Epstein-Barr Virus-Positive Large Cell Neuroendocrine Carcinoma of the Nasopharynx: Report of a Case with Complete Clini
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CASE REPORT
Epstein-Barr Virus-Positive Large Cell Neuroendocrine Carcinoma of the Nasopharynx: Report of a Case with Complete Clinical and Radiological Response After Combined Chemoradiotherapy Jason K. Wasserman1,2 · Sylvia Papp1,2 · Andrew J. Hope1,2 · Bayardo Perez‑Ordóñez1,2 Received: 17 November 2017 / Accepted: 29 December 2017 © Springer Science+Business Media, LLC, part of Springer Nature 2018
Abstract Neuroendocrine carcinomas of the head and neck are rare and are classified as well differentiated, moderately differentiated, and poorly differentiated carcinomas with the latter category being subdivided into small cell and large cell neuroendocrine carcinoma (LCNEC). While most carcinomas in the nasopharynx are associated with Epstein-Barr virus (EBV), there has been only one previous report demonstrating a link between EBV and LCNEC of the nasopharynx. In this report we describe a second case of EBV-positive LCNEC arising in the nasopharynx with bilateral cervical metastases. The patient was treated with a combination of radiation and chemotherapy which resulted in a complete clinical and radiological response. The patient is still disease free 3 years after presentation. The results of this case suggest that EBV-positive LCNEC is sensitive to chemoradiotherapy and as a result may have better prognosis than EBV-negative LCNEC arising in the nasopharynx or other sites. Keywords Epstein-Barr virus · Large cell neuroendocrine carcinoma · Nasopharynx · Radiation therapy
Introduction Neuroendocrine carcinomas (NEC) of the head and neck are rare and the majority of tumours arise in the larynx. The 2016 WHO Classification of Head and Neck Tumours includes three categories of NEC: well-differentiated, moderately differentiated, and poorly differentiated [1]. The well differentiated category is synonymous with the term “carcinoid” while the poorly differentiated category includes both small cell neuroendocrine carcinoma (SCNEC) and large cell neuroendocrine carcinoma (LCNEC). Etiologic agents described include smoking and HPV, which largely mirror the agents known to be associated with squamous cell carcinoma in the oral cavity and oropharynx, respectively [2, 3]. In contrast, most non-neuroendocrine tumours that arise * Bayardo Perez‑Ordóñez bayardo.perez‑[email protected] 1
Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada
Laboratory Medicine Program, Department of Pathology, University Health Network, Toronto General Hospital, 200 Elizabeth Street, Toronto, ON M5G 2C4, Canada
2
in the nasopharynx are associated with Epstein-Barr virus (EBV) although very little is known about the role of EBV in the development NEC in this site [4]. Moreover, poorly differentiated NEC of the head and neck are associated with poor prognosis and it is unclear if the presence of EBV alters the outcome for these patients [5]. The current report expands on our knowledge by describing a case of LCNEC associated with EBV arising in the nasopharynx which demonstrated complete clini
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