|
|
| (76 intermediate revisions by 2 users not shown) |
| Line 1: |
Line 1: |
| __NOTOC__ | | __NOTOC__ |
| {{SI}} | | {{Sarcomatoid carcinoma of the lung}} |
| {{CMG}} {{AE}} {{Trusha}} {{MV}} | | {{CMG}}; {{AE}} {{Trusha}}, {{MV}} |
| | | |
| {{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 |
| | | |
| ==Overview== | | ==[[Sarcomatoid carcinoma of the lung overview|Overview]]== |
| '''Sarcomatoid carcinomas''' are a group of poorly differentiated non-small cell lung carcinomas that contain a component of sarcoma or sarcoma-like differentiation such as spindle and/or giant cell. Sarcomatoid cancer of lung is a rare type of malignancy and it comprises of only 0.1% - 0.4% cases of all non-small cell lung cancers. The pathogenesis of sarcomatoid carcinoma of the lung is characterized by a rare epithelial origin, and morphologic features suggestive of a malignant mesenchymal tumor. The [[EGFR]] gene and [[Ras oncogene|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, exposure to high levels of air pollution, asbestos, radiation therapy to the chest, radon gas, occupational exposure to chemical carcinogens, and previous lung diseases. If left untreated, the majority of patients with sarcomatoid carcinoma of the lung may develop distant [[metastasis]] via lymphogenic route. Common complications of sarcomatoid carcinoma of the lung include respiratory failure, pneumonia, and distant metastasis. Prognosis is generally poor, even worse than other non-small cell carcinoma of lung. The 5-year survival rate for patients with sarcomatoid cancer of lung is 28.7%.
| |
|
| |
|
| ==Historical Perspective== | | ==[[Sarcomatoid carcinoma of the lung historical perspective|Historical Perspective]]== |
| *In 1981, World Health Organization (WHO) classification on lung cancers, mentioned pleomorphic carcinomas showing a prevalence of spindle or giant cells as morphologic variants of squamous cell carcinomas and large-cell carcinomas, respectively, and defined carcinosarcomas as biphasic tumors consisting of an intimate admixture of carcinoma and sarcoma.<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>
| |
| *
| |
|
| |
|
| ==Classification== | | ==[[Sarcomatoid carcinoma of the lung classification|Classification]]== |
| *Sarcomatoid carcinoma of the lung may be classified according to WHO classification into 5 subtypes:
| |
| :*Pleomorphic carcinoma
| |
| :*Spindle cell carcinoma
| |
| :*Giant cell carcinoma
| |
| :*Carcinosarcoma
| |
| :*Pulmonary blastoma
| |
|
| |
|
| ==Pathophysiology== | | ==[[Sarcomatoid carcinoma of the lung pathophysiology|Pathophysiology]]== |
| *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>
| |
| *Sarcomatoid carcinoma of the lung arises from epithelial cells but has morphologic features suggestive of a malignant mesenchymal tumor.
| |
| *The molecular pathogenesis of sarcomatoid carcinoma of the lung is characterized by the overexpression of the EGFR protein.
| |
| *The EGFR and K-ras gene mutations have been associated with the development of sarcomatoid carcinoma of the lung.
| |
| *On gross pathology, a large-sized mass lesion, with upper lobe and peripheral location are characteristic findings of sarcomatoid carcinoma of the lung.
| |
| *On microscopic histopathological analysis, sarcomatous components are characteristic findings of sarcomatoid carcinoma of the lung.
| |
|
| |
|
| ==Causes== | | ==[[Sarcomatoid carcinoma of the lung causes|Causes]]== |
| * 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>
| |
|
| |
| ==Differentiating sarcomatoid carcinoma of the lung from other Diseases==
| |
| *Sarcomatoid carcinoma of the lung must be differentiated from other diseases that cause cough, weight loss, hemoptysis, and dyspnea among adults, such as:
| |
| :*[[Tuberculosis]]
| |
| :*[[Adenocarcinoma of the lung]]
| |
| :*Pulmonary fungal disease
| |
| :*[[Lung abscess]]
| |
|
| |
| ==Epidemiology and Demographics==
| |
| ===Prevalence===
| |
| * 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>
| |
| *Sarcomatoid carcinoma comprises of 0.3% to 1.3% of total lung malignancies.<ref>{{cite book |last1=Travis WD |last2=Brambilla E |last3=Müller-Hermelink K |last4=Harris C |last5=Kleihues C |last6=Sobin P |title=World Health Organization Classification of Tumours; Pathology and genetics of tumors of the lung, pleura, thymus, and heart |publisher=IARC Press |orig-year=2004 |pages=53-58|chapterurl=https://www.iarc.fr/wp-content/uploads/2018/07/BB10.pdf |isbn=ISBN 92 832 2418 3}}</ref>
| |
| *The overall prevalence of sarcomatoid carcinoma of the lung is 0.1% to 0.4% of all non-small cell lung malignancies.<ref name="BrambillaTravis2001">{{cite journal|last1=Brambilla|first1=E.|last2=Travis|first2=W.D.|last3=Colby|first3=T.V.|last4=Corrin|first4=B.|last5=Shimosato|first5=Y.|title=The new World Health Organization classification of lung tumours|journal=European Respiratory Journal|volume=18|issue=6|year=2001|pages=1059–1068|issn=0903-1936|doi=10.1183/09031936.01.00275301}}</ref>
| |
| | |
| ===Age===
| |
| * Sarcomatoid carcinoma of the lung is more commonly diagnosed among patients aged 65 to 75 years old.<ref name="OuzianeBoutayeb2014">{{cite journal|last1=Ouziane|first1=Imane|last2=Boutayeb|first2=Saber|last3=Mrabti|first3=Hind|last4=Lalya|first4=Issam|last5=Rimani|first5=Mouna|last6=Errihani|first6=Hassan|title=Sarcomatoid carcinoma of the lung: A model of resistance of chemotherapy|journal=North American Journal of Medical Sciences|volume=6|issue=7|year=2014|pages=342|issn=1947-2714|doi=10.4103/1947-2714.136920}}</ref>
| |
| * Sarcomatoid carcinoma of the lung is more commonly observed among elderly patients and adults.
| |
| * Biphasic blastoma subtype is an exception and an average age of 35 years old patients being more commonly diagnosed
| |
| | |
| ===Gender===
| |
| *Males are more commonly affected with sarcomatoid carcinoma of the lung than females.<ref name="PetrovVlassov2003">{{cite journal|last1=Petrov|first1=D.B|last2=Vlassov|first2=V.I|last3=Kalaydjiev|first3=G.T|last4=Plochev|first4=M.A|last5=Obretenov|first5=E.D|last6=Stanoev|first6=V.I|last7=Danon|first7=S.E|title=Primary pulmonary sarcomas and carcinosarcomas – postoperative results and comparative survival analysis|journal=European Journal of Cardio-Thoracic Surgery|volume=23|issue=4|year=2003|pages=461–466|issn=10107940|doi=10.1016/S1010-7940(03)00024-1}}</ref><ref name="RoeselTerjung2016">{{cite journal|last1=Roesel|first1=Christian|last2=Terjung|first2=Sarah|last3=Weinreich|first3=Gerhard|last4=Hager|first4=Thomas|last5=Chalvatzoulis|first5=Eleftherios|last6=Metzenmacher|first6=Martin|last7=Welter|first7=Stefan|title=Sarcomatoid carcinoma of the lung: a rare histological subtype of non-small cell lung cancer with a poor prognosis even at earlier tumour stages|journal=Interactive CardioVascular and Thoracic Surgery|year=2016|pages=ivw392|issn=1569-9293|doi=10.1093/icvts/ivw392}}</ref>
| |
| *the male to female ratio is 4:1.<ref name="OuzianeBoutayeb2014">{{cite journal|last1=Ouziane|first1=Imane|last2=Boutayeb|first2=Saber|last3=Mrabti|first3=Hind|last4=Lalya|first4=Issam|last5=Rimani|first5=Mouna|last6=Errihani|first6=Hassan|title=Sarcomatoid carcinoma of the lung: A model of resistance of chemotherapy|journal=North American Journal of Medical Sciences|volume=6|issue=7|year=2014|pages=342|issn=1947-2714|doi=10.4103/1947-2714.136920}}</ref>
| |
| *Biphasic blastoma subtype is an exception, that affects men and women equally.
| |
|
| |
|
| ===Race=== | | ==[[Sarcomatoid carcinoma of the lung differential diagnosis|Differentiating Sarcomatoid carcinoma of the lung from other Diseases]]== |
| *There is no racial predilection for sarcomatoid carcinoma of the lung.
| |
|
| |
|
| ===Region=== | | ==[[Sarcomatoid carcinoma of the lung epidemiology and demographics|Epidemiology and Demographics]]== |
| Geographical location has no effect on epidemiology of sarcomatoid carcinoma of the lung.
| |
| | |
| ==Risk Factors==
| |
| *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>
| |
| | |
| == Natural History, Complications and Prognosis==
| |
| *The majority of patients with sarcomatoid carcinoma of the lung remain are initially asymptomatic.
| |
| *Early clinical features include chronic [[cough]], [[hemoptysis]], [[dyspnea]], and [[fatigue]].<ref>{{cite book |last1=Travis WD |last2=Brambilla E |last3=Müller-Hermelink K |last4=Harris C |last5=Kleihues C |last6=Sobin P |title=World Health Organization Classification of Tumours; Pathology and genetics of tumors of the lung, pleura, thymus, and heart |publisher=IARC Press |orig-year=2004 |pages=53-58|chapterurl=https://www.iarc.fr/wp-content/uploads/2018/07/BB10.pdf |isbn=ISBN 92 832 2418 3}}</ref>
| |
| *The majority of patients with central sarcomatoid carcinoma of the lung may develop distant metastasis to esophagus, jejunum, rectum and kidney.
| |
| *Sarcomatoid carcinoma of lung located in the periphery of lungs tends be diagnosed later in the advanced stages with the large size and metastasis to the pleura and chest wall.
| |
| *Common complications of sarcomatoid carcinoma of the lung include [[respiratory failure]], [[pneumonia]], and distant metastasis.
| |
| *If left untreated Sarcomatoid carcinoma progresses to death.
| |
| *Prognosis is generally poor.<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>
| |
| *According to a study, the 3-year and 5-year survival rates for patients with sarcomatoid cancer of lung are 35.8 and 28.7%.<ref name="RoeselTerjung2016">{{cite journal|last1=Roesel|first1=Christian|last2=Terjung|first2=Sarah|last3=Weinreich|first3=Gerhard|last4=Hager|first4=Thomas|last5=Chalvatzoulis|first5=Eleftherios|last6=Metzenmacher|first6=Martin|last7=Welter|first7=Stefan|title=Sarcomatoid carcinoma of the lung: a rare histological subtype of non-small cell lung cancer with a poor prognosis even at earlier tumour stages|journal=Interactive CardioVascular and Thoracic Surgery|year=2016|pages=ivw392|issn=1569-9293|doi=10.1093/icvts/ivw392}}</ref>
| |
| | |
| == Diagnosis ==
| |
| ===Diagnostic Criteria===
| |
| *The diagnosis of sarcomatoid carcinoma of the lung is made by lung biopsy.
| |
| *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>
| |
| :*Pleomorphic carcinoma subtype
| |
| :*Spindle cell carcinoma subtype
| |
| :*Giant cell carcinoma subtype
| |
| :*Carcinosarcoma subtype
| |
| | |
| === Symptoms ===
| |
| *Common symptoms of sarcomatoid carcinoma of the lung, may 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>
| |
| :*[[Shortness of breath]]
| |
| :*[[Fatigue]]
| |
| :*[[Chest pain]]
| |
| :*[[Fatigue]]
| |
| | |
| === Physical Examination ===
| |
| *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>
| |
| *Physical examination may be remarkable for:
| |
| '''Inspection'''
| |
| *[[Hoarseness]]
| |
| *Rapid rate of breathing
| |
| '''Auscultation'''
| |
| *Present [[pleural friction rub]]
| |
| *Present [[egophony]]
| |
| *Crackling or bubbling noises
| |
| *Present whispered pectoriloquy
| |
| *Decreased/absent [[breath sounds]]
| |
| '''Percussion'''
| |
| *Hyporesonance
| |
| *Dull percussion
| |
| *[[Tactile fremitus]]
| |
| *Reduced chest expansion
| |
| | |
| === Laboratory Findings ===
| |
| *There are no specific laboratory findings associated with sarcomatoid carcinoma of the lung.
| |
|
| |
| ===Imaging Findings===
| |
| *CT scan of chest is the imaging investigation of choice for sarcomatoid carcinoma of the lung.
| |
| *On CT, sarcomatoid carcinoma of the lung is characterized by the following findings:<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>
| |
| **Sarcomatoid carcinoma of the lung are typically peripherally located
| |
| **Usually measure approximately 4 cm in diameter
| |
| **Perihilar and mediastinal involvement
| |
| **Ground glass opacity
| |
| **Rapid growth
| |
| * PET-CT<ref name="SilvestriGould2007">{{cite journal|last1=Silvestri|first1=Gerard A.|last2=Gould|first2=Michael K.|last3=Margolis|first3=Mitchell L.|last4=Tanoue|first4=Lynn T.|last5=McCrory|first5=Douglas|last6=Toloza|first6=Eric|last7=Detterbeck|first7=Frank|title=Noninvasive Staging of Non-small Cell Lung Cancer|journal=Chest|volume=132|issue=3|year=2007|pages=178S–201S|issn=00123692|doi=10.1378/chest.07-1360}}</ref>
| |
|
| |
|
| *
| | ==[[Sarcomatoid carcinoma of the lung risk factors|Risk Factors]]== |
|
| |
|
| === Other Diagnostic Studies === | | ==[[Sarcomatoid carcinoma of the lung screening|Screening]]== |
| *Sarcomatoid carcinoma of the lung may also be diagnosed using [[bronchoscopy]], [[mediastinoscopy]], and t[[Needle aspiration biopsy|ransthoracic percutaneous fine needle aspiration]].
| |
|
| |
|
| == Treatment == | | ==[[Sarcomatoid carcinoma of the lung natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| === Medical Therapy ===
| |
| *The mainstay of therapy of sarcomatoid carcinoma of the lung is supportive care.
| |
| *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>
| |
|
| |
|
| === 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 perihilar lymph node dissection) in conjunction with pathological evaluation is the most common approach to the treatment of sarcomatoid carcinoma of the lung.
| |
|
| |
|
| === Prevention === | | ==Treatment== |
| *Effective measures for the primary prevention of sarcomatoid carcinoma of the lung include CT screening.<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>
| | [[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)
| |
| *Once diagnosed and successfully treated, patients with sarcomatoid carcinoma of the lung are followed-up every year. Follow-up testing includes CT screening.
| |
|
| |
|
| ==References== | | ==Case Studies== |
| {{Reflist|2}}
| | [[Sarcomatoid carcinoma of the lung medical case study one|Case #1]] |
| | | |
| [[Category: Oncology]] | | [[Category: Oncology]] |