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SMARCB1 (INI-1)-Deficient Sinonasal Carcinoma: A Systematic Review and Pooled Analysis of Treatment Outcomes
Victor Ho-Fun Lee1,2, Raymond King-Yin Tsang3,4, Anthony Wing Ip Lo5
1Department of Clinical Oncology, School of Clinical Medicine, LKS Faculty of Medicine, The University of Hong Kong, Hong Kong, China.
Abstract:
(1) Background: SMARCB1 (INI-1)-deficient sinonasal carcinoma is a rare sinonasal malignancy; since its discovery and description in 2014, less than 200 cases have been identified. It is almost impossible to perform randomized-controlled trials on novel therapy to improve treatment outcomes in view of its rarity. We performed a systematic review of all the published case reports/series and included our patients for survival analysis. (2) Methods: In this systematic review, we searched from PubMed-MEDLINE, EMBASE, Scopus, Cochrane Library, CINAHL, and Google Scholar for individual patient data to identify and retrieve all reported SMARCB1-deficient sinonasal carcinoma. Clarification on treatment details and the most updated survival outcomes from all authors of the published case reports/series were attempted. Survival analysis for overall survival (OS) and identification of OS prognostic factors were performed. This systematic review was registered with PROSPERO (CRD42022306671). (3) Results: A total of 67 publications were identified from the systematic review and literature search. After excluding other ineligible and duplicated publications, 192 patients reported were considered appropriate for further review. After excluding duplicates and patients with incomplete pretreatment details and survival outcomes, 120 patients were identified to have a complete set of data including baseline demographics, treatment details, and survival outcomes. Together with 8 patients treated in our institution, 128 patients were included into survival analysis. After a median follow up of 17.5 months (range 0.3-149.0), 50 (46.3%) patients died. The 1-year, 2-year and 3-year OS rates were 84.3% (95% CI % 77.6-91.0), 62.9% (95% CI 53.1-72.7), and 51.8% (95% CI 40.8-62.8), respectively, and the median OS was 39.0 months (95% CI 28.5-49.5). Males (p = 0.029) and T4b disease (p = 0.013) were significant OS prognostic factors in univariable analysis, while only T4b disease (p = 0.017) remained significant in multivariable analysis. (4) Conclusions: SMARCB1-deficient sinonasal carcinoma is an extremely aggressive sinonasal malignancy with a dismal prognosis. Early diagnosis and a multimodality treatment strategy are essential for a better treatment and survival outcome.
Insights
SMARCB1-deficient sinonasal carcinoma is a rare and aggressive cancer. Early diagnosis and multimodal treatment are crucial for improving survival outcomes in patients with this malignancy.
Area of Science:
- Oncology
- Pathology
- Medical Research
Background:
- SMARCB1 (INI-1)-deficient sinonasal carcinoma is an exceptionally rare malignancy, with fewer than 200 cases identified since 2014.
- The rarity of this cancer makes randomized-controlled trials for novel therapies challenging.
- This study addresses the need for survival data through a systematic review and survival analysis.
Purpose of the Study:
- To conduct a systematic review of all published case reports and series of SMARCB1-deficient sinonasal carcinoma.
- To perform survival analysis on a combined cohort of patients from the literature and a single institution.
- To identify prognostic factors influencing overall survival (OS) in this rare cancer.
Main Methods:
- A comprehensive systematic review was performed using multiple databases (PubMed-MEDLINE, EMBASE, Scopus, Cochrane Library, CINAHL, Google Scholar).
- Individual patient data was extracted, and authors were contacted for treatment details and updated survival outcomes.
- Survival analysis, including overall survival (OS) and prognostic factor identification, was conducted on 128 patients (120 from literature, 8 from the institution).
Main Results:
- A total of 128 patients were included in the survival analysis, with a median follow-up of 17.5 months.
- The 1-year, 2-year, and 3-year OS rates were 84.3%, 62.9%, and 51.8%, respectively, with a median OS of 39.0 months.
- T4b disease was identified as a significant negative prognostic factor for OS in both univariable and multivariable analyses.
Conclusions:
- SMARCB1-deficient sinonasal carcinoma is characterized by aggressive behavior and a poor prognosis.
- Early diagnosis is critical for effective management.
- A multimodality treatment approach is essential to improve patient outcomes and survival.
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