Stella Giulia Maria, D'Agnano Vito, Piloni Davide, Saracino Laura, Lettieri Sara, Mariani Francesca, Lancia Andrea, Bortolotto Chandra, Rinaldi Pietro, Falanga Francesco, Primiceri Cristiano, Corsico Angelo Guido, Bianco Andrea
Department of Medical Sciences and Infective Diseases, Unit of Respiratory Diseases, IRCCS Policlinico San Matteo Foundation and University of Pavia Medical School, Pavia, Italy.
Department of Translational Medical Sciences, University of Campania "L. Vanvitelli", Napoli, Italy.
Transl Lung Cancer Res. 2022 Mar;11(3):472-496. doi: 10.21037/tlcr-21-880.
Translational research is a source of continuous innovation in medicine, more particularly for clinical research on new treatment modalities in idiopathic pulmonary fibrosis (IPF) patients. However, the heterogeneity of the disease is well recognized, and different pathological and molecular settings have been identified. The molecular mechanisms by which IPF proceeds in time and space remains poorly understood. Although some IPF features are reminiscent of cancer, the dynamics of malignant divergent clonal selective pressure and heterogeneity clearly differ from those occurring in IPF. This is reflected in the absence of patient proper selection and stratification to biological agents (pirfenidone, nintedanib) which limit therapeutic efficacy. Consequently, increased costs are related to the clinical management of advanced IPF patients. Steady collaboration and fluid communication between pneumo-oncologists, radiologists and molecular biologists is a clear priority for the correct interpretation of tests and the definition of effective personalized strategies against this orphan disease. The present work aims at providing the most relevant hints shared by cancer and IPF.
A systematic literature review was performed to identify all relevant data. The examined databases were Scopus, Web of Science, Cochrane, Google Scholar, and PubMed. The last search was run on January 5, 2022. We have primarily conducted separated research for lung cancer, IPF, genetics, epigenetics, surgery in IPF and cancer.
The data here presented mainly focus on gene mutations, epigenetics and novel therapeutic approaches. Moreover, epidemiology, prognostic variables and in new treatment strategies adopted in patients with IPF and lung cancer are discussed as well.
Overall, the findings of this narrative review will be of help in defining the key molecular features that could applied in IPF setting with promising rationale to improve therapy and to better manage those cases carrying IPF and cancer concomitantly.
转化研究是医学持续创新的源泉，尤其对于特发性肺纤维化（IPF）患者新治疗模式的临床研究而言。然而，该疾病的异质性已得到充分认识，并且已识别出不同的病理和分子背景。IPF在时间和空间上发展的分子机制仍知之甚少。尽管IPF的一些特征让人联想到癌症，但恶性分化克隆选择压力和异质性的动态变化显然与IPF中发生的情况不同。这反映在缺乏对生物制剂（吡非尼酮、尼达尼布）的患者适当选择和分层上，这限制了治疗效果。因此，晚期IPF患者的临床管理成本增加。肺肿瘤学家、放射科医生和分子生物学家之间的稳定合作和顺畅沟通是正确解读检查结果以及定义针对这种罕见疾病的有效个性化策略的明确优先事项。本研究旨在提供癌症和IPF共有的最相关线索。
进行了系统的文献综述以识别所有相关数据。所检索的数据库包括Scopus、科学网、考克兰图书馆、谷歌学术和PubMed。最后一次检索于2022年1月5日进行。我们主要分别对肺癌、IPF、遗传学、表观遗传学、IPF和癌症中的手术进行了研究。
此处呈现的数据主要聚焦于基因突变、表观遗传学和新的治疗方法。此外，还讨论了IPF和肺癌患者的流行病学、预后变量以及新的治疗策略。
总体而言，本叙述性综述的结果将有助于确定关键分子特征，这些特征可应用于IPF背景下，具有改善治疗并更好地管理同时患有IPF和癌症病例的前景。
