Tazemetostat for the treatment of INI-1-deficient sinonasal tumor

Sinonasal tumors (SNTs) are a group of uncommon heterogeneous malignancies accounting for less than 5% of all head and neck tumors [ [1] Turner J.H. Reh D.D. Incidence and survival in patients with sinonasal cancer: a historical analysis of population-based data. Head Neck. 2012; 34: 877-885https://doi.org/10.1002/hed.21830 Crossref PubMed Scopus (342) Google Scholar ]. Squamous cell carcinoma and adenocarcinoma (intestinal-type adenocarcinoma (ITAC), salivary type, non-ITAC/non-salivary) are the most common histological subtypes of SNTs. However, small round blue cell tumors that include a lot of poorly differentiated and aggressive SNTs and notably neuroendocrine carcinomas, melanomas, sarcomas, NUT carcinoma, and SNUC remain challenging cases because of their aggressive behavior and a low number of validated therapeutic strategies [ [2] Stelow E.B. Bishop J.A. Update from the 4th edition of the world health organization classification of head and neck tumours: tumors of the nasal cavity, paranasal sinuses and skull base. Head Neck Pathol. 2017; 11: 3-15https://doi.org/10.1007/s12105-017-0791-4 Crossref PubMed Scopus (60) Google Scholar ].