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.

Classification

Abbreviations

Definitions

Signs and symptoms

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.

Pathology findings

Microscopic

Hematoxylin and eosin stained image of a biphenotypic sinonasal sarcoma.

Ancillary tests

Immunohistochemistry

Genetic Testing

Differential diagnoses

Epidemiology

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).

Management

References

  1. 1 2 Lewis JT, Oliveira AM, Nascimento AG, Schembri-Wismayer D, Moore EA, Olsen KD, Garcia JG, Lonzo ML, Lewis JE (April 2012). "Low-grade sinonasal sarcoma with neural and myogenic features: a clinicopathologic analysis of 28 cases". Am J Surg Pathol. 36 (4): 517–25. PMID 22301502. doi:10.1097/PAS.0b013e3182426886.
  2. Wang X, Bledsoe KL, Graham RP, Asmann YW, Viswanatha DS, Lewis JE, Lewis JT, Chou MM, Yaszemski MJ, Jen J, Westendorf JJ, Oliveira AM (July 2014). "Recurrent PAX3-MAML3 fusion in biphenotypic sinonasal sarcoma". Nat Genet. 46 (7): 666–8. PMC 4236026Freely accessible. PMID 24859338. doi:10.1038/ng.2989.
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