A Unique Case of Primary EBV-Positive, HPV-Negative Nasopharyngeal Carcinoma Located in the Tonsil
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CASE REPORTS
A Unique Case of Primary EBV‑Positive, HPV‑Negative Nasopharyngeal Carcinoma Located in the Tonsil Sally Nguyen1,2 · Timothy Kong2,3 · Eric Berthelet2,3 · Tony Ng2,4 · Eitan Prisman1,2 Received: 21 July 2020 / Accepted: 10 October 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Nasopharyngeal carcinomas (NPC) are non-keratinizing squamous cell carcinomas of the nasopharynx associated with Epstein-Barr virus (EBV). When occurring outside of the nasopharynx, they are referred to as lymphoepithelioma-like carcinomas (LELCs) and present the same morphology as NPC. LELC have been described in other head and neck regions such as the salivary glands and the soft palate. LELC can also occur in the oropharynx, are associated with human papillomavirus (HPV) and are typically negative for EBV. We herein present a unique case of a 78-year-old Chinese male with EBV-positive, HPV-negative NPC of the left tonsil. His presenting symptom was a left-sided lymph node. There was no evidence of nasopharyngeal lesion seen on physical examination, PET and MRI. The patient was treated with curative-intent external beam radiotherapy which delivered 70 Gy (Gy) to the gross tumour and lymph nodes, and 56 Gy electively to the ipsilateral neck using a volumetric modulated arc therapy technique. This is the first case of primary tonsil EBV-positive NPC described in the literature. Keywords Nasopharyngeal carcinoma · Lymphoepithelial carcinoma · Epstein-barr virus · Oropharynx
Introduction Nasopharyngeal carcinoma (NPC) is a rare tumour occurring most commonly in Inuit, South-Eastern Chinese and Northern Africans [1]. In 2018, there were 129,079 newly diagnosed cases of NPC worldwide, representing 0.7% of all cancers, and causing 0.8% of death related to cancer (all sites) [2]. According to the 2017 World Health Organization (WHO) classification for Head and Neck tumours, nasopharyngeal carcinoma can be subdivided into three histopathology types: (1) Non-keratinizing squamous cell carcinoma (NK-SCC), (2) keratinizing squamous cell carcinoma and (3) basaloid squamous cell carcinoma [1]. NK-SCC can * Eitan Prisman [email protected] 1
Division of Otolaryngology—Head and Neck Surgery, Department of Surgery, Vancouver General Hospital, Vancouver, BC, Canada
2
University of British Columbia, Vancouver, BC, Canada
3
Division of Radiation Oncology, Department of Surgery, BC Cancer Agency, Vancouver, BC, Canada
4
Department of Pathology and Laboratory Medicine, Vancouver General Hospital, Vancouver, BC, Canada
be further categorized into undifferentiated (most common) and differentiated carcinoma, but these sub-classifications of NK-SCC has been shown to have little prognostic value. Epstein-Barr virus (EBV) has a strong association with NPC, especially the NK-SCC subtype, and is conversely absent in keratinizing squamous cell carcinoma. The most common localization for NPC is the pharyngeal recess (fossa of Rosenmuller), but most patients consult for a neck mass as presenting sympt
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