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Approaches to Tumor Classification in Pulmonary Sarcomatoid Carcinoma

机译：肺瘤样癌肿瘤分类方法

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Pulmonary sarcomatoid carcinoma (PSC) is a heterogeneous category of primary lung cancer accounting from 0.3% to 3% of all primary lung malignancies. According to the most recent 2015 World Health Organization (WHO) classification, PSC includes several different variants of malignant epithelial tumors (carcinomas) histologically mimicking sarcomas showing or entirely lacking a conventional component of non-small cell lung cancer (NSCLC). Thus, this rare subheading of lung neoplasms includes pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, pulmonary blastoma, and carcinosarcoma. A diagnosis of PSC may be suspected on small biopsy or cytology, but commonly requires a surgical resection to reach a conclusive definition. The majority of patients with PSC consists of elderly, smoking men with a large, peripheral mass characterized by well-defined margins. However, presentation with a central, polypoid endobronchial lesion is well-documented, particularly in pleomorphic carcinoma and carcinosarcoma showing a squamous cell carcinoma component. As expected, PSC may pose diagnostic problems and immunohistochemistry is largely used when pathologists deal these tumors in routine practice. Indeed, PSC tends to overexpress molecules associated with the epithelial-to-mesenchymal transition, such as vimentin, but the panel of immunostains also includes epithelial markers (cytokeratins, EMA), TTF-1, p40 and negative markers (e.g., melanocytic, mesothelial and sarcoma-related primary antibodies). Although rare, PSC has increased their interest among oncologist community for different reasons: a. identification of the epithelial-to-mesenchymal phenomenon as a major mechanism of secondary resistance to tyrosine kinase inhibitors; b. over-expression of PD-L1 and effective treatment with immunotherapy; c. identification of c-MET exon 14 skipping mutation representing an effective target to crizotinib and other specific inhibitors. In this review, the feasibility of the diagnosis of PSC, its differential diagnosis and novel molecular findings characterizing this group of lung tumor are discussed.
机译：肺肉瘤样癌（PSC）是原发性肺癌的异质类别，占所有原发性肺部恶性肿瘤的0.3％至3％。根据最近2015年世界卫生组织（世卫组织）分类，PSC包括几种不同的恶性上皮肿瘤（癌）组织学模仿肉瘤，显示或完全缺乏非小细胞肺癌（NSCLC）的常规组分。因此，这种肺肿瘤的罕见副标题包括茂物癌，梭形细胞癌，巨型细胞癌，肺囊囊肿和癌症瘤。 PSC的诊断可以怀疑小的活组织检查或细胞学，但通常需要手术切除来达到结论性定义。 PSC大多数患者由老年人组成，吸烟男性具有大，周式质量，其特征在于明确定义的边距。然而，用中央蛋白蛋白胚胎病变呈现良好地记录，特别是在含有鳞状细胞癌组分的亲主癌和癌癌中。正如预期的那样，PSC可能会造成诊断问题，当病理学家在常规实践中处理这些肿瘤时，免疫组织化学在很大程度上使用。实际上，PSC倾向于过表达与上皮 - 间充质转换相关的分子，例如Vimentin，但免疫蛋白板也包括上皮标记物（细胞角蛋白，EMA），TTF-1，P40和阴性标记物（例如，Melanocytic，Mesithelial和肉瘤相关的一抗）。虽然罕见，但由于不同的原因，PSC增加了肿瘤科学家群体的兴趣：a。鉴定上皮 - 间充质现象作为副耐药于酪氨酸激酶抑制剂的主要机理;湾PD-L1的过度表达和免疫疗法有效治疗; C。鉴定C-Met外显子14跳过突变，其代表屈曲in和其他特异性抑制剂的有效靶标。在本综述中，讨论了PSC诊断的可行性，其表征该组肺肿瘤的鉴别诊断和新的分子结果。

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