multiple genetic mutations such as:<ref name="BillahStewart2011">{{cite journal|last1=Billah|first1=Shahreen|last2=Stewart|first2=John|last3=Staerkel|first3=Gregg|last4=Chen|first4=Su|last5=Gong|first5=Yun|last6=Guo|first6=Ming|title=EGFR and KRAS mutations in lung carcinoma|journal=Cancer Cytopathology|volume=119|issue=2|year=2011|pages=111–117|issn=1934662X|doi=10.1002/cncy.20151}}</ref>
*[[EGFR]]
*[[EGFR]]
*[[K ras|K-ras]]
*[[KRAS|K-ras]]


==Associated Conditions==
==Associated Conditions==
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* Sarcomatoid carcinomas can arise centrally or peripherally.<ref name="pmid12604887">{{cite journal |vauthors=Rossi G, Cavazza A, Sturm N, Migaldi M, Facciolongo N, Longo L, Maiorana A, Brambilla E |title=Pulmonary carcinomas with pleomorphic, sarcomatoid, or sarcomatous elements: a clinicopathologic and immunohistochemical study of 75 cases |journal=Am. J. Surg. Pathol. |volume=27 |issue=3 |pages=311–24 |date=March 2003 |pmid=12604887 |doi= |url=}}</ref><ref name="pmid10584705">{{cite journal |vauthors=Koss MN, Hochholzer L, Frommelt RA |title=Carcinosarcomas of the lung: a clinicopathologic study of 66 patients |journal=Am. J. Surg. Pathol. |volume=23 |issue=12 |pages=1514–26 |date=December 1999 |pmid=10584705 |doi= |url=}}</ref>
* Sarcomatoid carcinomas can arise centrally or peripherally.<ref name="pmid12604887">{{cite journal |vauthors=Rossi G, Cavazza A, Sturm N, Migaldi M, Facciolongo N, Longo L, Maiorana A, Brambilla E |title=Pulmonary carcinomas with pleomorphic, sarcomatoid, or sarcomatous elements: a clinicopathologic and immunohistochemical study of 75 cases |journal=Am. J. Surg. Pathol. |volume=27 |issue=3 |pages=311–24 |date=March 2003 |pmid=12604887 |doi= |url=}}</ref><ref name="pmid10584705">{{cite journal |vauthors=Koss MN, Hochholzer L, Frommelt RA |title=Carcinosarcomas of the lung: a clinicopathologic study of 66 patients |journal=Am. J. Surg. Pathol. |volume=23 |issue=12 |pages=1514–26 |date=December 1999 |pmid=10584705 |doi= |url=}}</ref>
* Most commonly present as poorly circumscribed solitary peripheral masses.
* Most commonly present as poorly circumscribed solitary peripheral masses.
* Sarcomatoid carcinoma is commonly found in upper lobe of lungs.
* Sarcomatoid carcinoma is commonly found in upper lobe of the lungs.
* Tumors are large, ranging from 1 to 13 cm, with a median of 4.9 cm, and often invade the chest wall.
* Tumors are large, ranging from 1 to 13 cm, with a median of 4.9 cm, and often invade the chest wall.
* Tumor consistency is described as soft and fleshy or firm, hard, or rubbery.  
* Tumor consistency is described as soft and fleshy or firm, hard, or rubbery.  
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*On microscopic histopathologically sarcomatoid carcinoma is classified into 5 subtypes:<ref name="pmid19830024">{{cite journal |vauthors=Hountis P, Moraitis S, Dedeilias P, Ikonomidis P, Douzinas M |title=Sarcomatoid lung carcinomas: a case series |journal=Cases J |volume=2 |issue= |pages=7900 |date=June 2009 |pmid=19830024 |pmc=2740247 |doi=10.4076/1757-1626-2-7900 |url=}}</ref>
*On microscopic histopathologically sarcomatoid carcinoma is classified into 5 subtypes:<ref name="pmid19830024">{{cite journal |vauthors=Hountis P, Moraitis S, Dedeilias P, Ikonomidis P, Douzinas M |title=Sarcomatoid lung carcinomas: a case series |journal=Cases J |volume=2 |issue= |pages=7900 |date=June 2009 |pmid=19830024 |pmc=2740247 |doi=10.4076/1757-1626-2-7900 |url=}}</ref>
**'''Pleomorphic carcinoma'''
**'''Pleomorphic carcinoma'''
***Poorly differentiated, sarcomatoid carcinoma composed of malignant, spindle and giant cells.
***Poorly differentiated, sarcomatoid carcinoma composed of malignant spindle and giant cells.
***Spindle cell may vary from epithelioid to strikingly spindled and are arranged in haphazard fascicles or storiform pattern.  
***Spindle cell may vary from epithelioid to strikingly spindled and are arranged in haphazard fascicles or storiform pattern.  
***Giant cells are discohesive, uni/multinucleated, have moderate to abundant, dense, eosinophilic cytoplasm which may show emperipolesis by [[Granulocyte|polymorphonuclear leukocytes]] or [[Lymphocyte|lymphocytes]].
***Giant cells are discohesive, uni/multinucleated, have moderate to abundant, dense, [[eosinophilic]] cytoplasm which may show emperipolesis by [[Granulocyte|polymorphonuclear leukocytes]] or [[Lymphocyte|lymphocytes]].
***Giant cells are anaplastic, with many bizarre forms.
***Giant cells are [[Anaplasia|anaplastic]], with many bizarre forms.
***Tumor cells are embedded in a fibrous or myxoid stroma.
***Tumor cells are embedded in a fibrous or myxoid stroma.
** '''Spindle cell carcinoma'''  
** '''Spindle cell carcinoma'''  

Revision as of 19:55, 3 January 2019

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

Overview

Sarcomatoid carcinomas are rare, aggressive, malignant cancer and can occur in different organs such as the thyroid gland, bone, skin, breast, pancreas, liver, urinary tract, and lung. Sarcomatoid carcinoma of the lung is a group of poorly differentiated non-small cell lung carcinomas. Sarcomatoid carcinoma is defined as a biphasic tumor with morphological characteristics of both, carcinomatous and sarcomatous components. The exact pathogenesis of sarcomatoid carcinoma of the lung is not fully understood, but the EGFR and K-ras gene mutations have been associated with the development of sarcomatoid carcinoma of the lung.

Pathophysiology

Pathogenesis

  • The pathogenesis of sarcomatoid carcinoma of the lung is characterized by a rare epithelial origin and morphologic features suggestive of a malignant mesenchymal tumor.[1][2][3]
  • Four major hypotheses may explain sarcomatoid neoplasms:[4][5]
    • The embryonic rest hypothesis: This theory suggests that sarcomatoid tumors are the result of misplaced “mini-organs” that has epithelial and stromal component.
    • The collision hypothesis: This theory implies separate but concomitant malignant proliferation of epithelium and mesenchyme.
    • The stromal induction/metaplasia hypothesis: This theory suggests that sarcomatous elements are an atypical response to the growth of a carcinoma.
    • Totipotential hypothesis: This hypothesis proposes an origin from a single totipotential stem cell that differentiates into epithelial and mesenchymal components.

Genetics

  • The development of sarcomatoid carcinoma is the result of multiple genetic mutations such as:[6]
  • EGFR
  • K-ras

Associated Conditions

There are no conditions associated with sarcomatoid carcinoma of the lung.

Gross Pathology

  • Sarcomatoid carcinomas can arise centrally or peripherally.[7][8]
  • Most commonly present as poorly circumscribed solitary peripheral masses.
  • Sarcomatoid carcinoma is commonly found in upper lobe of the lungs.
  • Tumors are large, ranging from 1 to 13 cm, with a median of 4.9 cm, and often invade the chest wall.
  • Tumor consistency is described as soft and fleshy or firm, hard, or rubbery.
  • Cut surfaces vary from white-grey to yellow, frequently show hemorrhagic and necrotic foci, and occasionally demonstrate cavitation.

Microscopic Pathology

  • On microscopic histopathologically sarcomatoid carcinoma is classified into 5 subtypes:[9]
    • Pleomorphic carcinoma
      • Poorly differentiated, sarcomatoid carcinoma composed of malignant spindle and giant cells.
      • Spindle cell may vary from epithelioid to strikingly spindled and are arranged in haphazard fascicles or storiform pattern.
      • Giant cells are discohesive, uni/multinucleated, have moderate to abundant, dense, eosinophilic cytoplasm which may show emperipolesis by polymorphonuclear leukocytes or lymphocytes.
      • Giant cells are anaplastic, with many bizarre forms.
      • Tumor cells are embedded in a fibrous or myxoid stroma.
    • Spindle cell carcinoma
      • Sarcomatoid carcinoma composed only of spindle-shaped tumor cells.
    • Giant cell carcinoma
      • Sarcomatoid carcinoma composed only of anaplastic, giant tumor cells.
    • Carcinosarcoma
    • Pulmonary blastoma
      • A biphasic tumor composed of a primitive epithelial component with well-differentiated, fetal adenocarcinoma and a primitive mesenchymal stroma that may contain rhabdomyosarcoma, osteosarcoma, or chondrosarcoma.

References

  1. ↑ Sobin LH (1981). "The international histological classification of tumours". Bull. World Health Organ. 59 (6): 813–9. PMC 2396133. PMID 6978190.
  2. ↑ Brambilla E, Travis WD, Colby TV, Corrin B, Shimosato Y (December 2001). "The new World Health Organization classification of lung tumours". Eur. Respir. J. 18 (6): 1059–68. PMID 11829087.
  3. ↑ Franks TJ, Galvin JR (2010). "Sarcomatoid carcinoma of the lung: histologic criteria and common lesions in the differential diagnosis". Arch. Pathol. Lab. Med. 134 (1): 49–54. doi:10.1043/2008-0547-RAR.1. PMID 20073605.
  4. ↑ Thompson L, Chang B, Barsky SH (March 1996). "Monoclonal origins of malignant mixed tumors (carcinosarcomas). Evidence for a divergent histogenesis". Am. J. Surg. Pathol. 20 (3): 277–85. PMID 8772780.
  5. ↑ Franks TJ, Galvin JR (January 2010). "Sarcomatoid carcinoma of the lung: histologic criteria and common lesions in the differential diagnosis". Arch. Pathol. Lab. Med. 134 (1): 49–54. doi:10.1043/2008-0547-RAR.1. PMID 20073605.
  6. ↑ Billah, Shahreen; Stewart, John; Staerkel, Gregg; Chen, Su; Gong, Yun; Guo, Ming (2011). "EGFR and KRAS mutations in lung carcinoma". Cancer Cytopathology. 119 (2): 111–117. doi:10.1002/cncy.20151. ISSN 1934-662X.
  7. ↑ Rossi G, Cavazza A, Sturm N, Migaldi M, Facciolongo N, Longo L, Maiorana A, Brambilla E (March 2003). "Pulmonary carcinomas with pleomorphic, sarcomatoid, or sarcomatous elements: a clinicopathologic and immunohistochemical study of 75 cases". Am. J. Surg. Pathol. 27 (3): 311–24. PMID 12604887.
  8. ↑ Koss MN, Hochholzer L, Frommelt RA (December 1999). "Carcinosarcomas of the lung: a clinicopathologic study of 66 patients". Am. J. Surg. Pathol. 23 (12): 1514–26. PMID 10584705.
  9. ↑ Hountis P, Moraitis S, Dedeilias P, Ikonomidis P, Douzinas M (June 2009). "Sarcomatoid lung carcinomas: a case series". Cases J. 2: 7900. doi:10.4076/1757-1626-2-7900. PMC 2740247. PMID 19830024.

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