Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2017 Dec;11(4):460-468.
doi: 10.1007/s12105-017-0809-y. Epub 2017 Mar 27.

NUT Midline Carcinoma of the Sublingual Gland: Clinical Presentation and Review

Nolan B Seim  1 , Ramez H W Philips  2 , Lynn Schoenfield  3 , Theodoros N Teknos  1 , James W Rocco  1 , Amit Agrawal  1 , Enver Ozer  1 , Ricardo L Carrau  1 , Stephen Y Kang  1 , Matthew O Old  4   5
Affiliations
Review

NUT Midline Carcinoma of the Sublingual Gland: Clinical Presentation and Review

Nolan B Seim et al. Head Neck Pathol. 2017 Dec.

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.

Keywords: Floor of mouth; Head and neck cancer; Oral cancer; Sublingual salivary gland; Undifferentiated carcinoma.

PubMed Disclaimer

Conflict of interest statement

Conflict of interest

Nolan Seim declares that he has no conflict of interest. Ramez Phillips declares he has no conflict of interest. Lynn Schoenfield declares that she has no conflict of interest. Theodoros Teknos declares that he has no conflict of interest. James Rocco declares that he has no conflict of interest. Amit Agrawal declares that he has no conflict of interest. Enver Ozer declares that he has no conflict of interest. Ricardo Carrau declares that he has no conflict of interest. Stephen Kang declares that he has no conflict of interest. Matthew Old declares that he has no conflict of interest.

Ethical Approval

This article does not contain any studies with human participants or animals performed by any of the authors.

Informed Consent

Not indicated for this study.

Figures

Fig. 1
Fig. 1
Pre-operative CT neck imaging. Axial computed tomography (CT) with contrast showing 3.0 × 2.3 cm right oral cavity mass with numerous ipsilateral enlarged, necrotic submandibular and lateral chain lymph nodes indicating stage IVA disease
Fig. 2
Fig. 2
Pre and post-operative PET/CT imaging. Positron emission tomography (PET/CT) images before and after surgery. Image a shows several ipsilateral hypermetabolic cervical lymph nodes but no metastatic disease with lack of FDG-uptake (bright areas) elsewhere in the body. Image b shows repeat PET/CT images 1 month after surgery revealing a rapid development of regional recurrence and extensive bony and axial skeleton metastatic lesions seen here in the humeral heads, clavicle, thoracic and lumbar spine, pelvis and femurs
Fig. 3
Fig. 3
H&E staining of sublingual gland tumor. Hematoxylin and eosin staining of the primary lesion showing a hypercellular tumor with ovoid, monotonous cells without differentiation at low and high power magnification. Focal squamous differentiation is a common feature as well in 80% of cases
See this image and copyright information in PMC

References

    1. Carvalho AL, Nishimoto IN, Califano JA, Kowalski LP. Trends in incidence and prognosis for head and neck cancer in the United States: a site-specific analysis of the SEER database. Int J Cancer. 2005;114(5):806–816. doi: 10.1002/ijc.20740. - DOI - PubMed
    1. Spiro RH, Armstrong J, Harrison L, Geller NL, Lin S-Y, Strong EW. Carcinoma of major salivary glands. Recent trends. Arch Otolaryngol Head Neck Surg. 1989;115:316–332. doi: 10.1001/archotol.1989.01860270058015. - DOI - PubMed
    1. Lee RJ, Tong EL, Patel R, Satyadev N, Christensen RE. Malignant sublingual gland tumors: demographics, prognostic factors, and treatment outcomes. Oral Surg Oral Med Oral Pathol Oral Radiol. 2016;121(2):180–187. doi: 10.1016/j.oooo.2015.09.019. - DOI - PubMed
    1. Rutt AL, Hawkshaw MJ, Lurie D, Sataloff RT. Salivary gland cancer in patients younger than 30years. Ear Nose Throat J. 2011;90(4):174–184. - PubMed
    1. Andersen LJ, Therkildsen MH, Ockelmann HH, Bentzen JD, Schiødt T, Hansen HS. Malignant epithelial tumors in the minor salivary glands, the submandibular gland, and the sublingual gland. Prognostic factors and treatment results. Cancer. 1991;68(11):2431–2437. doi: 10.1002/1097-0142(19911201)68:11<2431::AID-CNCR2820681118>3.0.CO;2-N. - DOI - PubMed

LinkOut - more resources