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Unilateral Nasal Mass in a Woman in Her 20s

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 29-year-old healthy woman presented with a 2-month history of left-sided epiphora, headache, and facial pressure. A polypoid lesion was identified at the left lateral nasal wall. Noncontrasted computed tomography showed a hyperdense mass involving the left medial canthus extending through the nasolacrimal duct into the left nasal cavity. The patient underwent endoscopic left maxillary antrostomy, ethmoidectomy, and biopsy of mass, with results of pathology analysis showing poorly differentiated squamous cell carcinoma. Immunohistochemical (IHC) staining was positive for cytokeratin 5/6 and p63, and negative for Epstein-Barr virus, p16, CD45 (leukocyte common antigen), and synaptophysin. The patient was referred to a tertiary care facility for further treatment. On examination, there was a palpable subcutaneous nodule 1 cm inferior to the left medial canthus with normal overlying skin. On nasal endoscopy, residual tumor was visible at the anterior aspect of the lateral nasal wall. Computed tomography showed postoperative changes with enhancement of left medial canthus extending into nasolacrimal duct and along the lateral nasal sidewall (Figure, A).

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C. NUT carcinoma

NUT carcinoma (NC) is an aggressive poorly differentiated carcinoma that is characterized by a specific genetic rearrangement involving the nuclear protein in testis (NUT) gene.1 This is a relatively newly recognized pathologic entity first described in 19912 and is defined by its genetic alteration, in contrast to the complex and multiple genetic rearrangements found in typical squamous cell carcinoma.3

NUT carcinoma was initially thought to arise predominantly in children; however, this is likely from selection bias because pediatric tumors are more likely to undergo cytogenetic testing. Within the past decade, cases have been reported in patients ages 3 to 78 years, with 1 case series showing a mean age of 54 years.2,4 The majority of tumors arise in the mediastinum or head and neck, with head and neck sites representing roughly 45% of cases.4 Within the head and neck, tumors have been reported to involve the paranasal sinuses and nasal cavity, nasopharynx, oropharynx, hypopharynx, larynx, orbit, and salivary glands, and the majority of patients present with locally advanced or metastatic disease at diagnosis.4,5 There is a slight female predilection of 1.5:1.5

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Article Information

Corresponding Author: Kendall K. Tasche, MD, Department of Otolaryngology–Head & Neck Surgery, University of Iowa Hospitals & Clinics, 200 Hawkins Dr, 21200 PFP, Iowa City, IA 52242 (kendall-tasche@uiowa.edu).

Published Online: July 11, 2019. doi:10.1001/jamaoto.2019.1671

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Tumours of the nasal cavity, paranasal sinuses, and skull base. In: El-Naggar  AK, Chan  JKC, Grandis  JR, Takata  T, Slootweg  PJ, eds.  WHO Classification of Tumours of the Head and Neck. 4th ed. Lyon, France: IARC Press; 2017:20-21.
2.
French  CA.  NUT midline carcinoma.  Cancer Genet Cytogenet. 2010;203(1):16-20. doi:10.1016/j.cancergencyto.2010.06.007PubMedGoogle ScholarCrossref
3.
French  CA.  The importance of diagnosing NUT midline carcinoma.  Head Neck Pathol. 2013;7(1):11-16. doi:10.1007/s12105-013-0428-1PubMedGoogle ScholarCrossref
4.
Stelow  EB, Bellizzi  AM, Taneja  K,  et al.  NUT rearrangement in undifferentiated carcinomas of the upper aerodigestive tract.  Am J Surg Pathol. 2008;32(6):828-834. doi:10.1097/PAS.0b013e31815a3900PubMedGoogle ScholarCrossref
5.
Chau  NG, Hurwitz  S, Mitchell  CM,  et al.  Intensive treatment and survival outcomes in NUT midline carcinoma of the head and neck.  Cancer. 2016;122(23):3632-3640. doi:10.1002/cncr.30242PubMedGoogle ScholarCrossref
6.
Bauer  DE, Mitchell  CM, Strait  KM,  et al.  Clinicopathologic features and long-term outcomes of NUT midline carcinoma.  Clin Cancer Res. 2012;18(20):5773-5779. doi:10.1158/1078-0432.CCR-12-1153PubMedGoogle ScholarCrossref
7.
Stelow  EB.  A review of NUT midline carcinoma.  Head Neck Pathol. 2011;5(1):31-35. doi:10.1007/s12105-010-0235-xPubMedGoogle ScholarCrossref
8.
Stathis  A, Zucca  E, Bekradda  M,  et al.  Clinical response of carcinomas harboring the BRD4-NUT oncoprotein to the targeted bromodomain inhibitor OTX015/MK-8628.  Cancer Discov. 2016;6(5):492-500. doi:10.1158/2159-8290.CD-15-1335PubMedGoogle ScholarCrossref
9.
Bishop  JA, Westra  WH.  NUT midline carcinomas of the sinonasal tract.  Am J Surg Pathol. 2012;36(8):1216-1221. doi:10.1097/PAS.0b013e318254ce54PubMedGoogle ScholarCrossref
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