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| __NOTOC__ | | __NOTOC__ |
| {{SI}} | | {{Sarcomatoid carcinoma of the lung}} |
| {{CMG}} {{AE}} {{MV}} | | {{CMG}}; {{AE}} {{Trusha}}, {{MV}} |
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| {{SK}} Primary sarcoma of the lung; Lung sarcoma; Sarcomatoid tumor of the lung | | {{SK}} Primary sarcoma of the lung; Lung sarcoma; Sarcomatoid tumor of the lung; Pulmonary sarcomatoid carcinoma |
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| ==Overview== | | ==[[Sarcomatoid carcinoma of the lung overview|Overview]]== |
| Sarcomatoid carcinoma of the lung is a rare histologic subtype of non-small cell lung cancer. Sarcomatoid carcinoma of the lung is a "sarcoma-like" carcinoma neoplasm. The overall prevalence of sarcomatoid carcinoma of the lung is 0.1% to 0.4% of all lung malignancies.<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 mesenchymal tumor.<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 EGFR gene and K-ras mutations have been associated with the development of sarcomatoid carcinoma of the lung. The prevalence of sarcomatoid carcinoma of the lung is approximately 0.4 cases per 100,000 individuals worldwide. Common risk factors in the development of sarcomatoid carcinoma of the lung are smoking, family history of lung cancer, high levels of air pollution, radiation therapy to the chest, radon gas, asbestos, occupational exposure to chemical carcinogens, and previous lung diseases.<ref>Lung cancer. Canadian Cancer Society 2015. http://www.cancer.ca/en/cancer-information/cancer-type/lung/risks/?region=ab#Outdoor_air_pollution Accessed February 3, 2016</ref> If left untreated, the majority of patients with sarcomatoid carcinoma of the lung may progress to develop lymphatic invasion and distant metastasis. Common complications of sarcomatoid carcinoma of the lung include respiratory failure, pneumonia, and distant metastasis. Prognosis is generally poor, and the average 5-survival rate of patients with sarcomatoid carcinoma of the lung is approximately 6%.<ref name="pmid22739072">{{cite journal |vauthors=Yendamuri S, Caty L, Pine M, Adem S, Bogner P, Miller A, Demmy TL, Groman A, Reid M |title=Outcomes of sarcomatoid carcinoma of the lung: a Surveillance, Epidemiology, and End Results Database analysis |journal=Surgery |volume=152 |issue=3 |pages=397–402 |year=2012 |pmid=22739072 |doi=10.1016/j.surg.2012.05.007 |url=}}</ref>
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| ==Historical Perspective== | | ==[[Sarcomatoid carcinoma of the lung historical perspective|Historical Perspective]]== |
| *In 1987, EGFR gene mutations were first identified in the pathogenesis of sarcomatoid carcinoma of the lung.<ref name="ref">Timeline of lung cancer. http://cancerprogress.net/timeline/lung-cancer Accessed on February 17, 2016</ref>
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| ==Classification== | | ==[[Sarcomatoid carcinoma of the lung classification|Classification]]== |
| *Sarcomatoid carcinoma of the lung may be classified according to WHO classification into 5 subtypes:<ref name="wiki">Sarcomatoid carcinoma of the lung. Wikipedia. https://en.wikipedia.org/wiki/Sarcomatoid_carcinoma_of_the_lung Accessed on March 31, 2016</ref>
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| :*Pleomorphic carcinoma
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| :*Spindle cell carcinoma
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| :*Giant cell carcinoma
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| :*Carcinosarcoma
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| :*Pulmonary blastoma
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| ==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 mesenchymal tumor.<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>
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| *Sarcomatoid carcinoma of the lung arises from epithelial cells but has morphologic features suggestive of a malignant mesenchymal tumor.
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| *The EGFR gene and K-ras mutations have been associated with the development of sarcomatoid carcinoma of the lung.
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| *On gross pathology, a large sized mass lesion, with upper lobe and peripheral location are characteristic findings of sarcomatoid carcinoma of the lung.
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| *On microscopic histopathological analysis, sarcomatous components are characteristic findings of sarcomatoid carcinoma of the lung.
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| ==Causes== | | ==[[Sarcomatoid carcinoma of the lung pathophysiology|Pathophysiology]]== |
| * Sarcomatoid carcinoma of the lung may be caused by a mutation in the EGFR and K-ras genes.<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>
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| ==Differentiating sarcomatoid carcinoma of the lung from other Diseases==
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| *Sarcomatoid carcinoma of the lung must be differentiated from other diseases that cause cough, weight loss, hemoptysis, and dyspnea among adults, such as:
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| :*Tuberculosis
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| :*Adenocarcinoma of the lung
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| :*Pulmonary fungal disease
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| :*Lung abscess
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| ==Epidemiology and Demographics==
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| * The prevalence of sarcomatoid carcinoma of the lung is approximately 0.4 cases per 100,000 individuals worldwide.<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>
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| *The overall prevalence of sarcomatoid carcinoma of the lung is 0.1% to 0.4% of all lung malignancies.<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>
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| ===Age===
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| * Sarcomatoid carcinoma of the lung is more commonly observed among patients aged 50 to 75 years old.
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| * Sarcomatoid carcinoma of the lung is more commonly observed among elderly patients and adults
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| ===Gender===
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| *Males are more commonly affected with sarcomatoid carcinoma of the lung than females.
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| ===Race=== | | ==[[Sarcomatoid carcinoma of the lung causes|Causes]]== |
| *There is no racial predilection for sarcomatoid carcinoma of the lung.
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| ==Risk Factors==
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| *Common risk factors in the development of sarcomatoid carcinoma of the lung are smoking, family history of lung cancer, high levels of air pollution, radiation therapy to the chest, radon gas, asbestos, occupational exposure to chemical carcinogens, and previous lung diseases.<ref>Lung cancer. Canadian Cancer Society 2015. http://www.cancer.ca/en/cancer-information/cancer-type/lung/risks/?region=ab#Outdoor_air_pollution Accessed February 3, 2016</ref>
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| == Natural History, Complications and Prognosis== | | ==[[Sarcomatoid carcinoma of the lung differential diagnosis|Differentiating Sarcomatoid carcinoma of the lung from other Diseases]]== |
| *The majority of patients with sarcomatoid carcinoma of the lung remain are initially asymptomatic.
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| *Early clinical features include chronic coughing, chest pain, and fatigue.
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| *If left untreated, the majority of patients with sarcomatoid carcinoma of the lung may progress to develop lymphatic invasion and distant metastasis.<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 |year=2009 |pmid=19830024 |pmc=2740247 |doi=10.4076/1757-1626-2-7900 |url=}}</ref>
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| *Common complications of sarcomatoid carcinoma of the lung include respiratory failure, pneumonia, and distant metastasis.
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| *Prognosis is generally poor, and the average 5-survival rate of patients with sarcomatoid carcinoma of the lung is approximately 6%.<ref name="pmid22739072">{{cite journal |vauthors=Yendamuri S, Caty L, Pine M, Adem S, Bogner P, Miller A, Demmy TL, Groman A, Reid M |title=Outcomes of sarcomatoid carcinoma of the lung: a Surveillance, Epidemiology, and End Results Database analysis |journal=Surgery |volume=152 |issue=3 |pages=397–402 |year=2012 |pmid=22739072 |doi=10.1016/j.surg.2012.05.007 |url=}}</ref><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 |year=2009 |pmid=19830024 |pmc=2740247 |doi=10.4076/1757-1626-2-7900 |url=}}</ref>
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| == Diagnosis == | | ==[[Sarcomatoid carcinoma of the lung epidemiology and demographics|Epidemiology and Demographics]]== |
| ===Diagnostic Criteria===
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| *The diagnosis of sarcomatoid carcinoma of the lung is made by lung biopsy.
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| *Biopsy findings associated with sarcomatoid carcinoma of the lung will depend on the subtype of tumor histology, common findings include:<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 |year=2009 |pmid=19830024 |pmc=2740247 |doi=10.4076/1757-1626-2-7900 |url=}}</ref>
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| :*Pleomorphic carcinoma subtype
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| :*Spindle cell carcinoma subtype
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| :*Giant cell carcinoma subtype
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| :*Carcinosarcoma subtype
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| === Symptoms === | | ==[[Sarcomatoid carcinoma of the lung risk factors|Risk Factors]]== |
| *Common symptoms of sarcomatoid carcinoma of the lung may include the following:<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 |year=2009 |pmid=19830024 |pmc=2740247 |doi=10.4076/1757-1626-2-7900 |url=}}</ref>
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| :*Shortness of breath
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| :*Fatigue
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| :*Chest pain
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| :*Fatigue
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| === Physical Examination ===
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| *Patients with sarcomatoid carcinoma of the lung usually appear older in age in relation to their chronological age.<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 |year=2009 |pmid=19830024 |pmc=2740247 |doi=10.4076/1757-1626-2-7900 |url=}}</ref>
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| *Physical examination may be remarkable for:
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| '''Inspection'''
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| *Performed in the anterior chest/posterior chest
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| *Hoarseness
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| *Rapid rate of breathing
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| '''Auscultation'''
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| *Present [[pleural friction rub]]
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| *Present [[egophony]]
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| *Crackling or bubbling noises
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| *Present whispered pectoriloquy
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| *Decreased/absent [[breath sounds]]
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| '''Percussion'''
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| *Hyporesonance
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| *Dull percussion
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| *[[Tactile fremitus]]
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| *Reduced chest expansion
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| === Laboratory Findings ===
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| *There are no specific laboratory findings associated with sarcomatoid carcinoma of the lung.
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| ===Imaging Findings===
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| *CT scan is the imaging modality of choice for sarcomatoid carcinoma of the lung.
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| *On CT, sarcomatoid carcinoma of the lung is characterized by the following findings:
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| :*Sarcomatoid carcinoma of the lung are typically peripherally located
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| :*Usually measure <4 cm in diameter, very few show cavitation
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| :*Perihilar and mediastinal involvement
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| :*Ground glass opacity
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| :*Rapid growth
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| === Other Diagnostic Studies === | | ==[[Sarcomatoid carcinoma of the lung screening|Screening]]== |
| *Sarcomatoid carcinoma of the lung may also be diagnosed using bronchoscopy, mediastinoscopy, transthoracic percutaneous fine needle aspiration.
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| == Treatment == | | ==[[Sarcomatoid carcinoma of the lung natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| === Medical Therapy ===
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| *The mainstay of therapy of sarcomatoid carcinoma of the lung is supportive care.
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| *Common medical treatment for sarcomatoid carcinoma of the lung is the platinum-based chemotherapy.<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 |year=2009 |pmid=19830024 |pmc=2740247 |doi=10.4076/1757-1626-2-7900 |url=}}</ref>
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| === Surgery=== | | ==Diagnosis== |
| *Surgery is the mainstay of therapy for sarcomatoid carcinoma of the lung.
| | [[Sarcomatoid carcinoma of the lung diagnostic study of choice|Diagnostic study of choice]] | [[Sarcomatoid carcinoma of the lung history and symptoms|History and Symptoms]] | [[Sarcomatoid carcinoma of the lung physical examination|Physical Examination]] | [[Sarcomatoid carcinoma of the lung laboratory findings|Laboratory Findings]] | [[Sarcomatoid carcinoma of the lung electrocardiogram|Electrocardiogram]] | [[Sarcomatoid carcinoma of the lung x ray|X-Ray Findings]] | [[Sarcomatoid carcinoma of the lung echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Sarcomatoid carcinoma of the lung CT scan|CT-Scan Findings]] | [[Sarcomatoid carcinoma of the lung MRI|MRI Findings]] | [[Sarcomatoid carcinoma of the lung other imaging findings|Other Imaging Findings]] | [[Sarcomatoid carcinoma of the lung other diagnostic studies|Other Diagnostic Studies]] |
| *Lobectomy along with regional lymph nodes (peribronchial and perihiliar lymph node dissection) in conjunction with pathological evaluation is the most common approach to the treatment of sarcomatoid carcinoma of the lung.
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| === Prevention === | | ==Treatment== |
| *Effective measures for the primary prevention of sarcomatoid carcinoma of the lung include CT screening.
| | [[Sarcomatoid carcinoma of the lung medical therapy|Medical Therapy]] | [[Sarcomatoid carcinoma of the lung surgery|Surgery]] | [[Sarcomatoid carcinoma of the lung primary prevention|Primary Prevention]] | [[Sarcomatoid carcinoma of the lung secondary prevention|Secondary Prevention]] | [[Sarcomatoid carcinoma of the lung cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Sarcomatoid carcinoma of the lung future or investigational therapies|Future or Investigational Therapies]] |
| *According to the U.S. Preventive Services Task Force (USPSTF), screening for lung cancer by low-dose computed tomography is recommended every year among smokers who are between 55 to 80 years old and who have history of smoke 30 pack-years or more and either continue to smoke or have quit within the past 15 years (grade B recommendation)
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| *Once diagnosed and successfully treated, patients with sarcomatoid carcinoma of the lung are followed-up every year. Follow-up testing includes CT screening.
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| ==References== | | ==Case Studies== |
| {{Reflist|2}}
| | [[Sarcomatoid carcinoma of the lung medical case study one|Case #1]] |
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| [[Category: Oncology]] | | [[Category: Oncology]] |
| | [[Category:Up-To-Date]] |
| | [[Category:Oncology]] |
| | [[Category:Medicine]] |
| | [[Category:Pulmonology]] |
| | [[Category:Surgery]] |