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Updated: Apr 29, 2026

Identification of OTX1 and OTX2 As Two Possible Molecular Markers for Sinonasal Carcinomas and Olfactory Neuroblastomas
Published on: February 28, 2019
Recurrent PAX3-MAML3 fusion in biphenotypic sinonasal sarcoma
Xiaoke Wang1, Krista L Bledsoe2, Rondell P Graham1
11] Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA. [2].
Abstract:
Biphenotypic sinonasal sarcoma (SNS) is a newly described tumor of the nasal and paranasal areas. Here we report a recurrent chromosomal translocation in SNS, t(2;4)(q35;q31.1), resulting in a PAX3-MAML3 fusion protein that is a potent transcriptional activator of PAX3 response elements. The SNS phenotype is characterized by aberrant expression of genes involved in neuroectodermal and myogenic differentiation, closely simulating the developmental roles of PAX3.
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