Angela Pang, National University Health System and National University Cancer Institute, Singapore, Singapore; Mariana Carbini, Mount Sinai Medical Center; Andre L. Moreira, New York University Langone Medical Center, New York; and Robert G. Maki, Northwell Health Monter Cancer Center and Cold Spring Harbor Laboratory, Lake Success, NY.
J Clin Oncol. 2018 Jan 10;36(2):210-216. doi: 10.1200/JCO.2017.74.9523. Epub 2017 Dec 8.
In this review, we outline the biology and management of patients with carcinosarcomas and related malignancies, which are often included under the broader concept of sarcomatoid carcinomas. Carcinosarcomas are unusual tumors that are commonly gynecologic in origin, where they are referred to as malignant mixed Müllerian tumors, but may appear in any anatomic site. Although a variety of hypotheses have been presented as to the biphasic nature of these tumors, carcinosarcomas seem to represent the best example in human cancers of the concept of epithelial-mesenchymal transition (EMT), in which the two parts of the tumor are genomically related to one another, as opposed to the mesenchymal component that represents a second neoplasm or (benign) reactive process. In general, patients with carcinosarcomas fare worse than patients with carcinomas of the same anatomic site. Treatment paradigms for carcinosarcomas generally follow those of carcinomas of the same organ site, except where clinical trials provide more specific options. Agents that block or reverse EMT are worth examination in patients with carcinosarcoma and arguably may be even more effective in carcinomas, given evidence of dependence on EMT to generate successful metastases. Information about EMT may also inform other phase transitions in cancer, such as those between prostate or lung carcinoma and more aggressive tumors with neuroendocrine differentiation.
在这篇综述中，我们概述了癌肉瘤和相关恶性肿瘤患者的生物学特性和治疗管理，这些肿瘤通常包含在更广泛的肉瘤样癌概念中。癌肉瘤是一种不常见的肿瘤，通常起源于妇科，被称为恶性混合性 Müllerian 肿瘤，但也可能出现在任何解剖部位。尽管已经提出了多种假说来解释这些肿瘤的双相性质，但癌肉瘤似乎是人类癌症中上皮-间充质转化（EMT）概念的最佳例证，其中肿瘤的两个部分在基因组上彼此相关，而不是代表第二个肿瘤或（良性）反应性过程的间充质成分。一般来说，患有癌肉瘤的患者比患有同一解剖部位的癌患者预后更差。癌肉瘤的治疗方案通常遵循同一器官部位的癌的治疗方案，除非临床试验提供了更具体的选择。阻断或逆转 EMT 的药物值得在癌肉瘤患者中进行检查，并且鉴于 EMT 对产生成功转移的依赖性，这些药物在癌中可能更有效。关于 EMT 的信息也可能为癌症的其他相变提供信息，例如前列腺癌或肺癌与具有神经内分泌分化的更具侵袭性肿瘤之间的相变。
