Abstract:SMARCB1 (INI-1)-deficient sinonasal carcinoma (SDSC) is a rare and highly aggressive malignancy of the nasal cavity and paranasal sinuses. Immunohistochemical staining to detect the loss of INI-1 protein expression in the nucleus is the most valuable method for the diagnosis of SDSC. Due to the non-specific clinical manifestations, most patients are diagnosed with the disease at an advanced stage, which greatly reduces the survival rate and quality of life of patients. The main treatment means in clinical work are the comprehensive treatment mode of complete surgical resection of the tumor, postoperative adjuvant radiotherapy, an chemotherapy. However, there is still a high recurrence rate and mortality. The effectiveness of neoadjuvant therapy and targeted therapy for this disease is still in clinical trials. Therefore, the early diagnosis of the disease and the exploration of the best treatment strategies are critical for patients. This paper reviews the progress in the diagnosis and treatment of SDSC, which is helpful to improve the clinical cognition of this rare tumor.
