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NUT Carcinoma of the Submandibular Gland: A Case at This Uncommon Site with Review of the Literature

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Abstract

Nuclear protein in testis (NUT) carcinoma is a rare, highly aggressive, undifferentiated carcinoma that harbors a characteristic rearrangement of the NUTM1 gene. The majority arise in adolescents and young adults especially from the midline structures of the thorax, head, and neck. Until the present, there have only been three reported cases of NUT carcinoma of the submandibular gland, two of which were reported in children and another one in an adult from Korea. Here, we report the first case of NUT carcinoma arising in the submandibular gland of an adult female in the United States, representing the fourth case worldwide. A fine needle aspiration and biopsy was performed, and the diagnosis was confirmed by NUT immunohistochemical staining and fusion of the BRD4 (19p13.12) and NUTM1 (15q14) gene loci by fluorescence in-situ hybridization on the resection specimen. Salivary gland is an unusual site for NUT carcinoma and is rarely described in submandibular gland. We reviewed the clinicopathologic features of this entity at this site along with role of NUTM1 gene rearrangements in NUT tumorigenesis.

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All authors contributed to the material preparation, data collection, and analysis of the case report. Literature review and analysis were performed by VM and KS. The first draft of the manuscript was written by VM and KS. All authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

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Correspondence to Karan Saluja.

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Moreno, V., French, C.A., Guo, T. et al. NUT Carcinoma of the Submandibular Gland: A Case at This Uncommon Site with Review of the Literature. Head and Neck Pathol 16, 881–892 (2022). https://doi.org/10.1007/s12105-021-01400-x

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