Epidemiology, etiology, and diagnosis of nasopharyngeal carcinoma
- Edwin P Hui, MD
Edwin P Hui, MD
- Clinical Associate Professor (Honorary)
- Department of Clinical Oncology
- Prince of Wales Hospital
- The Chinese University of Hong Kong
- Anthony TC Chan, MD
Anthony TC Chan, MD
- Professor of Clinical Oncology
- Prince of Wales Hospital
- The Chinese University of Hong Kong
- Section Editors
- Bruce E Brockstein, MD
Bruce E Brockstein, MD
- Section Editor — Cancer of the Head and Neck
- Clinical Professor of Medicine
- University of Chicago Pritzker School of Medicine
- David M Brizel, MD
David M Brizel, MD
- Section Editor — Radiation Therapy
- Leonard R Prosnitz Professor of Radiation Oncology
- Professor of Otolaryngology Head & Neck Surgery
- Duke University Cancer Institute
- Marvin P Fried, MD, FACS
Marvin P Fried, MD, FACS
- Section Editor — Head and Neck Surgery
- Professor and University Chairman, Department of Otorhinolaryngology - Head and Neck Surgery
- Montefiore Medical Center, Albert Einstein College of Medicine
Nasopharyngeal carcinoma is the predominant tumor type arising in the nasopharynx, the narrow tubular passage behind the nasal cavity. It differs from other head and neck squamous cell carcinomas in epidemiology, histology, natural history, and response to treatment.
This topic discusses the epidemiology, etiology, diagnosis, and staging of nasopharyngeal carcinoma. The pathology and treatment of nasopharyngeal carcinoma are presented elsewhere.To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Ferlay J, Soerjomataram I, Dikshit R, et al. Cancer incidence and mortality worldwide: sources, methods and major patterns in GLOBOCAN 2012. Int J Cancer 2015; 136:E359.
- Chua ML, Wee JT, Hui EP, Chan AT. Nasopharyngeal carcinoma. Lancet 2016; 387:1012.
- Chang ET, Adami HO. The enigmatic epidemiology of nasopharyngeal carcinoma. Cancer Epidemiol Biomarkers Prev 2006; 15:1765.
- Barnes L, Eveson JW, Reichart P, Sidransky D. Pathology and Genetics of Head and Neck Tumours. In: World Health Organization Classification of Tumors, IARC Press, Lyon 2005.
- Lee AW, Foo W, Mang O, et al. Changing epidemiology of nasopharyngeal carcinoma in Hong Kong over a 20-year period (1980-99): an encouraging reduction in both incidence and mortality. Int J Cancer 2003; 103:680.
- Yu MC, Yuan JM. Epidemiology of nasopharyngeal carcinoma. Semin Cancer Biol 2002; 12:421.
- Hsu C, Shen YC, Cheng CC, et al. Difference in the incidence trend of nasopharyngeal and oropharyngeal carcinomas in Taiwan: implication from age-period-cohort analysis. Cancer Epidemiol Biomarkers Prev 2006; 15:856.
- Vaughan TL, Shapiro JA, Burt RD, et al. Nasopharyngeal cancer in a low-risk population: defining risk factors by histological type. Cancer Epidemiol Biomarkers Prev 1996; 5:587.
- Raghupathy R, Hui EP, Chan AT. Epstein-Barr virus as a paradigm in nasopharyngeal cancer: from lab to clinic. Am Soc Clin Oncol Educ Book 2014; :149.
- Xu FH, Xiong D, Xu YF, et al. An epidemiological and molecular study of the relationship between smoking, risk of nasopharyngeal carcinoma, and Epstein-Barr virus activation. J Natl Cancer Inst 2012; 104:1396.
- Hsu WL, Chen JY, Chien YC, et al. Independent effect of EBV and cigarette smoking on nasopharyngeal carcinoma: a 20-year follow-up study on 9,622 males without family history in Taiwan. Cancer Epidemiol Biomarkers Prev 2009; 18:1218.
- Guo X, Johnson RC, Deng H, et al. Evaluation of nonviral risk factors for nasopharyngeal carcinoma in a high-risk population of Southern China. Int J Cancer 2009; 124:2942.
- Ung A, Chen CJ, Levine PH, et al. Familial and sporadic cases of nasopharyngeal carcinoma in Taiwan. Anticancer Res 1999; 19:661.
- Loh KS, Goh BC, Lu J, et al. Familial nasopharyngeal carcinoma in a cohort of 200 patients. Arch Otolaryngol Head Neck Surg 2006; 132:82.
- Hildesheim A, Apple RJ, Chen CJ, et al. Association of HLA class I and II alleles and extended haplotypes with nasopharyngeal carcinoma in Taiwan. J Natl Cancer Inst 2002; 94:1780.
- Lu CC, Chen JC, Jin YT, et al. Genetic susceptibility to nasopharyngeal carcinoma within the HLA-A locus in Taiwanese. Int J Cancer 2003; 103:745.
- Feng BJ, Huang W, Shugart YY, et al. Genome-wide scan for familial nasopharyngeal carcinoma reveals evidence of linkage to chromosome 4. Nat Genet 2002; 31:395.
- Hu SP, Day NE, Li DR, et al. Further evidence for an HLA-related recessive mutation in nasopharyngeal carcinoma among the Chinese. Br J Cancer 2005; 92:967.
- Tiwawech D, Srivatanakul P, Karalak A, Ishida T. Cytochrome P450 2A6 polymorphism in nasopharyngeal carcinoma. Cancer Lett 2006; 241:135.
- Cao Y, Miao XP, Huang MY, et al. Polymorphisms of XRCC1 genes and risk of nasopharyngeal carcinoma in the Cantonese population. BMC Cancer 2006; 6:167.
- Dogan S, Hedberg ML, Ferris RL, et al. Human papillomavirus and Epstein-Barr virus in nasopharyngeal carcinoma in a low-incidence population. Head Neck 2014; 36:511.
- Stenmark MH, McHugh JB, Schipper M, et al. Nonendemic HPV-positive nasopharyngeal carcinoma: association with poor prognosis. Int J Radiat Oncol Biol Phys 2014; 88:580.
- Lo KW, Chung GT, To KF. Deciphering the molecular genetic basis of NPC through molecular, cytogenetic, and epigenetic approaches. Semin Cancer Biol 2012; 22:79.
- Pathology and genetics of head and neck tumors. In: World Health Organization Classification of Tumours, Barnes L, Eveson JW, Reichart P, Sidransky D (Eds), IARC Press, Lyon 2005.
- Müller E, Beleites E. The basaloid squamous cell carcinoma of the nasopharynx. Rhinology 2000; 38:208.
- Wei WI, Sham JS. Nasopharyngeal carcinoma. Lancet 2005; 365:2041.
- King AD, Lam WW, Leung SF, et al. MRI of local disease in nasopharyngeal carcinoma: tumour extent vs tumour stage. Br J Radiol 1999; 72:734.
- Aiken RD. Neurologic complications of head and neck cancers. Semin Oncol 2006; 33:348.
- Hsu MM, Tu SM. Nasopharyngeal carcinoma in Taiwan. Clinical manifestations and results of therapy. Cancer 1983; 52:362.
- Vokes EE, Liebowitz DN, Weichselbaum RR. Nasopharyngeal carcinoma. Lancet 1997; 350:1087.
- Altun M, Fandi A, Dupuis O, et al. Undifferentiated nasopharyngeal cancer (UCNT): current diagnostic and therapeutic aspects. Int J Radiat Oncol Biol Phys 1995; 32:859.
- Sakata K, Hareyama M, Tamakawa M, et al. Prognostic factors of nasopharynx tumors investigated by MR imaging and the value of MR imaging in the newly published TNM staging. Int J Radiat Oncol Biol Phys 1999; 43:273.
- Liao XB, Mao YP, Liu LZ, et al. How does magnetic resonance imaging influence staging according to AJCC staging system for nasopharyngeal carcinoma compared with computed tomography? Int J Radiat Oncol Biol Phys 2008; 72:1368.
- Cvitkovic E, Bachouchi M, Boussen H, et al. Leukemoid reaction, bone marrow invasion, fever of unknown origin, and metastatic pattern in the natural history of advanced undifferentiated carcinoma of nasopharyngeal type: a review of 255 consecutive cases. J Clin Oncol 1993; 11:2434.
- Leung SF, Zee B, Ma BB, et al. Plasma Epstein-Barr viral deoxyribonucleic acid quantitation complements tumor-node-metastasis staging prognostication in nasopharyngeal carcinoma. J Clin Oncol 2006; 24:5414.
- Liu FF, Frappier L, Kim J, et al. East-West Symposium on nasopharyngeal cancer. Int J Radiat Oncol Biol Phys 2007; 67:703.
- Chan AT, Lo YM, Zee B, et al. Plasma Epstein-Barr virus DNA and residual disease after radiotherapy for undifferentiated nasopharyngeal carcinoma. J Natl Cancer Inst 2002; 94:1614.
- Lin JC, Wang WY, Chen KY, et al. Quantification of plasma Epstein-Barr virus DNA in patients with advanced nasopharyngeal carcinoma. N Engl J Med 2004; 350:2461.
- Zhang W, Chen Y, Chen L, et al. The clinical utility of plasma Epstein-Barr virus DNA assays in nasopharyngeal carcinoma: the dawn of a new era?: a systematic review and meta-analysis of 7836 cases. Medicine (Baltimore) 2015; 94:e845.
- Chua ML, Ong SC, Wee JT, et al. Comparison of 4 modalities for distant metastasis staging in endemic nasopharyngeal carcinoma. Head Neck 2009; 31:346.
- Chang MC, Chen JH, Liang JA, et al. Accuracy of whole-body FDG-PET and FDG-PET/CT in M staging of nasopharyngeal carcinoma: a systematic review and meta-analysis. Eur J Radiol 2013; 82:366.
- Tang LQ, Chen QY, Fan W, et al. Prospective study of tailoring whole-body dual-modality [18F]fluorodeoxyglucose positron emission tomography/computed tomography with plasma Epstein-Barr virus DNA for detecting distant metastasis in endemic nasopharyngeal carcinoma at initial staging. J Clin Oncol 2013; 31:2861.
- Lee AWM, Lydiatt WM, Colevas AD, et al. Nasopharynx. In: AJCC Cancer Staging Manual, 8th, Amin MB (Ed), Springer, New York 2017. p.103.
- Lo YM, Chan LY, Lo KW, et al. Quantitative analysis of cell-free Epstein-Barr virus DNA in plasma of patients with nasopharyngeal carcinoma. Cancer Res 1999; 59:1188.
- Stevens SJ, Verkuijlen SA, Hariwiyanto B, et al. Diagnostic value of measuring Epstein-Barr virus (EBV) DNA load and carcinoma-specific viral mRNA in relation to anti-EBV immunoglobulin A (IgA) and IgG antibody levels in blood of nasopharyngeal carcinoma patients from Indonesia. J Clin Microbiol 2005; 43:3066.
- Chan KC, Lo YM. Circulating EBV DNA as a tumor marker for nasopharyngeal carcinoma. Semin Cancer Biol 2002; 12:489.
- Chan KC, Hung EC, Woo JK, et al. Early detection of nasopharyngeal carcinoma by plasma Epstein-Barr virus DNA analysis in a surveillance program. Cancer 2013; 119:1838.
- Lit LC, Chan KC, Leung SF, et al. Distribution of cell-free and cell-associated Epstein-Barr virus (EBV) DNA in the blood of patients with nasopharyngeal carcinoma and EBV-associated lymphoma. Clin Chem 2004; 50:1842.
- Chan KCA, Woo JKS, King A, et al. Analysis of Plasma Epstein-Barr Virus DNA to Screen for Nasopharyngeal Cancer. N Engl J Med 2017; 377:513.
- Chua DT, Ji M, Zong Y, Chan K. Screening of nasopharyngeal carcinoma by serology and nasopharyngoscopy and treatment outcome in endemic region. J Clin Oncol 2009; 27:308s.
- Zong YS, Sham JS, Ng MH, et al. Immunoglobulin A against viral capsid antigen of Epstein-Barr virus and indirect mirror examination of the nasopharynx in the detection of asymptomatic nasopharyngeal carcinoma. Cancer 1992; 69:3.
- Zeng Y, Zhang LG, Wu YC, et al. Prospective studies on nasopharyngeal carcinoma in Epstein-Barr virus IgA/VCA antibody-positive persons in Wuzhou City, China. Int J Cancer 1985; 36:545.
- Liu Z, Ji MF, Huang QH, et al. Two Epstein-Barr virus-related serologic antibody tests in nasopharyngeal carcinoma screening: results from the initial phase of a cluster randomized controlled trial in Southern China. Am J Epidemiol 2013; 177:242.
- Chan AT, Ma BB, Lo YM, et al. Phase II study of neoadjuvant carboplatin and paclitaxel followed by radiotherapy and concurrent cisplatin in patients with locoregionally advanced nasopharyngeal carcinoma: therapeutic monitoring with plasma Epstein-Barr virus DNA. J Clin Oncol 2004; 22:3053.
- Leung SF, Lo YM, Chan AT, et al. Disparity of sensitivities in detection of radiation-naïve and postirradiation recurrent nasopharyngeal carcinoma of the undifferentiated type by quantitative analysis of circulating Epstein-Barr virus DNA1,2. Clin Cancer Res 2003; 9:3431.
- Hui EP, Ma BB, Chan KC, et al. Clinical utility of plasma Epstein-Barr virus DNA and ERCC1 single nucleotide polymorphism in nasopharyngeal carcinoma. Cancer 2015; 121:2720.
- Geographic and ethnic distribution
- Sex and age distribution
- Secular trend
- ETIOLOGY AND RISK FACTORS
- Epstein-Barr virus
- Human papillomavirus
- Molecular pathogenesis
- CLINICAL PRESENTATION
- INITIAL DIAGNOSTIC EVALUATION
- SCREENING BASED ON EBV VIRUS TESTING
- Monitoring for treatment response and recurrence
- SUMMARY AND RECOMMENDATIONS