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 carcinomas are 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. Sarcomatoid cancer of the lung is a rare type of malignancy and it comprises of only 0.3% to 1.3% of cases of all 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 the lymphogenic route. Prognosis is generally poor, even worse than other non-small cell carcinoma of the lung. The 5-year survival rate for patients with sarcomatoid cancer of the lung is 28.7%.

Historical Perspective

  • Sarcomatoid carcinoma was first described by Virchow in 1865 as a “biphasic” lesion of adenocarcinomatous or squamous cell carcinoma with spindle cell or giant cell component.[1]
  • 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.[2]

Classification

Sarcomatoid carcinoma of the lung can be classified according to WHO classification into 5 subtypes:[2][3]

  • Pleomorphic carcinoma
  • Spindle cell carcinoma
  • Giant cell carcinoma
  • Carcinosarcoma
  • Pulmonary blastoma

Sarcomatoid carcinoma of the lung can also be classified according to their location including:[3]

  • Central
  • Peripheral

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][4]
  • The EGFR and K-ras gene mutations have been associated with the development of sarcomatoid carcinoma of the lung.[5]
  • Four major hypotheses may explain sarcomatoid neoplasms:[6][7]
    • 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.

Gross Pathology[8]

  • Sarcomatoid carcinomas can arise centrally or peripherally.[9]
  • Most commonly present as poorly circumscribed solitary peripheral masses.
  • Sarcomatoid carcinoma is commonly found in upper lobe of 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:[10]
    • 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
      • A mixture of carcinoma and true sarcomas such as malignant bone sarcoma, cartilage, or skeletal muscle sarcoma.
      • The carcinoma component may be squamous cell carcinoma, adenocarcinoma, adenocarcinoma mixed with squamous cell carcinoma, and large cell carcinoma, whereas the sarcoma component may be rhabdomyosarcoma, osteosarcoma mixed with chondrosarcoma, and osteosarcoma.
    • 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.

Causes

There are no direct causes of sarcomatoid carcinoma of lung. However, there are a few risk factors that can lead to genetic mutation and cause sarcomatoid carcinoma of lung.

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

Prevalence

  • The prevalence of sarcomatoid carcinoma of the lung is approximately 0.4 cases per 100,000 individuals worldwide.[4]
  • Sarcomatoid carcinoma comprises of 0.3% to 1.3% of total lung malignancies.[11]
  • 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.[12]
  • 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.[13][14]
  • the male to female ratio is 4:1.[12]
  • Biphasic blastoma subtype is an exception, that affects men and women equally.

Race

  • There is no racial predilection for sarcomatoid carcinoma of the lung.

Region

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 the following:[15][16][17][18]

  • History of cigarettes, cigars, or pipe smoking
  • Secondhand or passive smoking
  • Family history of lung cancer
  • Genetic predisposition
  • High levels of air pollution
  • Radiation therapy to the chest
  • Heavy alcohol consumption
  • Radon gas
  • Occupational exposure to silica, asbestos, uranium, arsenic, chromium, or nuclear radiation
  • Previous history of lung diseases

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, weight loss and fatigue.[19]
  • 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.[20][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%.[14]

Diagnosis

Diagnostic Study of Choice

  • Diagnostic modality of choice for sarcomatoid carcinoma of lung is CT scan of the chest.[21]
  • The definitive diagnosis of sarcomatoid carcinoma of the lung is made by immunohistochemical analysis and pathological subtyping of the specimen collected from biopsy, needle aspiration, sputum cytology or surgical resection of the tumor.[14]

History and Symptoms

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

Physical Examination

Physical exam findings for sarcomatoid carcinoma of lung is similar to physical exam finding of other lung cancer.

  • Please click here for physical exam finding of lung cancer.

Laboratory Findings

  • There are no specific laboratory findings associated with sarcomatoid carcinoma of the lung.

X-ray

An x-ray may be helpful in the diagnosis of lung cancer. Findings on an x-ray suggestive of lung cancer include:[22][23][24]

CT scan

  • CT scan of chest is the imaging investigation of choice for sarcomatoid carcinoma of the lung.[21]
  • CT scan is useful in diagnosing the tumor and also to rule out any possible metastasis.
  • CT scan finding of sarcomatoid carcinoma of lung is similar to CT scan finding of lung cancer.
    • For more information on CT scan finding of lung cancer click here.
  • On CT, sarcomatoid carcinoma of the lung is characterized by the following findings:
    • Sarcomatoid carcinoma of the lung may arise from center or the peripheral part of the lung
    • Usually measure approximately 5 cm in diameter
    • Mixed density
    • Perihilar and mediastinal involvement
    • Ground glass opacity
    • Rapid growth
  • For more information on PET-CT findings of lung cancer, click here.

Other Diagnostic Studies

  • The definitive diagnosis of sarcomatoid carcinoma of the lung is made by immunohistochemical analysis and pathological subtyping of the specimen collected from biopsy, needle aspiration, sputum cytology or surgical resection of the tumor.[14]
  • Specimen for immunohistochemical analysis and pathological subtyping may be collected by transthoracic needle biopsy, bronchoscopy with biopsy, surgical resection, video-assisted thoracoscopic surgery (VATS), endoscopic ultrasound guided fine needle aspiration (EUS-FNA), endobronchial ultrasound guided transbronchial needle aspiration (EBUS-TBNA) and rarely sputum cytology.

Biopsy

  • Biopsy findings associated with sarcomatoid carcinoma of the lung will depend on the histopathological subtypes.[2]

Immunohistochemical Staining

  • Sarcomatoid carcinoma of lung may be positive for the following immunohistochemical markers: [25][26]
    • CAM5.2, CK7, thyroid transcription factor 1(TTF-1), napsin A, AE1/AE3, WT1, calretinin, Sox2, CK5/6, p40, S-100, desmin, SMAD2-40 and/or desmocollin 3.
  • Pleomorphic, spindle and/or giant cell carcinoma may show findings positive for component of squamous cell carcinoma, adenocarcinoma, or large cell carcinoma.
  • In case of poorly differentiated pleomorphic, spindle and/or giant cell carcinoma markers for epithelial components such as keratin antibodies and EMA (epithelial membrane marker).
  • Pleomorphic, spindle and/or giant cell carcinoma may also be positive for cytokeratin, vimentin, carcinoembryonic antigen, and smooth muscle markers.
  • Giant cell carcinomas may be positive for TTF-1.
  • Carcinosarcomas may be positive for keratin antibodies.
  • Variants of carcinosarcoma sub type such as chondrosarcoma may be positive for S-100 protein and rhabdomyosarcoma for muscle markers.

Treatment

Medical Therapy

  • The medical therapy of sarcomatoid carcinoma of the lung depends on the staging of the tumor.
  • Medical therapy for sarcomatoid carcinoma of lung is similar to medical therapy of other non-small cell carcinoma of the lung.
  • For more information regarding medical therapy of sarcomatoid carcinoma of lung click here.

Surgery

  • Surgery is the mainstay of therapy for sarcomatoid carcinoma of the lung.
  • Surgical therapy for sarcomatoid carcinoma of the lung is similar to surgical therapy of other non-small cell carcinoma of the lung.
  • For more information on surgical therapy of sarcomatoid carcinoma of the lung click here.

Prevention

  • Effective measures for the primary prevention of sarcomatoid carcinoma of the lung include CT screening.
  • For information regarding current lung cancer screening guidelines click here.

References

  1. ↑ Virchow, Rudolf (1863). Die KRANKHAFTEN GESCHWÜLSTE. 2. Retrieved 1864. Check date values in: |access-date= (help)
  2. ↑ 2.0 2.1 2.2 Sobin LH (1981). "The international histological classification of tumours". Bull. World Health Organ. 59 (6): 813–9. PMC 2396133. PMID 6978190.
  3. ↑ 3.0 3.1 3.2 3.3 Brambilla, E.; Travis, W.D.; Colby, T.V.; Corrin, B.; Shimosato, Y. (2001). "The new World Health Organization classification of lung tumours". European Respiratory Journal. 18 (6): 1059–1068. doi:10.1183/09031936.01.00275301. ISSN 0903-1936.
  4. ↑ 4.0 4.1 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.
  5. ↑ 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.
  6. ↑ 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.
  7. ↑ 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.
  8. ↑ 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.
  9. ↑ 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.
  10. ↑ 10.0 10.1 10.2 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.
  11. ↑ Travis WD; Brambilla E; Müller-Hermelink K; Harris C; Kleihues C; Sobin P. https://www.iarc.fr/wp-content/uploads/2018/07/BB10.pdf |chapterurl= missing title (help) (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 Check |isbn= value: invalid character (help).
  12. ↑ 12.0 12.1 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. doi:10.4103/1947-2714.136920. ISSN 1947-2714.
  13. ↑ 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. doi:10.1016/S1010-7940(03)00024-1. ISSN 1010-7940.
  14. ↑ 14.0 14.1 14.2 14.3 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. doi:10.1093/icvts/ivw392. ISSN 1569-9293.
  15. ↑ Henley SJ, Thun MJ, Chao A, Calle EE (June 2004). "Association between exclusive pipe smoking and mortality from cancer and other diseases". J. Natl. Cancer Inst. 96 (11): 853–61. PMID 15173269.
  16. ↑ Hackshaw AK, Law MR, Wald NJ (October 1997). "The accumulated evidence on lung cancer and environmental tobacco smoke". BMJ. 315 (7114): 980–8. PMC 2127653. PMID 9365295.
  17. ↑ Freudenheim JL, Ritz J, Smith-Warner SA, Albanes D, Bandera EV, van den Brandt PA, Colditz G, Feskanich D, Goldbohm RA, Harnack L, Miller AB, Rimm E, Rohan TE, Sellers TA, Virtamo J, Willett WC, Hunter DJ (September 2005). "Alcohol consumption and risk of lung cancer: a pooled analysis of cohort studies". Am. J. Clin. Nutr. 82 (3): 657–67. doi:10.1093/ajcn.82.3.657. PMID 16155281.
  18. ↑ Boffetta P (August 2004). "Epidemiology of environmental and occupational cancer". Oncogene. 23 (38): 6392–403. doi:10.1038/sj.onc.1207715. PMID 15322513.
  19. ↑ Travis WD; Brambilla E; Müller-Hermelink K; Harris C; Kleihues C; Sobin P. https://www.iarc.fr/wp-content/uploads/2018/07/BB10.pdf |chapterurl= missing title (help) (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 Check |isbn= value: invalid character (help).
  20. ↑ 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. doi:10.1016/j.surg.2012.05.007. PMID 22739072.
  21. ↑ 21.0 21.1 Purandare, NilenduC; Rangarajan, Venkatesh (2015). "Imaging of lung cancer: Implications on staging and management". Indian Journal of Radiology and Imaging. 25 (2): 109. doi:10.4103/0971-3026.155831. ISSN 0971-3026.
  22. ↑ Jackman, David M; Johnson, Bruce E (2005). "Small-cell lung cancer". The Lancet. 366 (9494): 1385–1396. doi:10.1016/S0140-6736(05)67569-1. ISSN 0140-6736.
  23. ↑ Whitten, Camilla R.; Khan, Sameer; Munneke, Graham J.; Grubnic, Sisa (2007). "A Diagnostic Approach to Mediastinal Abnormalities". RadioGraphics. 27 (3): 657–671. doi:10.1148/rg.273065136. ISSN 0271-5333.
  24. ↑ Purandare, NilenduC; Rangarajan, Venkatesh (2015). "Imaging of lung cancer: Implications on staging and management". Indian Journal of Radiology and Imaging. 25 (2): 109. doi:10.4103/0971-3026.155831. ISSN 0971-3026.
  25. ↑ Weissferdt A, Kalhor N, Rodriguez Canales J, Fujimoto J, Wistuba II, Moran CA (January 2017). "Spindle cell and pleomorphic ("sarcomatoid") carcinomas of the lung: an immunohistochemical analysis of 86 cases". Hum. Pathol. 59: 1–9. doi:10.1016/j.humpath.2016.08.003. PMID 27569293.
  26. ↑ Terra SB, Aubry MC, Yi ES, Boland JM (February 2014). "Immunohistochemical study of 36 cases of pulmonary sarcomatoid carcinoma--sensitivity of TTF-1 is superior to napsin". Hum. Pathol. 45 (2): 294–302. doi:10.1016/j.humpath.2013.09.005. PMID 24331839.
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