Background: Biphenotypic Sinonasal Sarcoma (BSNS) is a recently described tumor with
its site of origin limited to the nose and paranasal sinus. It is a rare low-grade sarcoma
characterized by concomitant neural and myogenic differentiation with high recurrence rates
but lacks metastatic potential. Limited literature is available about the standard treatment
regimens due to their rarity.
Case Presentation: Herein we describe the details of the case of a 55-year-old male,
known case of recurrent biphenotypic sinonasal Sarcoma who presented with bilateral nasal
obstruction. The diagnosis was confirmed by immunohistochemistry. He underwent surgery
but developed recurrence twice, and later treated with Radiation Therapy.
Conclusions: Mainstay of the treatment remains surgery but due to its rarity, no definite
treatment guidelines are defined. Radica or adjuvant treatment with chemotherapy and/or
radiotherapy have been tried with limited data on the outcome.