Nasopharyngeal Carcinoma

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Pediatric Surgical Oncology

Abstract

Nasopharyngeal carcinoma (NPC) is a relatively rare tumor in the pediatric population, accounting for less than 1% of all pediatric malignancies. Most prevalent in teenagers than in children under 10 years of age, NPC is also highly related to the Epstein-Barr virus (EBV). NPC initially presents with nonspecific signs and symptoms such as nasal congestion, epistaxis, and/or headaches in a previously healthy child. Due to its nonspecific presenting signs, NPC is often diagnosed at a loco-regionally advanced stage in the pediatric and adolescent populations. However, it carries a better prognosis in children than in adults, with a 5-year overall survival reportedly close to 90%. NPC is sensitive to chemotherapy and radiation therapy, and concurrent chemoradiotherapy with or without neoadjuvant chemotherapy is considered as the first-line treatment in children, while surgery is only recommended for recurrences or nonresponding cases.

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References

  1. SEER Cancer Statistics Review 1975-2006 – Previous Version – SEER Cancer Statistics [Internet]. SEER. [cited 2021 Aug 21]. Available from: https://seer.cancer.gov/archive/csr/1975_2006/index.html

    Google Scholar 

  2. Sultan I, Casanova M, Ferrari A, Rihani R, Rodriguez-Galindo C. Differential features of nasopharyngeal carcinoma in children and adults: a SEER study. Pediatr Blood Cancer. 2010;55(2):279–84.

    Article  Google Scholar 

  3. Richards MK, Dahl JP, Gow K, Goldin AB, Doski J, Goldfarb M, et al. Factors associated with mortality in pediatric vs adult nasopharyngeal carcinoma. JAMA Otolaryngol–Head Neck Surg. 2016;142(3):217–22.

    Article  Google Scholar 

  4. Dourthe M-E, Bolle S, Temam S, Jouin A, Claude L, Reguerre Y, et al. Childhood nasopharyngeal carcinoma: state-of-the-art, and questions for the future. J Pediatr Hematol Oncol. 2018;40(2):85–92.

    Article  Google Scholar 

  5. Ayan I, Kaytan E, Ayan N. Childhood nasopharyngeal carcinoma: from biology to treatment. Lancet Oncol. 2003;4(1):13–21.

    Article  CAS  Google Scholar 

  6. Dawson CW, Port RJ, Young LS. The role of the EBV-encoded latent membrane proteins LMP1 and LMP2 in the pathogenesis of nasopharyngeal carcinoma (NPC). Semin Cancer Biol. 2012;22(2):144–53.

    Article  CAS  Google Scholar 

  7. Mertens R, Granzen B, Lassay L, Gademann G, Hess CF, Heimann G. Nasopharyngeal carcinoma in childhood and adolescence: concept and preliminary results of the cooperative GPOH study NPC-91. Gesellschaft für Pädiatrische Onkologie und Hämatologie. Cancer. 1997;80(5):951–9.

    Article  CAS  Google Scholar 

  8. Goldsmith DB, West TM, Morton R. HLA associations with nasopharyngeal carcinoma in southern Chinese: a meta-analysis. Clin Otolaryngol Allied Sci. 2002;27(1):61–7.

    Article  CAS  Google Scholar 

  9. Chan SH, Chew CT, Prasad U, Wee GB, Srinivasan N, Kunaratnam N. HLA and nasopharyngeal carcinoma in Malays. Br J Cancer. 1985;51(3):389–92.

    Article  CAS  Google Scholar 

  10. Rodriguez S, Khabir A, Keryer C, Perrot C, Drira M, Ghorbel A, et al. Conventional and array-based comparative genomic hybridization analysis of nasopharyngeal carcinomas from the Mediterranean area. Cancer Genet Cytogenet. 2005;157(2):140–7.

    Article  CAS  Google Scholar 

  11. Hyare H, Wisco JJ, Alusi G, Cohen M, Nabili V, Abemayor E, et al. The anatomy of nasopharyngeal carcinoma spread through the pharyngobasilar fascia to the trigeminal mandibular nerve on 1.5 T MRI. Surg Radiol Anat. 2010;32(10):937–44.

    Article  Google Scholar 

  12. Rouge M-È, Brisse H, Helfre S, Teissier N, Freneaux P, Orbach D. Undifferentiated nasopharyngeal carcinoma in adolescent and children. Bull Cancer. 2011;98(3):337–45.

    Article  Google Scholar 

  13. Pathmanathan R, Prasad U, Sadler R, Flynn K, Raab-Traub N. Clonal proliferations of cells infected with Epstein-Barr virus in preinvasive lesions related to nasopharyngeal carcinoma. N Engl J Med. 1995;333(11):693–8.

    Article  CAS  Google Scholar 

  14. Banko AV, Lazarevic IB, Folic MM, Djukic VB, Cirkovic AM, Karalic DZ, et al. Characterization of the variability of Epstein-Barr virus genes in nasopharyngeal biopsies: potential predictors for carcinoma progression. PLoS One. 2016;11(4):e0153498.

    Article  Google Scholar 

  15. Ma BBY, King A, Lo YMD, Yau YY, Zee B, Hui EP, et al. Relationship between pretreatment level of plasma Epstein-Barr virus DNA, tumor burden, and metabolic activity in advanced nasopharyngeal carcinoma. Int J Radiat Oncol Biol Phys. 2006;66(3):714–20.

    Article  CAS  Google Scholar 

  16. Qiu W, Lv X, Guo X, Yuan Y. Clinical implications of plasma Epstein-Barr virus DNA in children and adolescent nasopharyngeal carcinoma patients receiving intensity-modulated radiotherapy. Front Oncol. 2020;10:356.

    Article  Google Scholar 

  17. Glastonbury CM. Nasopharyngeal carcinoma: the role of magnetic resonance imaging in diagnosis, staging, treatment, and follow-up. Top Magn Reson Imaging. 2007;18(4):225–35.

    Article  Google Scholar 

  18. Dubrulle F, Souillard R, Hermans R. Extension patterns of nasopharyngeal carcinoma. Eur Radiol. 2007;17(10):2622–30.

    Article  CAS  Google Scholar 

  19. Tang L-L, Li W-F, Chen L, Sun Y, Chen Y, Liu L-Z, et al. Prognostic value and staging categories of anatomic masticator space involvement in nasopharyngeal carcinoma: a study of 924 cases with MR imaging. Radiology. 2010;257(1):151–7.

    Article  Google Scholar 

  20. Liu W, Tang Y, Gao L, Huang X, Luo J, Zhang S, et al. Nasopharyngeal carcinoma in children and adolescents - a single institution experience of 158 patients. Radiat Oncol 2014 Dec 5;9:274.

    Google Scholar 

  21. Amin MB, Edge S, Greene F, Byrd DR, Brookland RK, Washington MK, et al., editors. AJCC Cancer Staging Manual [Internet]. 8th ed. Springer International Publishing; 2017 [cited 2021 Aug 21]. Available from: https://www.springer.com/gp/book/9783319406176

  22. Tang L-L, Chen Y-P, Mao Y-P, Wang Z-X, Guo R, Chen L, et al. Validation of the 8th edition of the UICC/AJCC staging system for nasopharyngeal carcinoma from endemic areas in the intensity-modulated radiotherapy era. J Natl Compr Cancer Netw. 2017;15(7):913–9.

    Article  Google Scholar 

  23. Cheuk DKL, Billups CA, Martin MG, Roland CR, Ribeiro RC, Krasin MJ, et al. Prognostic factors and long-term outcomes of childhood nasopharyngeal carcinoma. Cancer. 2011;117(1):197–206.

    Article  CAS  Google Scholar 

  24. Ribassin-Majed L, Marguet S, Lee AWM, Ng WT, Ma J, Chan ATC, et al. What is the best treatment of locally advanced nasopharyngeal carcinoma? An individual patient data network meta-analysis. J Clin Oncol. 2017;35(5):498–505.

    Article  Google Scholar 

  25. Buehrlen M, Zwaan CM, Granzen B, Lassay L, Deutz P, Vorwerk P, et al. Multimodal treatment, including interferon beta, of nasopharyngeal carcinoma in children and young adults: preliminary results from the prospective, multicenter study NPC-2003-GPOH/DCOG. Cancer. 2012;118(19):4892–900.

    Article  CAS  Google Scholar 

  26. Ben-Ami T, Kontny U, Surun A, Brecht IB, Almaraz RL, Dragomir M, et al. Nasopharyngeal carcinoma in children and adolescents: the EXPeRT/PARTNER diagnostic and therapeutic recommendations. Pediatr Blood Cancer. 2021;68(Suppl 4):e29018.

    Google Scholar 

  27. Teymoortash A, Werner JA. Current advances in diagnosis and surgical treatment of lymph node metastasis in head and neck cancer. GMS Curr top Otorhinolaryngol. Head Neck Surg. 2012;11:Doc04.

    CAS  Google Scholar 

  28. Chen WC, Jackson A, Budnick AS, Pfister DG, Kraus DH, Hunt MA, et al. Sensorineural hearing loss in combined modality treatment of nasopharyngeal carcinoma. Cancer. 2006;106(4):820–9.

    Article  Google Scholar 

  29. Petsuksiri J, Sermsree A, Thephamongkhol K, Keskool P, Thongyai K, Chansilpa Y, et al. Sensorineural hearing loss after concurrent chemoradiotherapy in nasopharyngeal cancer patients. Radiat Oncol. 2011;6:19.

    Article  Google Scholar 

  30. Rodriguez-Galindo C, Krailo MD, Krasin MJ, Huang L, McCarville MB, Hicks J, et al. Treatment of childhood nasopharyngeal carcinoma with induction chemotherapy and concurrent Chemoradiotherapy: results of the Children’s oncology group ARAR0331 study. J Clin Oncol. 2019;37(35):3369–76.

    Article  CAS  Google Scholar 

  31. Abayomi OK. Pathogenesis of cognitive decline following therapeutic irradiation for head and neck tumors. Acta Oncol. 2002;41(4):346–51.

    Article  Google Scholar 

  32. Hsu Y-C, Wang L-F, Lee K-W, Ho K-Y, Huang C-J, Kuo W-R. Cerebral radionecrosis in patients with nasopharyngeal carcinoma. Kaohsiung J Med Sci. 2005;21(10):452–9.

    Article  Google Scholar 

  33. Guo Z, Han L, Yang Y, He H, Li J, Chen H, et al. Longitudinal brain structural alterations in patients with nasopharyngeal carcinoma early after radiotherapy. Neuroimage Clin. 2018;19:252–9.

    Article  Google Scholar 

  34. Malone JP, Levin RJ. Second malignant tumors after treatment of nasopharyngeal carcinoma. Skull Base. 2002;12(2):87–91.

    Article  Google Scholar 

  35. ** M, Liu M-Z, Wang H-X, Cai L, Zhang L, **e C-F, et al. Radiation-induced sarcoma in patients with nasopharyngeal carcinoma. Cancer. 2010;116(23):5479–86.

    Article  Google Scholar 

  36. Shen C, Gao Y, Xu T, Wang X, Ying H, Hu C. Carcinoma of the nasopharynx in young patients: a single institution experience. Clin Oncol (R Coll Radiol). 2009;21(8):617–22.

    Article  Google Scholar 

  37. Yan Z, **a L, Huang Y, Chen P, Jiang L, Zhang B. Nasopharyngeal carcinoma in children and adolescents in an endemic area: a report of 185 cases. Int J Pediatr Otorhinolaryngol. 2013;77(9):1454–60.

    Article  Google Scholar 

  38. Mertens R, Granzen B, Lassay L, Bucsky P, Hundgen M, Stetter G, et al. Treatment of nasopharyngeal carcinoma in children and adolescents. Cancer. 2005;104(5):1083–9.

    Article  Google Scholar 

  39. Downing NL, Wolden S, Wong P, Petrik DW, Hara W, Le Q-T. Comparison of treatment results between adult and juvenile nasopharyngeal carcinoma. Int J Radiat Oncol Biol Phys. 2009;75(4):1064–70.

    Article  Google Scholar 

  40. Zhang L, Tang L-Q, Chen Q-Y, Liu H, Guo S-S, Liu L-T, et al. Plasma Epstein-Barr viral DNA complements TNM classification of nasopharyngeal carcinoma in the era of intensity-modulated radiotherapy. Oncotarget. 2015;7(5):6221–30.

    Article  Google Scholar 

  41. Lin J-C, Wang W-Y, Liang W-M, Chou H-Y, Jan J-S, Jiang R-S, et al. Long-term prognostic effects of plasma epstein-barr virus DNA by minor groove binder-probe real-time quantitative PCR on nasopharyngeal carcinoma patients receiving concurrent chemoradiotherapy. Int J Radiat Oncol Biol Phys. 2007;68(5):1342–8.

    Article  CAS  Google Scholar 

  42. Twu C-W, Wang W-Y, Liang W-M, Jan J-S, Jiang R-S, Chao J, et al. Comparison of the prognostic impact of serum anti-EBV antibody and plasma EBV DNA assays in nasopharyngeal carcinoma. Int J Radiat Oncol Biol Phys. 2007;67(1):130–7.

    Article  CAS  Google Scholar 

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Correspondence to Anthony Sheyn .

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Richard, C., Sheyn, A. (2022). Nasopharyngeal Carcinoma. In: Lakhoo, K., Abdelhafeez, A.H., Abib, S. (eds) Pediatric Surgical Oncology. Springer, Cham. https://doi.org/10.1007/978-3-030-71113-9_105-1

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  • DOI: https://doi.org/10.1007/978-3-030-71113-9_105-1

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