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

Abstract

AbstractBackgroundWe evaluate outcomes of SMARCB1‐deficient sinonasal carcinomas in the largest single‐institution study.MethodsRetrospective 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.ResultsThere 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.ConclusionsSMARCB1‐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.