Review Article: Pulmonary Sarcomatoid Carcinomas: A Practical Overview

Abstract

Pulmonary sarcomatoid carcinomas (PSCs) are currently defined as poorly differentiated non-small-cell carcinomas containing a component with sarcoma or sarcoma-like (spindle and/or giant cell) features. They consist of 5 major histological variants, namely pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma, and pulmonary blastoma. The segregation of PSCs into a distinct clinicopathologic entity seems justified on the basis of morphologic, behavioral, and genotypic/phenotypic attributes. As a group, PSCs generally run an aggressive clinical course and may cause major difficulties in the differential diagnosis with other primary and secondary malignancies of the lung. At present, PSCs are believed to represent a family of carcinomas "in transition," in which diverse pathways of clonal evolution account for histological differences of a common ancestor lesion. The sarcomatous or sarcomatoid component of these tumors is thought to derive from carcinoma cells during the progression of carcinogenesis through the activation of an epithelial-mesenchymal transition program leading to sarcomatous transformation or metaplasia (conversion paradigm). Conceivably, targeting the epithelial-mesenchymal transition program could become a valid therapeutic strategy for these life-threatening tumors, whose sensitivity to current medical manipulation is disappointing.

Utility of Immunohistochemistry in the Diagnosis of Pleuropulmonary and Mediastinal Cancers: A Review and Update

“…21,[46][47][48][49] The EGFR and KRAS mutations have been found in about 15% to 20% and 9.8% of pleomorphic carcinomas, respectively. 50 Table 4 shows markers commonly used in supporting the diagnosis of sarcomatoid carcinoma.…”

“…Table 8 shows markers that are useful for distinguishing pleural mesothelioma from certain types of sarcomas and sarcomatoid carcinomas involving the pleura. [47][48][49][50][51] Sometimes, in order to achieve the right diagnosis, input from gross and/or radiographic findings is required because of overlapping histologic and immunohistochemical features among these tumors. Mesotheliomas typically cause diffuse pleural thickening, while sarcomatoid carcinomas present as localized peripheral intrapulmonary masses and can invade the chest wall.…”

Advanced primary pulmonary carcinosarcoma: a case report and review of the literature

“…Two reports have demonstrated efficacy of doxorubicin-based chemotherapeutic regimen and combination of cisplatin and doxorubicin (19). Aggressive nature and poor differentiation of this tumor render treatment difficult and result in poor prognosis (20). …”

Pemetrexed and bevacizumab-containing chemotherapy for pleomorphic carcinoma of the lung

“…Pleomorphic carcinoma of the lung is a rare, highly malignant tumor, accounting for 0.1-0.4% of primary lung cancer cases (1)(2)(3). It has a more aggressive clinical course compared with other types of non-small-cell lung cancer (NSCLC) (4)(5)(6), as well as a poorer outcome (4,7,8).…”