AUTHOR=Vuong Huy Gia , Le Thoa , Le Trang T.B. , Le Hieu Trong , El-Rassi Edward T. , McKinney Kibwei A. , Dunn Ian F. 

TITLE=Clinicopathological features and prognostic outcomes of molecularly defined entities in the new edition of the WHO classification of sinonasal carcinoma

JOURNAL=Frontiers in Oncology

VOLUME=13

YEAR=2023

URL=https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2023.1117865

DOI=10.3389/fonc.2023.1117865

ISSN=2234-943X

ABSTRACT=<sec><title>Introduction</title><p>We investigated the clinicopathological features and prognoses of the new molecularly defined entities in latest edition of the World Health Organization (WHO) classification of sinonasal carcinoma (SNC)</p></sec><sec><title>Methods</title><p>Integrated data were combined into an individual patient data (IPD) meta-analysis.</p></sec><sec><title>Results</title><p>We included 61 studies with 278 SNCs including 25 <italic>IDH2-</italic>mutant, 41 NUT carcinoma, 187 SWI/SNF loss, and 25 triple negative SNCs (without IDH2 mutation, <italic>NUTM1</italic> rearrangement, and SWI/SNF inactivation) for analyses. Compared to other molecular groups, NUT carcinoma was associated with a younger age at presentation and an inferior disease-specific survival. Among SNCs with SWI/SNF inactivation, <italic>SMARCB1-</italic>deficient tumors presented later in life and were associated with a higher rate of radiotherapy administration. <italic>SMARCA4-</italic>deficiency was mostly found in teratocarcinosarcoma while <italic>SMARCB1-</italic>deficient tumors were associated with undifferentiated carcinoma and non-keratinizing squamous cell carcinoma.</p></sec><sec><title>Conclusion</title><p>Our study facilitates our current understanding of this developing molecular-defined spectrum of tumors and their prognoses.</p></sec>