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Treatment for SMARCB1 (INI-1) deficient sinonasal tumor: a single-institution study
Tian Wang1, Jie Wang1, Tianci Tang1
1Department of Radiation Oncology, Eye and ENT Hospital, Fudan University, Shanghai, China.
Abstract:
Currently, less than 200 cases of SMARCB1-deficient sinus cancer (SDSC) have been documented. Little information is available about the best treatment options or prognosis for SDSC. From September 2016 to November 2022, the medical records of 22 people with SDSC were evaluated retrospectively. Patient demographics, staging, pathology findings, treatment details, recurrence, metastasis, and survival outcomes were all investigated by the researchers. The 1-, 2-, and 3-year overall survival (OS) rates for the entire cohort were 89.8%, 84.2%, and 45.1%, respectively, as were the 1-, 2-, and 3-year progression-free survival (PFS) rates of 81.8%, 63.8%, and 31.9%. After induction chemotherapy, 66.7% (10/15) of patients exhibited decreased tumor volume. Patients who accepted chemoradiotherapy had a better 2-year OS (100% vs. 72.7%, p=0.048) than those who accepted surgery as a preference. However, there is no difference in 2-year PFS between the two groups (53.0% vs. 75.8%, p=0.59). Patients with progressed or stable disease after induction chemotherapy had a higher risk of developing local recurrence (p=0.007); they also showed poor 2-year PFS (40.0% vs. 82.1%, p=0.019). SDSC had a poor 3-year OS, with a PFS of less than 50%. For locally advanced SDSC, chemoradiotherapy might be managed before surgery, especially in patients who benefit from induction chemotherapy.
Insights
SMARCB1-deficient sinus cancer (SDSC) is rare, with limited treatment data. Chemoradiotherapy may improve survival for advanced SDSC, especially after induction chemotherapy shows tumor reduction.
Area of Science:
- Oncology
- Genetics
- Cancer Research
Background:
- SMARCB1-deficient sinus cancer (SDSC) is a rare malignancy with fewer than 200 documented cases.
- Limited data exists regarding optimal treatment strategies and prognosis for SDSC.
Purpose of the Study:
- To retrospectively evaluate patient demographics, staging, pathology, treatment, recurrence, metastasis, and survival outcomes in SDSC.
- To identify potential prognostic factors and optimal treatment approaches for SDSC.
Main Methods:
- Retrospective analysis of medical records from 22 patients diagnosed with SDSC between September 2016 and November 2022.
- Investigation of patient demographics, tumor staging, histopathological findings, treatment modalities (chemotherapy, chemoradiotherapy, surgery), and survival data (overall survival and progression-free survival).
Main Results:
- 1-, 2-, and 3-year overall survival (OS) rates were 89.8%, 84.2%, and 45.1%, respectively. 1-, 2-, and 3-year progression-free survival (PFS) rates were 81.8%, 63.8%, and 31.9%, respectively.
- Induction chemotherapy led to tumor volume reduction in 66.7% of patients.
- Chemoradiotherapy was associated with improved 2-year OS (100%) compared to surgery (72.7%), though PFS showed no significant difference.
- Progressed or stable disease post-induction chemotherapy correlated with higher local recurrence risk and poorer 2-year PFS.
Conclusions:
- SDSC has a poor 3-year OS and PFS (<50%).
- For locally advanced SDSC, neoadjuvant chemoradiotherapy may be a preferred strategy, particularly for patients responding to induction chemotherapy.
- Further research is warranted to establish definitive treatment guidelines for SDSC.
