Pulmonary sarcomatoid carcinomas (PSCs) are poorly differentiated non-small cell lung carcinomas (NSCLCs) containing a sarcoma-like element (malignant spindle or giant cells) or sarcomatous component (neoplastic bone, cartilage, or striated muscle) [8
]. In the World Health Organization (WHO) classification, spindle cell carcinoma is classified as a variant of squamous cell carcinoma and giant cell carcinoma is classified as a variant of large cell carcinoma [9
]. However, Fishback et al
. designated SC as pleomorphic (spindle/giant cell) carcinomas of the lung because both spindle and giant cell components are often found in the same tumors [10
PSC patients are predominantly male and smokers, with a male-to-female ratio of 4.4: 1 and average age at presentation of 51.4 years in the published series [11
]. The patient in our case also had a long history of cigarette smoking. Besides, it is believed by some authors that radiotherapy for the treatment of a patient's other cancers may also induce a sarcomatoid change in lung tissue, which can accelerate the subsequent development of SC [12
Pulmonary sarcomatoid carcinoma presents as either a central or peripheral lesion, most often in the right upper lobes. It grows by invading the bronchial tree, the pulmonary parenchyma, and the adjacent anatomical structures (mediastinum and chest wall) in the form of widely necrotic and hemorrhagic, round to bosselated large masses [13
]. In our case, the lobulated mass occupied the left hilum and even stretched to mediastinal structures, such as the left pulmonary artery. Significant BVI obstruction was also observed. The tumor was ovoid in shape, poorly circumscribed, yellow-tan in color, heavy, hemorrhagic, and necrotic.
The clinical signs and symptoms may be related to tumor localization. Symptoms such as chest pain, accumulation of excessive fluid in the pleural cavity, and difficulty breathing are shared by most pulmonary carcinomas. This makes it hard to distinguish them from each other. In endobronchial tumors, coughing and blood-tinged sputum usually occur, but peripheral tumors are asymptomatic [14
]. However, no paraneoplastic syndrome was reported to accompany PSC, although such symptoms occurred in about 15–20% of small cell lung carcinomas (SCLCs) and 5–8% of NSCLCs [15
]. PSCs metastasize via lymph and blood vessel routes to the same anatomical sites as conventional NSCLCs. Therefore, in general, PSC is classified using the same TNM criteria as other NSCLCs.
Although the final diagnosis of PSC depends on the close scrutiny of histopathology, its immunohistochemical analysis may help to better highlight the different cell components occurring in these tumors [16
]. The epithelial component is stained with antibodies to cytokeratins, EMA, and carcinoembryonic antigen (CEA), whereas the opposite holds true for the sarcomatous or sarcomatoid component, which is instead consistently immunoreactive for vimentin. In our case, we found that the PSC specimen was positive for EMA, CK, and vimentin, but negative for TTF-1, SMA, and cytokeratin 5/6. This result suggested that our case had sarcomatoid components and epithelial phenotype, which was in accordance with previous reports [17
Pulmonary sarcomatoid carcinoma is an aggressive cancer and the resultant tumors are frequently symptomatic, locally advanced, and have high rates of recurrence. Recently, although radical surgery is still reported as the best option for PSC patients [20
], many non-surgical treatments are recommended for patients with clinically advanced sarcomatoid cancer, such as chemotherapy. Chemotherapy is often performed to treat patients according to the results of trials that enrolled patients with lung squamous cell carcinoma or adenocarcinoma. Italiano et al
] reported the short-term outcomes of using doxorubicin and ifosfamide in the treatment of metastatic SC. Half of their patients with metastatic SC experienced an initial response to chemotherapy with doxorubicin and ifosfamide. However, all patients ultimately died of disease despite continued treatment [22
]. Therefore, due to the limited treatment, the five-year survival rate for patients with PSC was only 24.5%, relatively worse than for patients with other forms of NSCLC (46.3%).