Abstract
Biphenotypic sinonasal sarcoma (BSNS) is a rare malignant tumour of the upper nasal cavity and ethmoid sinuses that presents predominantly in middle aged female patients and show a characteristic infiltrative and hypercellular proliferation of spindle cells that demonstrate a specific immunoreactivity. We present three cases with BSNS that had different presenting complaints, either sinonasal or orbital problems, underwent endoscopic surgical treatment and/or radiotherapy and have been disease free on long follow up. A systematic review of all published cases was performed to identify all BSNS cases known at present. BSNS requires prompt and correct diagnosis with accurate surgical resection as well as consideration of radiotherapy. Our three cases confirm the findings of the literature and support that BSNS is an aggressive but treatable malignant disease of the sinonasal tract.