Sinonasal biphenotypic sarcoma has recently been described and included in the 2017 WHO classification. It is a low-grade sarcoma which is characterized by PAX3 rearrangements. It remains a diagnostic challenge because of its scarcity, and its considerable histologic overlap with other cellular spindle cell neoplasms. The histologic features, the immunoprofile and the main differential diagnoses of sinonasal biphenotypic sarcoma are presented through a case.
Keywords: Fosse nasale; Nasal cavity; PAX3; Pax3; Sarcoma; Sarcome; Sarcome nasosinusien biphénotypique; Sinonasal biphenotypic sarcoma.
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