{"entity":{"id":"pulmonary-sarcomatoid-carcinoma","kind":"cancer","name":"Sarcomatoid carcinoma of the lung","aka":["Pulmonary sarcomatoid carcinoma","Sarcomatoid carcinoma of the lung","Pleomorphic carcinoma of the lung","Spindle cell carcinoma of the lung","Giant-cell carcinoma of the lung","Giant cell carcinoma of the lung","Pulmonary carcinosarcoma"],"tldr":"Sarcomatoid carcinoma is a rare, aggressive form of lung cancer in which part or all of the tumour looks like a sarcoma, with spindle-shaped or giant cells. It is treated like other non-small-cell lung cancers, but is worth testing for a MET gene fault, which is found in a sizeable minority and can be treated with a tablet.","summary":"The WHO classification groups five poorly differentiated non-small-cell carcinomas under sarcomatoid carcinoma: pleomorphic carcinoma (a carcinoma with at least 10 percent spindle or giant cells), spindle cell carcinoma, giant cell carcinoma, carcinosarcoma (carcinoma with true sarcoma such as bone, cartilage or skeletal muscle) and pulmonary blastoma, which has its own page (Nicholson 2022). Patients are mostly men who smoke: in the French series of 93 cases, 77 percent were men, median age 63, 84 percent smokers, and about 70 percent presented with locally advanced or metastatic disease; median survival after surgery was 16.4 months and distant recurrence was common (Vieira 2016).\n\nHow it differs from its parent: it is the most treatment-refractory histology of non-small-cell lung cancer, and it carries MET alterations far more often. Whole-exome sequencing of pulmonary sarcomatoid carcinoma found MET exon 14 skipping mutations at high frequency, and a patient with advanced disease responded to the MET inhibitor crizotinib (Liu 2016); a later multicentre study of 124 resected cases found MET exon 14 skipping in 7.3 percent, MET amplification in 4.8 percent and MET protein overexpression in 20.2 percent, the exon 14 cases being pleomorphic, spindle cell and carcinosarcoma (Translational Oncology 2020).\n\nHow common: 0.4 percent of lung cancers in SEER (Yendamuri 2012).\n\nTreatment: no randomised trial exists for the histology; stage-based treatment follows the parent page, and MET exon 14 skipping is treated on the MET-altered page with capmatinib or tepotinib. Checkpoint inhibitors are used as for other non-small-cell lung cancer.","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Sarcomatoid_carcinoma","links":[{"label":"Nicholson 2022, JTO: the 2021 WHO classification of lung tumours","url":"https://doi.org/10.1016/j.jtho.2021.11.003"},{"label":"Yendamuri 2012, Surgery: outcomes of sarcomatoid carcinoma of the lung, SEER analysis","url":"https://doi.org/10.1016/j.surg.2012.05.007"},{"label":"Vieira 2016, Clinical Lung Cancer: characteristics and outcomes of 93 sarcomatoid carcinomas of the lung","url":"https://doi.org/10.1016/j.cllc.2016.03.001"},{"label":"Liu 2016, JCO: sequencing of pulmonary sarcomatoid carcinoma reveals frequent MET exon 14 mutations","url":"https://doi.org/10.1200/jco.2015.62.0674"},{"label":"Translational Oncology 2020: MET exon 14 skipping, amplification and overexpression in 124 pulmonary sarcomatoid carcinomas","url":"https://doi.org/10.1016/j.tranon.2020.100868"}],"tags":["subtype-page","wave4","lung"],"related":["nsclc","met-altered-nsclc","pulmonary-blastoma","large-cell-lung-carcinoma","lung-adenocarcinoma"],"cancers":[],"sections":[],"technologies":[],"targets":["met"],"drugs":["capmatinib","tepotinib","crizotinib","savolitinib"],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"lung","burden":"0.4 percent of lung cancers: 3,647 of 878,810 patients in the US SEER registry (Yendamuri 2012). Pleomorphic carcinoma is the commonest form, 64 percent of 93 cases in two French centres (Vieira 2016).","subtypes":["Pleomorphic carcinoma (at least 10 percent spindle or giant cells; the commonest)","Spindle cell carcinoma","Giant cell carcinoma","Carcinosarcoma (carcinoma with heterologous sarcoma)","Pulmonary blastoma (own page)","MET exon 14-mutated sarcomatoid carcinoma"],"biomarkers":["MET exon 14 skipping (7.3 percent), MET amplification and overexpression","KRAS, TP53, PIK3CA, STK11 and RB1 mutations (Liu 2016)","PD-L1","Cytokeratin positivity in the spindle component (distinguishes it from sarcoma)"],"standardOfCare":[{"setting":"All stages","approach":"Treated as non-small-cell lung cancer by stage; MET exon 14 skipping treated on the MET-altered page with capmatinib or tepotinib.","refs":["nsclc","met-altered-nsclc","capmatinib","tepotinib"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"nsclc"},"route":"/cancers/pulmonary-sarcomatoid-carcinoma/","neighbours":{"cancer":[{"id":"lung-adenocarcinoma","kind":"cancer","name":"Adenocarcinoma of the lung","route":"/cancers/lung-adenocarcinoma/"},{"id":"large-cell-lung-carcinoma","kind":"cancer","name":"Large cell carcinoma of the lung","route":"/cancers/large-cell-lung-carcinoma/"},{"id":"met-altered-nsclc","kind":"cancer","name":"MET exon 14 and MET-amplified non-small-cell lung cancer","route":"/cancers/met-altered-nsclc/"},{"id":"nsclc","kind":"cancer","name":"Non-small-cell lung cancer","route":"/cancers/nsclc/"},{"id":"pulmonary-blastoma","kind":"cancer","name":"Pulmonary blastoma (adult)","route":"/cancers/pulmonary-blastoma/"}],"target":[{"id":"met","kind":"target","name":"MET","route":"/targets/met/"}],"drug":[{"id":"capmatinib","kind":"drug","name":"Capmatinib","route":"/drugs/capmatinib/"},{"id":"crizotinib","kind":"drug","name":"Crizotinib","route":"/drugs/crizotinib/"},{"id":"savolitinib","kind":"drug","name":"Savolitinib","route":"/drugs/savolitinib/"},{"id":"tepotinib","kind":"drug","name":"Tepotinib","route":"/drugs/tepotinib/"}]}}