Sarcomatoid carcinoma of the lung

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Trusha Tank, M.D.[2] Maria Fernanda Villarreal, M.D. [3]

Synonyms and Keywords: Primary sarcoma of the lung; Lung sarcoma; Sarcomatoid tumor of the lung

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. 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 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

  • In 1987, EGFR gene mutations were first identified in the pathogenesis of sarcomatoid carcinoma of the lung.[1]

Classification

  • Sarcomatoid carcinoma of the lung may be classified according to WHO classification into 5 subtypes:[2]
  • Pleomorphic carcinoma
  • Spindle cell carcinoma
  • Giant cell carcinoma
  • Carcinosarcoma
  • Pulmonary blastoma

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.[3]
  • 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 may be caused by a mutation in the EGFR and K-ras genes.[3]

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:

Epidemiology and Demographics

  • The prevalence of sarcomatoid carcinoma of the lung is approximately 0.4 cases per 100,000 individuals worldwide.[3]
  • The overall prevalence of sarcomatoid carcinoma of the lung is 0.1% to 0.4% of all non-small cell lung malignancies.[3]

Age

  • Sarcomatoid carcinoma of the lung is more commonly diagnosed among patients aged 65 to 75 years old.[4]
  • Sarcomatoid carcinoma of the lung is more commonly observed among elderly patients and adults.

Gender

  • Males are more commonly affected with sarcomatoid carcinoma of the lung than females.[5][6]
  • the male to female ratio is 4:1.[4]

Race

  • There is no racial predilection for 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.[7]

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.[8]
  • 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.[9][10]
  • 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%.[6]

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:[10]
  • Pleomorphic carcinoma subtype
  • Spindle cell carcinoma subtype
  • Giant cell carcinoma subtype
  • Carcinosarcoma subtype

Symptoms

  • Common symptoms of sarcomatoid carcinoma of the lung, may include:[10]

Physical Examination

  • Patients with sarcomatoid carcinoma of the lung usually appear older in age in relation to their chronological age.[10]
  • Physical examination may be remarkable for:

Inspection

Auscultation

Percussion

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:[10]
    • 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[11]

Other Diagnostic Studies

Treatment

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.[10]

Surgery

  • Surgery is the mainstay of therapy for sarcomatoid carcinoma of the lung.
  • 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

  • Effective measures for the primary prevention of sarcomatoid carcinoma of the lung include CT screening.[10]
  • 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

  1. Timeline of lung cancer. http://cancerprogress.net/timeline/lung-cancer Accessed on February 17, 2016
  2. Sarcomatoid carcinoma of the lung. Wikipedia. https://en.wikipedia.org/wiki/Sarcomatoid_carcinoma_of_the_lung Accessed on March 31, 2016
  3. 3.0 3.1 3.2 3.3 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. PMID 20073605. doi:10.1043/2008-0547-RAR.1. 
  4. 4.0 4.1 Ouziane, Imane; Boutayeb, Saber; Mrabti, Hind; Lalya, Issam; Rimani, Mouna; Errihani, Hassan (2014). "Sarcomatoid carcinoma of the lung: A model of resistance of chemotherapy". North American Journal of Medical Sciences. 6 (7): 342. ISSN 1947-2714. doi:10.4103/1947-2714.136920. 
  5. Petrov, D.B; Vlassov, V.I; Kalaydjiev, G.T; Plochev, M.A; Obretenov, E.D; Stanoev, V.I; Danon, S.E (2003). "Primary pulmonary sarcomas and carcinosarcomas – postoperative results and comparative survival analysis". European Journal of Cardio-Thoracic Surgery. 23 (4): 461–466. ISSN 1010-7940. doi:10.1016/S1010-7940(03)00024-1. 
  6. 6.0 6.1 Roesel, Christian; Terjung, Sarah; Weinreich, Gerhard; Hager, Thomas; Chalvatzoulis, Eleftherios; Metzenmacher, Martin; Welter, Stefan (2016). "Sarcomatoid carcinoma of the lung: a rare histological subtype of non-small cell lung cancer with a poor prognosis even at earlier tumour stages". Interactive CardioVascular and Thoracic Surgery: ivw392. ISSN 1569-9293. doi:10.1093/icvts/ivw392. 
  7. 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
  8. Travis WD; Brambilla E; Müller-Hermelink K; Harris C; Kleihues C; Sobin P. https://www.iarc.fr/wp-content/uploads/2018/07/BB10.pdf (PDF). World Health Organization Classification of Tumours; Pathology and genetics of tumors of the lung, pleura, thymus, and heart. IARC Press. pp. 53–58. ISBN ISBN 92 832 2418 3. 
  9. Yendamuri S, Caty L, Pine M, Adem S, Bogner P, Miller A, Demmy TL, Groman A, Reid M (2012). "Outcomes of sarcomatoid carcinoma of the lung: a Surveillance, Epidemiology, and End Results Database analysis". Surgery. 152 (3): 397–402. PMID 22739072. doi:10.1016/j.surg.2012.05.007. 
  10. 10.0 10.1 10.2 10.3 10.4 10.5 10.6 Hountis P, Moraitis S, Dedeilias P, Ikonomidis P, Douzinas M (2009). "Sarcomatoid lung carcinomas: a case series". Cases J. 2: 7900. PMC 2740247Freely accessible. PMID 19830024. doi:10.4076/1757-1626-2-7900. 
  11. Silvestri, Gerard A.; Gould, Michael K.; Margolis, Mitchell L.; Tanoue, Lynn T.; McCrory, Douglas; Toloza, Eric; Detterbeck, Frank (2007). "Noninvasive Staging of Non-small Cell Lung Cancer". Chest. 132 (3): 178S–201S. ISSN 0012-3692. doi:10.1378/chest.07-1360. 

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