Biphenotypic Sinonasal Sarcoma

Biphenotypic sinonasal sarcoma is a newly recognized, very rare, low grade malignant tumor of the nasal cavity which formerly was probably included in fibrosarcoma and synovial sarcoma cases. It is newly recognized by the World Health Organization Classification of Head and Neck Tumours.

Abbreviations

Definitions

The tumor will often affect multiple sites within the upper aerodigestive tract, frequently showing extension into the orbit (25%) or through the cribriform plate (11%) of the ethmoid sinus into the skull base. The patient present with non-specific findings, including difficulty breathing, facial pressure, congestion, pain, and mild epiphora. Often concurrent benign sinonasal tract inflammatory polyps will be seen.

Genetic Testing

A very rare tumor, although as it is newly described, probably is under reported. Patients present over a wide age range (24–85 years), with a mean age of 52 years. There is a female predominance (F:M 3:1).

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