Abstract

SMARCB1 (INI-1) are rare and locally aggressive group of sinonasal tract malignancy. Less than 200 cases have been reported in the literature. Presentation of this group of tumours is very late with intraorbital and intracranial extensions. There is no definitive treatment protocol for this entity. We present a case of SMARCB1 (INH) - Deficient sinonasal tumour in a 51 year old female. Mass was diagnosed on Contrast enhanced computed tomography (CECT) and diagnostic nasal endoscopy (DNE). Biopsy and debulking was planned under general anesthesia. Intraoperative samples were sent for frozen section sampling and procedure was completed once diagnosis was made. Immunohistochemical staining was done which showed complete loss of INI-1. Patient was furthur referred to Oncologist for Chemotherapy and subsequent Radiotherapy. This condition is quite rare and presentation is also late. So early diagnosis and prompt management improves the treatment outcome and prognosis of the patient.