title: NUT Midline Carcinoma of the Sublingual Gland: Clinical Presentation and Review.
abstract: NUT midline carcinoma (NMC) is a rare and aggressive disease encountered in the midline of the head and neck or mediastinum. Due to its sparse incidence and subtle pathologic features, we aim to increase knowledge and awareness for this pathologic entity. We present an exemplary case of a young, healthy male presenting with oral cavity pain and cervical lymphadenopathy. This patient was initially diagnosed with an unspecified, highly aggressive sublingual gland malignancy and underwent locoregional resection with free flap reconstruction however suffered a rapid local recurrence and widely extensive metastasis within just 1 month. After rigorous analysis, final pathologic diagnosis revealed a poorly differentiated carcinoma with evidence of squamous differentiation that eventually, post-mortem tested positive for NMC. Only one prior case of sublingual gland NMC has been previously reported as we discuss the literature regarding all sublingual gland malignancies as well as the pathologic features and treatment options for NMC. We recommend consideration of testing for the NUT proto-oncogene at the time of biopsy in the clinical setting of a poorly differentiated midline carcinoma, especially with squamous differentiation, of the head or neck in order to identify patients for clinical trial enrollment and appropriately counsel on the poor clinical prognosis. Improving clinician awareness is critical to increase diagnostic accuracy and need to study prospective treatment outcomes as the first step toward improving management of this difficult disease.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Arthur G. James Cancer Hospital and Richard J. Solove Research Institute, The Ohio State University Wexner Medical Center, 4000 Eye and Ear Institute, 915 Olentangy River Road, Columbus, OH, 43212, USA.
authors.forename: Nolan B
authors.initials: NB
authors.lastname: Seim
authors.affiliation: The Ohio State University College of Medicine, 370 W. 9th Ave, Columbus, OH, 43210, USA.
authors.forename: Ramez H W
authors.initials: RHW
authors.lastname: Philips
authors.affiliation: Department of Pathology, The Ohio State University Wexner Medical Center, Doan Hall, N337B, 410 West 10th Ave., Columbus, OH, 43210, USA.
authors.forename: Lynn
authors.initials: L
authors.lastname: Schoenfield
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Arthur G. James Cancer Hospital and Richard J. Solove Research Institute, The Ohio State University Wexner Medical Center, 4000 Eye and Ear Institute, 915 Olentangy River Road, Columbus, OH, 43212, USA.
authors.forename: Theodoros N
authors.initials: TN
authors.lastname: Teknos
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Arthur G. James Cancer Hospital and Richard J. Solove Research Institute, The Ohio State University Wexner Medical Center, 4000 Eye and Ear Institute, 915 Olentangy River Road, Columbus, OH, 43212, USA.
authors.forename: James W
authors.initials: JW
authors.lastname: Rocco
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Arthur G. James Cancer Hospital and Richard J. Solove Research Institute, The Ohio State University Wexner Medical Center, 4000 Eye and Ear Institute, 915 Olentangy River Road, Columbus, OH, 43212, USA.
authors.forename: Amit
authors.initials: A
authors.lastname: Agrawal
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Arthur G. James Cancer Hospital and Richard J. Solove Research Institute, The Ohio State University Wexner Medical Center, 4000 Eye and Ear Institute, 915 Olentangy River Road, Columbus, OH, 43212, USA.
authors.forename: Enver
authors.initials: E
authors.lastname: Ozer
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Arthur G. James Cancer Hospital and Richard J. Solove Research Institute, The Ohio State University Wexner Medical Center, 4000 Eye and Ear Institute, 915 Olentangy River Road, Columbus, OH, 43212, USA.
authors.forename: Ricardo L
authors.initials: RL
authors.lastname: Carrau
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Arthur G. James Cancer Hospital and Richard J. Solove Research Institute, The Ohio State University Wexner Medical Center, 4000 Eye and Ear Institute, 915 Olentangy River Road, Columbus, OH, 43212, USA.
authors.forename: Stephen Y
authors.initials: SY
authors.lastname: Kang
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Arthur G. James Cancer Hospital and Richard J. Solove Research Institute, The Ohio State University Wexner Medical Center, 4000 Eye and Ear Institute, 915 Olentangy River Road, Columbus, OH, 43212, USA. matthew.old@osumc.edu.|Department of Otolaryngology-Head and Neck Surgery, The James Cancer Hospital and Solove Research Institute, Wexner Medical Center, The Ohio State University, Starling Loving Hall, B217, 320 West 10th Ave, Columbus, OH, 43210, USA. matthew.old@osumc.edu.
authors.forename: Matthew O
authors.initials: MO
authors.lastname: Old
mesh_terms: D000328:Adult; D002277:Carcinoma / Q000473:pathology*; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D000090063:Proto-Oncogene Mas; D013362:Sublingual Gland Neoplasms / Q000473:pathology*
keywords: Floor of mouth; Head and neck cancer; Oral cancer; Sublingual salivary gland; Undifferentiated carcinoma