Abstract
Abstract
Purpose of Review
An emerging subset of dismal sinonasal cancers are those characterized by the loss of a SWItch/Sucrose Non-Fermentable (SWI/SNF) complex unit, such as the SWI/SNF-related Matrix-associated Actin-dependent Regulator of Chromatin (SMARC), which includes two main subtypes: SMARCB1- and SMARCA4-deficient sinonasal carcinomas, ultimately leading to four distinct SWI/SNF-deficient sinonasal tumors. These cancers are rare entities and low treatment responsive malignancies. In fact, they are poorly differentiated and usually detected at a late stage, when invasion of facial and cranial regions had already occurred.
Recent Findings
From a histological standpoint, SWI/SNF-deficient sinonasal carcinomas belong to the group of sinonasal undifferentiated carcinomas (SNUC); however, their distinctive features disclose a special category for these cancers. The identification of biomarkers and signaling pathways has led to the development of emerging therapies, such as immunotherapy and personalized treatments. Finally, we report preliminary findings on 3D in vitro models of sinonasal cancers, as a multidisciplinary tool that could empower the understanding of SWI/SNF-deficient cancer biology.
Summary
Here, we review the current knowledge about histological and molecular features of SWI/SNF-deficient sinonasal cancers, with a focus on treatment options and multidisciplinary research perspectives. The possibility of studying SWI/SNF-deficient sinonasal tumors in-depth would be fostered by the establishment of tumor cell lines.
Publisher
Springer Science and Business Media LLC
Subject
Neurology (clinical),Otorhinolaryngology,Immunology and Allergy,Surgery