Definition
Biphenotypic sinonasal sarcoma (BSNS) represents a locally aggressive spindle cell sarcoma, characterized by myogenic and neural differentiation, occurring in the sinonasal region of adult patients (Lewis et al. 2012).
Clinical Features
Incidence
Extremely rare tumor with about 100 cases reported in literature until now (Le Loarer et al. 2019).
Age
The tumor affects typically middle-aged adults (Lewis et al. 2012; Le Loarer et al. 2019).
Sex
Female predilection is observed (Lewis et al. 2012; Le Loarer et al. 2019).
Site
This tumor involves exclusively the nasal cavity and/or paranasal sinuses (Lewis et al. 2012; Le Loarer et al. 2019).
Treatment
The treatment of choice is surgical resection associated with radio- or chemotherapy in rare cases (Lewis et al. 2012; Le Loarer et al. 2019).
Outcome
Typically, the tumor exhibits infiltrative growth with destruction of the sinonasal bone. Local recurrences are frequently observed. Distant metastases have never been reported (Lewis...
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References and Further Reading
Fritchie, K. J., Jin, L., Wang, X., et al. (2016). Fusion gene profile of biphenotypic sinonasal sarcoma: an analysis of 44 cases. Histopathology, 69, 930–936.
Huang, S.-C., Ghossein, R. A., Bishop, J. A., et al. (2016). Novel PAX3-NCOA1 fusions in biphenotypic sinonasal sarcoma with focal rhabdomyoblastic differentiation. Am J Surg Pathol, 40, 51–59.
Jo, V. Y., Mariño-Enriquez, A., Fletcher, C. D. M., et al. (2018). Expression of PAX3 distinguishes biphenotypic sinonasal sarcoma from histologic mimics. Am J Surg Pathol, 42, 1275–1285.
Le Loarer, F., Laffont, S., Leshuyes, T., et al. (2019). Clinicopathologic and molecular features of a series of 41 biphenotypic sinonasal sarcomas expanding their molecular spectrum. Am J Surg Pathol, 43, 747–754.
Lewis, J. T., Oliveira, A. M., Nascimento, A. G., et al. (2012). Low-grade sinonasal sarcoma with neural and myogenic features: a clinicopathologic analysis of 28 cases. Am J Surg Pathol, 36, 517–525.
Rooper, L. M., Huang, S.-C., Antonescu, C. R., et al. (2016). Biphenotypic sinonasal sarcoma: an expanded immunoprofile including consistent nuclear β-catenin positivity and absence of SOX10 expression. Hum Pathol, 55, 44–50.
Wang, X., Bledsoe, K. L., Graham, R. P., et al. (2014). Recurrent PAX3-MAML3 fusion in biphenotypic sinonasal sarcoma. Nat Genet, 46, 666–668.
Wong, W. J., Lauria, A., Hornick, J. L., et al. (2016). Alternate PAX3-FOXO1 oncogenic fusion in biphenotypic sinonasal sarcoma. Genes Chromosom Cancer, 55, 25–29.
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Sbaraglia, M. (2020). Biphenotypic Sinonasal Sarcoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5357-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5357-1
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