{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Longo V"],"funding":["Italian Ministry of Health"],"pagination":["3237"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC10970296"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["25(6)"],"pubmed_abstract":["Recently, the fifth edition of the WHO classification recognized the thoracic <i>SMARCA4</i>-deficient undifferentiated tumor (SMARCA4-UT) as a separate entity from conventional non-small cell lung cancer with <i>SMARCA4</i> deficiency because of the different clinicopathological characteristics of these two diseases. SMARCA4-UT mainly occurs in young to middle-aged adults and involves a large mass compressing the tissues surrounding the mediastinum and lung parenchyma. Unfortunately, SMARCA4-UT shows a high probability of recurrence after upfront surgery as well as radiotherapy resistance; moreover, chemotherapy has low efficacy. Moreover, given the recent classification of SMARCA4-UT, no data concerning specific clinical trials are currently available. However, several case reports show immunotherapy efficacy in patients with this disease not only in a metastatic setting but also in a neoadjuvant manner, supporting the development of clinical trials. In addition, preclinical data and initial clinical experiences suggest that inhibiting pathways such as CDK4/6, AURKA, ATR, and EZH2 may be a promising therapeutic approach to SMARCA4-UT."],"journal":["International journal of molecular sciences"],"pubmed_title":["Treatment of Thoracic SMARCA4-Deficient Undifferentiated Tumors: Where We Are and Where We Will Go."],"pmcid":["PMC10970296"],"funding_grant_id":["deliberation 187/2023"],"pubmed_authors":["Montagna ES","Galetta D","Pesola F","Nardone A","Perrone A","Montrone M","Gesualdo M","Marech I","Longo V","Catino A","Pizzutilo P"],"additional_accession":[]},"is_claimable":false,"name":"Treatment of Thoracic SMARCA4-Deficient Undifferentiated Tumors: Where We Are and Where We Will Go.","description":"Recently, the fifth edition of the WHO classification recognized the thoracic <i>SMARCA4</i>-deficient undifferentiated tumor (SMARCA4-UT) as a separate entity from conventional non-small cell lung cancer with <i>SMARCA4</i> deficiency because of the different clinicopathological characteristics of these two diseases. SMARCA4-UT mainly occurs in young to middle-aged adults and involves a large mass compressing the tissues surrounding the mediastinum and lung parenchyma. Unfortunately, SMARCA4-UT shows a high probability of recurrence after upfront surgery as well as radiotherapy resistance; moreover, chemotherapy has low efficacy. Moreover, given the recent classification of SMARCA4-UT, no data concerning specific clinical trials are currently available. However, several case reports show immunotherapy efficacy in patients with this disease not only in a metastatic setting but also in a neoadjuvant manner, supporting the development of clinical trials. In addition, preclinical data and initial clinical experiences suggest that inhibiting pathways such as CDK4/6, AURKA, ATR, and EZH2 may be a promising therapeutic approach to SMARCA4-UT.","dates":{"release":"2024-01-01T00:00:00Z","publication":"2024 Mar","modification":"2025-04-21T21:27:28.431Z","creation":"2025-04-05T18:22:12.785Z"},"accession":"S-EPMC10970296","cross_references":{"pubmed":["38542211"],"doi":["10.3390/ijms25063237"]}}