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Year : 2019  |  Volume : 2  |  Issue : 1  |  Page : 5-8

Biphenotypic sinonasal sarcoma: An infrequent neoplasm with classical clinical presentation and mimetic histology. Report of two cases

1 Pathology Department, Complejo Hospitalario Metropolitano C. S. S, Panama, Florida, USA
2 Department Anatomic Pathology, University of Rome Tor Vergata, Rome, Italy
3 Istituto Nazionale Tumori Regina Elena, Rome, Italy

Correspondence Address:
Marina Pacheco
Pathology Department, Complejo Hospitalario Metropolitano C.S.S, Panama, Florida
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JHNP.JHNP_2_20

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Biphenotypic sinonasal sarcoma (BSNS) is a rare low-grade sarcoma that occurs mainly in the upper sinonasal tract of middle-aged women. It exhibits a dual immunophenotype characterized by the expression of neural and myogenic markers. It is characterized by PAX3 rearrangement with various fusion partners, being MAML3 the most frequent. BSNS is a locally aggressive neoplasm, with frequent local recurrences but no metastasis reported. Here, we present the clinical, histological, immunohistochemical, and molecular findings of two new cases of BSNS that fulfill the canonical demographic, clinical, morphologic, and molecular criteria described for this infrequent entity.

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