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6196907 
Book/Book Chapter 
Nasopharyngeal Carcinoma: Diagnosis and Treatment 
Szymańska, K 
2019 
Academic Press 
Oxford 
Encyclopedia of Cancer (Third Edition) 
571-577 
Nasopharyngeal cancer (NPC) is the most common cancer of the nasopharynx but a relatively rare malignancy overall, with a striking geographical variation in incidence. About 70% of cases are diagnosed in east and south-east Asia, almost 30% in other parts of Asia and some parts of Africa, while in Western countries NPC is rare. The term encompasses three distinct entities: nonkeratinizing, keratinizing, and basaloid squamous cell carcinoma. Nonkeratinizing NPC subtype is associated with Epstein-Barr virus (EBV) infections and occurs mainly in areas of high EBV prevalence. Other risk factors include Chinese-style salted fish consumption as well as exposure to formaldehyde and wood dust. Genetic alterations are found, among others, in TP53 and genes of the chromatin remodeling pathway (e.g., ARID1A). NPC is a highly aggressive tumor and a majority of patients present with advanced disease. The diagnostic workup includes imaging and pathology. Testing for EBV is helpful in disease diagnosis, prognostication, and monitoring the response to treatment. However, the protocols for EBV testing urgently need to be validated and standardized. Most tumors are not curable by surgical resection, and are treated with a combination of radiotherapy and chemotherapy. Molecular and immunotherapies hold promise for the future. In particular, EGFR and VEGF inhibitors have shown clinical efficacy in patients with platinum-refractory disease, and other molecules and vaccines are emerging. 
Chemoradiotherapy; Chromatin remodeling; Cisplatin; Epstein-Barr virus; Keratinizing; Nasopharyngeal cancer; Nasopharyngeal carcinoma; Nasopharynx; Nonkeratinizing; Squamous cell carcinoma; T-cell immunotherapy 
Hainaut, Pierre