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NUT Gene Rearrangement in a Poorly-differentiated Carcinoma of the Submandibular Gland

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Abstract

NUT midline carcinomas (NMC) are a rare, recently described class of poorly-differentiated tumors that exhibit rapid onset and highly aggressive clinicopathologic behavior. These tumors are defined by rearrangement of the nuclear protein in testis (NUT) gene on chromosome 15q14, most commonly in a balanced translocation with the BRD4 gene on chromosome 19p13.1, resulting in the characteristic BRD4-NUT fusion gene and protein which blocks epithelial differentiation through chromatin binding. NMC frequently involve midline structures of adolescents and young adults and affect the head and neck region in 50% of cases. To our knowledge, only one case has been previously reported involving a salivary gland. Here, we present a case of a NMC of the salivary gland in an adolescent male presenting with an intermittently painful left submandibular mass of 3 months duration.

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Authors and Affiliations

  1. Department of Pathology, Yale University School of Medicine, 333 Cedar Street, New Haven, CT, 06520, USA

    James Ziai

  2. Department of Pathology, Brigham and Women’s Hospital, 20 Shattuck Street, Boston, MA, 02115, USA

    Chris A. French

  3. Department of Pathology, Medical College of Wisconsin, 9200 W. Wisconsin Ave., Milwaukee, WI, 53226, USA

    Eduardo Zambrano

Authors
  1. James Ziai
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  2. Chris A. French
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  3. Eduardo Zambrano
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Correspondence to Eduardo Zambrano.

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Ziai, J., French, C.A. & Zambrano, E. NUT Gene Rearrangement in a Poorly-differentiated Carcinoma of the Submandibular Gland. Head and Neck Pathol 4, 163–168 (2010). https://doi.org/10.1007/s12105-010-0174-6

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  • DOI: https://doi.org/10.1007/s12105-010-0174-6

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