Case Report
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
Rosibel Rojas, Marco Pocci1, Renato Covello2, Marina Pacheco
Pathology Department, Complejo Hospitalario Metropolitano C. S. S, Panama, Florida, USA, 1 Department Anatomic Pathology, University of Rome Tor Vergata, 2 Istituto Nazionale Tumori Regina Elena, Rome, Italy
Address for correspondence:
Dr. Marina Pacheco, Pathology Department, Complejo Hospitalario Metropolitano C.S.S, Panama, Florida, USA.
E‐mail: marina.pacheco.rovira@gmail.com
Abstract
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.
Keywords: Biphenotypic, PAX3-MAML3, sarcoma, sinonasal
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
How to cite this article: Rojas R, Pocci M, Covello R, Pacheco M. Biphenotypic sinonasal sarcoma: An infrequent neoplasm with classical clinical presentation and mimetic histology. Report of two cases. Int J Head Neck Pathol 2019;2:5-8.
