Synapse - Outcomes of SMARCB1-deficient sinonasal carcinoma: Largest single-center cross-sectional study

Outcomes of SMARCB1-deficient sinonasal carcinoma: Largest single-center cross-sectional study Journal Article

Authors:	Woods, R.; Scholfield, D.; Axiotakis, L.; Fitzgerald, C.; Adilbay, D.; Cracchiolo, J.; Patel, S.; Shah, J.; Dunn, L.; Pfister, D.; Lee, N.; Dogan, S.; Ganly, I.; Cohen, M.
Article Title:	Outcomes of SMARCB1-deficient sinonasal carcinoma: Largest single-center cross-sectional study
Abstract:	Background: We evaluate outcomes of SMARCB1-deficient sinonasal carcinomas in the largest single-institution study. Methods: Retrospective cross-sectional study of patients with SMARCB1-deficient sinonasal carcinoma between 1998 and 2024. Disease-specific survival (DSS) and recurrence-free probability (RFP) at 1 and 5 years were measured by Kaplan–Meier method. Results: There were 47 patients with a median age of 53. Initial pathological diagnosis was altered in 33%. Twelve (34%) patients received neoadjuvant chemotherapy, with one partial response. Curative surgical approach was undertaken in 73%. Definitive chemoradiation was administered in 20%. DSS at 1 and 5 years was 93% and 45%, respectively. RFP at 1 and 5 years was 73% and 33%, respectively. On multivariate analysis, cranial nerve involvement (p = 0.01 for DSS) remained significantly worse for DSS and overall survival. Conclusions: SMARCB1-deficient tumors had limited response to neoadjuvant chemotherapy. Cranial nerve involvement was associated with worse prognosis. Optimal treatment is unclear. Surgery should be offered to patients with resectable disease. © 2024 Wiley Periodicals LLC.
Keywords:	adult; clinical article; treatment outcome; treatment response; aged; disease-free survival; middle aged; retrospective studies; overall survival; genetics; mortality; cisplatin; disease free survival; neoadjuvant therapy; etoposide; pathology; surgical approach; retrospective study; cancer specific survival; carcinoma; cross-sectional study; cross-sectional studies; skull base; kaplan meier method; neoadjuvant chemotherapy; paranasal sinus neoplasms; therapy; cranial neuropathy; chemoradiotherapy; induction chemotherapy; paranasal sinus tumor; kaplan-meier estimate; clinical outcome; sinonasal carcinoma; ini-1; smarcb1 protein, human; humans; prognosis; human; male; female; article; smarcb1 protein; tazemetostat; swi/snf related matrix associated actin dependent regulator of chromatin subfamily b member 1; smarcb-1
Journal Title:	Head & Neck
Volume:	46
Issue:	12
ISSN:	1043-3074
Publisher:	John Wiley & Sons, Inc.
Date Published:	2024-12-01
Start Page:	3076
End Page:	3084
Language:	English
DOI:	10.1002/hed.27859
PUBMED:	39044555
PROVIDER:	scopus
DOI/URL:	https://www.scopus.com/inward/record.uri?eid=2-s2.0-85199356799&doi=10.1002%2fhed.27859&partnerID=40&md5=f0dbb3bf1d56654df35f83b153de5625
Notes:	The MSK Cancer Center Support Grant (P30 CA008748) is acknowledged in the PubMed record and PDF. Corresponding MSK authors are Marc Cohen and Ian Ganly -- Source: Scopus