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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.
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).
How 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).
How common: 0.4 percent of lung cancers in SEER (Yendamuri 2012).
Treatment: 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.
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).
Central tumours arise in the large airways, peripheral ones in the alveoli; both drain to hilar then mediastinal nodes, and the pleural lining is a separate cancer site.
Same organ: Mediastinal germ cell tumour, Pleuropulmonary blastoma (types I, Ir, II and III), Type A and type AB thymoma, Type B1 and type B2 thymoma, Type B3 thymoma, Micronodular thymoma with lymphoid stroma, Adenocarcinoma of the lung, Squamous cell carcinoma of the lung, Large cell carcinoma of the lung, Adenosquamous carcinoma of the lung, Invasive mucinous adenocarcinoma of the lung, Adenocarcinoma in situ and minimally invasive adenocarcinoma of the lung, Basaloid squamous cell carcinoma of the lung, Lymphoepithelial carcinoma of the lung, Pulmonary blastoma (adult), Non-small-cell lung cancer, Lung cancer (all types), Small-cell lung cancer, Mesothelioma, Pleural mesothelioma, Thymoma and thymic carcinoma, Childhood lung and airway tumours (pleuropulmonary blastoma, tracheobronchial tumours), Inflammatory myofibroblastic tumour (IMT), EGFR-mutated non-small-cell lung cancer, ALK-positive non-small-cell lung cancer, KRAS G12C-mutant non-small-cell lung cancer, ROS1-positive non-small-cell lung cancer, MET exon 14 and MET-amplified non-small-cell lung cancer, RET fusion-positive non-small-cell lung cancer, BRAF V600E-mutant non-small-cell lung cancer, HER2-mutant non-small-cell lung cancer, NTRK fusion-positive non-small-cell lung cancer, PD-L1-high non-small-cell lung cancer without a driver mutation, Resectable stage I to III non-small-cell lung cancer, Unresectable stage III non-small-cell lung cancer, Limited-stage small-cell lung cancer, Extensive-stage small-cell lung cancer, Lung neuroendocrine tumours (typical and atypical carcinoid), Thymoma (WHO types A, AB, B1, B2 and B3), Thymic carcinoma
Treated as non-small-cell lung cancer by stage; MET exon 14 skipping treated on the MET-altered page with capmatinib or tepotinib.
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Query for this cancer: (TITLE:"Sarcomatoid carcinoma of the lung" OR ABSTRACT:"Sarcomatoid carcinoma of the lung" OR TITLE:"Pulmonary sarcomatoid carcinoma" OR ABSTRACT:"Pulmonary sarcomatoid carcinoma" OR TITLE:"Pleomorphic carcinoma of the lung" OR ABSTRACT:"Pleomorphic carcinoma of the lung" OR TITLE:"Spindle cell carcinoma of the lung" OR ABSTRACT:"Spindle cell carcinoma of the lung" OR TITLE:"Giant-cell carcinoma of the lung" OR ABSTRACT:"Giant-cell carcinoma of the lung" OR TITLE:"Giant cell carcinoma of the lung" OR ABSTRACT:"Giant cell carcinoma of the lung") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Sarcomatoid carcinoma of the lung, not a curated reading list.
The targets of this cancer's medicines and the ones linked to it directly.
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Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
See all on the product pages:CapmatinibTepotinib·Printable cards in the navigator
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