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Progressive Bilateral Nasal Obstruction Due to Nasopharyngeal Mass

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

A healthy Asian man in his 20s presented with a 5-month history of progressive bilateral nasal obstruction. He also complained of recurrent epistaxis. He had no notable medical history except stable asthma. On physical examination, nasopharyngoscopy showed a pedunculated mass with a smooth mucosal surface arising from the roof of the nasopharynx. Computed tomographic (CT) imaging (Figure, A and B) revealed a midline, well-defined, pedunculated polypoid lesion with areas of calcifications in the nasopharynx, measuring approximately 1.2 × 1.5 × 2.0 cm. An endoscopic resection via retrovelar approach using a 70-degree endoscope combined with transnasal approach was performed under general anesthesia. The mass was completely resected with its stalk and with adequate surgical margin. Histopathological results (Figure, C and D) showed that the tumor cells were arranged in papillary structures with fibrovascular cores and these cells also had nuclear grooves. Results of other investigations after surgery were negative for cancer, including magnetic resonance imaging (MRI) of the neck and thyroid ultrasonography. In addition, no residual tumor or systemic metastasis was observed on positron emission tomography (PET)-CT results. There was no evidence of recurrence at 12-month postoperative follow-up.

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C. Papillary adenocarcinoma

Primary adenocarcinoma originating from the nasopharynx (nasopharyngeal adenocarcinomas [NPACs]) are uncommon neoplasms with a great variety of histologic types and clinical behavior, and as a result, reported cases of NPACs have been limited.1 In particular, nasopharyngeal papillary adenocarcinoma (NPPA) is an extremely rare neoplasm characterized by morphological similarity to papillary thyroid carcinoma because it exhibits papillary growth and abnormal expression of thyroid transcription factor-1 (TTF-1).2 A study by Wenig et al3 first described thyroid-like papillary adenocarcinoma of the nasopharynx in 1988; in 2005, NPPA was included in the World Health Organization classification system of malignant epithelial tumors of the nasopharynx owing to its indolent clinical behavior and low-grade histological features.4 Typically, NPPAs closely resemble papillary thyroid carcinoma; thus, differential diagnosis with the metastasis of papillary thyroid carcinoma is essential.1 Histopathologically, NPPAs exhibit papillary architecture with fibrovascular cores, overlapping nuclei with clear chromatin, psammoma bodies, and prominent nucleoli attached to the nuclear membrane identified by their light microscopic appearance.5

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Article Information

Corresponding Author: Kyung Soo Kim, MD, PhD, Department of Otorhinolaryngology–Head and Neck Surgery, Chung-Ang University College of Medicine, 224-1, Heukseok-dong, Dongjak-gu, Seoul 156-755, South Korea (99-21045@hanmail.net).

Published Online: August 15, 2019. doi:10.1001/jamaoto.2019.2254

Conflict of Interest Disclosures: None reported.

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

References
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2.
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3.
Wenig  BM, Hyams  VJ, Heffner  DK.  Nasopharyngeal papillary adenocarcinoma: a clinicopathologic study of a low-grade carcinoma.  Am J Surg Pathol. 1988;12(12):946-953. doi:10.1097/00000478-198812000-00005PubMedGoogle ScholarCrossref
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Pineda-Daboin  K, Neto  A, Ochoa-Perez  V, Luna  MA.  Nasopharyngeal adenocarcinomas: a clinicopathologic study of 44 cases including immunohistochemical features of 18 papillary phenotypes.  Ann Diagn Pathol. 2006;10(4):215-221. doi:10.1016/j.anndiagpath.2005.11.002PubMedGoogle ScholarCrossref
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Li  M, Wei  J, Yao  X, Wang  C.  Clinicopathological features of low-grade thyroid-like nasopharyngeal papillary adenocarcinoma.  Cancer Res Treat. 2017;49(1):213-218. doi:10.4143/crt.2016.195PubMedGoogle ScholarCrossref
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Sillings  CN, Weathers  DR, Delgaudio  JM.  Thyroid-like papillary adenocarcinoma of the nasopharynx: a case report in a 19-year-old male.  Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2010;110(3):e25-e28. doi:10.1016/j.tripleo.2010.04.029PubMedGoogle ScholarCrossref
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Kawaguchi  M, Kato  H, Tomita  H,  et al.  Imaging characteristics of malignant sinonasal tumors.  J Clin Med. 2017;6(12):116. doi:10.3390/jcm6120116PubMedGoogle ScholarCrossref
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Ollivier  L, Vanel  D, Leclère  J.  Imaging of chondrosarcomas.  Cancer Imaging. 2003;4(1):36-38. doi:10.1102/1470-7330.2003.0022PubMedGoogle ScholarCrossref
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Khoo  ML, Asa  SL, Witterick  IJ, Freeman  JL.  Thyroid calcification and its association with thyroid carcinoma.  Head Neck. 2002;24(7):651-655. doi:10.1002/hed.10115PubMedGoogle ScholarCrossref
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