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Special Consideration in Pediatric Nasopharyngeal Cancer

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Nasopharyngeal Cancer

Part of the book series: Practical Guides in Radiation Oncology ((PGRO))

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Abstract

Nasopharyngeal cancer (NPC) is a rare condition in pediatric population. Its management poses major challenges to pediatric oncologists due to the significant difference in terms of disease and patient characteristics between pediatric and adult patients diagnosed with NPC. Pediatric NPC (PNCP) almost always have the undifferentiated variant, which is associated with advanced locoregional spread and distant metastases. Although radiotherapy remains the mainstay treatment for patients with nonmetastatic PNPC, radiation dose and volume reduction should be considered for PNPC with an aim to reduce radiation-induced adverse effects including second primary malignancy (SPM) while maintaining the efficacy. Photon-based intensity-modulated radiation therapy might improve dose profile in terms of reducing radiation to critical organs at risk (OARs); the increased volume encompassed by the collateral dose may induce more dismal long-term adverse effects especially the radiation-induced SPM in long-term survivors. Proton radiation therapy significantly reduces both volumes encompassing collateral dose and organs at risk (OARs) while maintains the dose coverage to target volumes and should be considered for PNPC when such technology is available. Clinical evidence of chemotherapy for PNPC is limited, and no comparative studies in randomized fashion have been published to support a favorable and effective chemotherapy regimen or schedule. Due to the lack of well-designed prospective clinical trials, it is not feasible to form a standard treatment guideline for PNPC. Currently, radiation therapy alone is the treatment of choice for patients with early-stage PNPC, and induction chemotherapy followed by concurrent chemoradiation therapy is suggested in patients present with locally advanced PNPC.

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References

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

    Article  CAS  PubMed  Google Scholar 

  2. Barista I, Varan A, Ozyar E. Bimodal age distribution in Hodgkin’s disease and nasopharyngeal carcinoma. Med Hypotheses. 2007;68(6):1421.

    Article  PubMed  Google Scholar 

  3. Li C, Wang Y. Factors associated with early diagnosis in pediatric vs adult nasopharyngeal carcinoma. Acta Otolaryngol. 2018;138(1):56–9.

    Article  PubMed  Google Scholar 

  4. Wu SG, Liao XL, He ZY, Tang LY, Chen XT, Wang Y, et al. Demographic and clinicopathological characteristics of nasopharyngeal carcinoma and survival outcomes according to age at diagnosis: a population-based analysis. Oral Oncol. 2017;73:83–7.

    Article  PubMed  Google Scholar 

  5. 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  PubMed  Google Scholar 

  6. 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  PubMed  Google Scholar 

  7. Hu S, Xu X, Xu J, Xu Q, Liu S. Prognostic factors and long-term outcomes of nasopharyngeal carcinoma in children and adolescents. Pediatr Blood Cancer. 2013;60(7):1122–7.

    Article  PubMed  Google Scholar 

  8. Shen T, Tang LQ, Gu WG, Luo DH, Chen QY, Li PJ, et al. Plasma Epstein-Barr viral deoxyribonucleic acid predicts worse outcomes in pediatric nonmetastatic nasopharyngeal carcinoma patients: an observational study of 89 cases in an endemic area. Medicine (Baltimore). 2015;94(50):e1945.

    Article  CAS  Google Scholar 

  9. Cheuk DK, Sabin ND, Hossain M, Wozniak A, Naik M, Rodriguez-Galindo C, et al. PET/CT for staging and follow-up of pediatric nasopharyngeal carcinoma. Eur J Nucl Med Mol Imaging. 2012;39(7):1097–106.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Al-Sarraf M, LeBlanc M, Giri PG, Fu KK, Cooper J, Vuong T, et al. Chemoradiotherapy versus radiotherapy in patients with advanced nasopharyngeal cancer: phase III randomized intergroup study 0099. J Clin Oncol. 1998;16(4):1310–7.

    Article  CAS  PubMed  Google Scholar 

  11. Wei WI, Sham JS. Present status of management of nasopharyngeal carcinoma. Expert Rev Anticancer Ther. 2001;1(1):134–41.

    Article  CAS  PubMed  Google Scholar 

  12. Ozyar E, Yildiz F, Akyol FH, Atahan IL. Comparison of AJCC 1988 and 1997 classifications for nasopharyngeal carcinoma. American joint committee on Cancer. Int J Radiat Oncol Biol Phys. 1999;44(5):1079–87.

    Article  CAS  PubMed  Google Scholar 

  13. Fernandez CH, Cangir A, Samaan NA, Rivera R. Nasopharyngeal carcinoma in children. Cancer. 1976;37(6):2787–91.

    Article  CAS  PubMed  Google Scholar 

  14. Gasparini M, Lombardi F, Rottoli L, Ballerini E, Morandi F. Combined radiotherapy and chemotherapy in stage T3 and T4 nasopharyngeal carcinoma in children. J Clin Oncol. 1988;6(3):491–4.

    Article  CAS  PubMed  Google Scholar 

  15. Lombardi F, Gasparini M, Gianni C, De Marie M, Molinari R, Pilotti S. Nasopharyngeal carcinoma in childhood. Med Pediatr Oncol. 1982;10(3):243–50.

    Article  CAS  PubMed  Google Scholar 

  16. Deutsch M, Mercado R Jr, Parsons JA. Cancer of the nasopharynx in children. Cancer. 1978;41(3):1128–33.

    Article  CAS  PubMed  Google Scholar 

  17. Jenkin RD, Anderson JR, Jereb B, Thompson JC, Pyesmany A, Wara WM, et al. Nasopharyngeal carcinoma—a retrospective review of patients less than thirty years of age: a report of Children’s Cancer Study Group. Cancer. 1981;47(2):360–6.

    Article  CAS  PubMed  Google Scholar 

  18. Jereb B, Huvos AG, Steinherz P, Unal A. Nasopharyngeal carcinoma in children review of 16 cases. Int J Radiat Oncol Biol Phys. 1980;6(4):487–91.

    Article  CAS  PubMed  Google Scholar 

  19. Berry MP, Smith CR, Brown TC, Jenkin RD, Rider WD. Nasopharyngeal carcinoma in the young. Int J Radiat Oncol Biol Phys. 1980;6(4):415–21.

    Article  CAS  PubMed  Google Scholar 

  20. Wolden SL, Steinherz PG, Kraus DH, Zelefsky MJ, Pfister DG, Wollner N. Improved long-term survival with combined modality therapy for pediatric nasopharynx cancer. Int J Radiat Oncol Biol Phys. 2000;46(4):859–64.

    Article  CAS  PubMed  Google Scholar 

  21. Daoud J, Toumi N, Bouaziz M, Ghorbel A, Jlidi R, Drira MM, et al. Nasopharyngeal carcinoma in childhood and adolescence: analysis of a series of 32 patients treated with combined chemotherapy and radiotherapy. Eur J Cancer. 2003;39(16):2349–54.

    Article  CAS  PubMed  Google Scholar 

  22. Laskar S, Sanghavi V, Muckaden MA, Ghosh S, Bhalla V, Banavali S, et al. Nasopharyngeal carcinoma in children: ten years’ experience at the Tata Memorial Hospital, Mumbai. Int J Radiat Oncol Biol Phys. 2004;58(1):189–95.

    Article  PubMed  Google Scholar 

  23. Kupeli S, Varan A, Ozyar E, Atahan IL, Yalcin B, Kutluk T, et al. Treatment results of 84 patients with nasopharyngeal carcinoma in childhood. Pediatr Blood Cancer. 2006;46(4):454–8.

    Article  PubMed  Google Scholar 

  24. Ozyar E, Selek U, Laskar S, Uzel O, Anacak Y, Ben-Arush M, et al. Treatment results of 165 pediatric patients with non-metastatic nasopharyngeal carcinoma: a Rare Cancer Network study. Radiother Oncol. 2006;81(1):39–46.

    Article  PubMed  Google Scholar 

  25. Habrand J, Petras S, Le Deley M, Patte C. Carcinoma of the nasopharynx in children and adolescents treated with initial chemotherapy followed by adapted doses of radiotherapy. Proceedings of 46th Annual ASTRO Meeting. Int J Radiat Oncol Biol Phys. 2004;s247:191.

    Google Scholar 

  26. Orbach D, Brisse H, Helfre S, Klijanienko J, Bours D, Mosseri V, et al. Radiation and chemotherapy combination for nasopharyngeal carcinoma in children: radiotherapy dose adaptation after chemotherapy response to minimize late effects. Pediatr Blood Cancer. 2008;50(4):849–53.

    Article  PubMed  Google Scholar 

  27. Lu S, Chang H, Sun X, Zhen Z, Sun F, Zhu J, et al. Long-term outcomes of nasopharyngeal carcinoma in 148 children and adolescents. Medicine (Baltimore). 2016;95(17):e3445.

    Article  CAS  Google Scholar 

  28. 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 fur Padiatrische Onkologie und Hamatologie. Cancer. 1997;80(5):951–9.

    Article  CAS  PubMed  Google Scholar 

  29. Mertens R, Granzen B, Lassay L, Bucsky P, Hundgen M, Stetter G, et al. Treatment of nasopharyngeal carcinoma in children and adolescents: definitive results of a multicenter study (NPC-91-GPOH). Cancer. 2005;104(5):1083–9.

    Article  PubMed  Google Scholar 

  30. 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  PubMed  Google Scholar 

  31. Rodriguez-Galindo C, Wofford M, Castleberry RP, Swanson GP, London WB, Fontanesi J, et al. Preradiation chemotherapy with methotrexate, cisplatin, 5-fluorouracil, and leucovorin for pediatric nasopharyngeal carcinoma. Cancer. 2005;103(4):850–7.

    Article  CAS  PubMed  Google Scholar 

  32. Rodriguez-Galindo CKM, Krasin MJ. Treatment of childhood nasopharyngeal carcinoma (cNPC) with neoadjuvant chemotherapy (NAC) and concomitant chemoradiotherapy (CCRT): Results of the Children’s Oncology Group ARAR0331 study. J Clin Oncol. 2016;34(10513)

    Google Scholar 

  33. Casanova M, Bisogno G, Gandola L, Cecchetto G, Di Cataldo A, Basso E, et al. A prospective protocol for nasopharyngeal carcinoma in children and adolescents: the Italian Rare Tumors in Pediatric Age (TREP) project. Cancer. 2012;118(10):2718–25.

    Article  PubMed  Google Scholar 

  34. Casanova M, Ozyar E, Patte C, Orbach D, Ferrari A, Veyrat-Follet C, et al. International randomized phase 2 study on the addition of docetaxel to the combination of cisplatin and 5-fluorouracil in the induction treatment for nasopharyngeal carcinoma in children and adolescents. Cancer Chemother Pharmacol. 2016;77(2):289–98.

    Article  CAS  PubMed  Google Scholar 

  35. Zaghloul MS, Eldebawy E, Ahmed S, Ammar H, Khalil E, Abdelrahman H, et al. Does primary tumor volume predict the outcome of pediatric nasopharyngeal carcinoma?: a prospective single-arm study using neoadjuvant chemotherapy and concomitant chemotherapy with intensity modulated radiotherapy. Asia Pac J Clin Oncol. 2016;12(2):143–50.

    Article  PubMed  Google Scholar 

  36. Selek U, Ozyar E, Ozyigit G, Varan A, Buyukpamukcu M, Atahan IL. Treatment results of 59 young patients with nasopharyngeal carcinoma. Int J Pediatr Otorhinolaryngol. 2005;69(2):201–7.

    Article  PubMed  Google Scholar 

  37. Chua DT, Sham JS, Au GK. A phase II study of docetaxel and cisplatin as first-line chemotherapy in patients with metastatic nasopharyngeal carcinoma. Oral Oncol. 2005;41(6):589–95.

    Article  CAS  PubMed  Google Scholar 

  38. Nakamura RA, Novaes PE, Antoneli CB, Fogaroli RC, Pellizzon AC, Ferrigno R, et al. High-dose-rate brachytherapy as part of a multidisciplinary treatment of nasopharyngeal lymphoepithelioma in childhood. Cancer. 2005;104(3):525–31.

    Article  PubMed  Google Scholar 

  39. Kam MK, Teo PM, Chau RM, Cheung KY, Choi PH, Kwan WH, et al. Treatment of nasopharyngeal carcinoma with intensity-modulated radiotherapy: the Hong Kong experience. Int J Radiat Oncol Biol Phys. 2004;60(5):1440–50.

    Article  PubMed  Google Scholar 

  40. Laskar S, Bahl G, Muckaden M, Pai SK, Gupta T, Banavali S, et al. Nasopharyngeal carcinoma in children: comparison of conventional and intensity-modulated radiotherapy. Int J Radiat Oncol Biol Phys. 2008;72(3):728–36.

    Article  PubMed  Google Scholar 

  41. Tao CJ, Liu X, Tang LL, Mao YP, Chen L, Li WF, et al. Long-term outcome and late toxicities of simultaneous integrated boost-intensity modulated radiotherapy in pediatric and adolescent nasopharyngeal carcinoma. Chin J Cancer. 2013;32(10):525–32.

    Article  PubMed  PubMed Central  Google Scholar 

  42. Qiu WZ, Peng XS, Xia HQ, Huang PY, Guo X, Cao KJ. A retrospective study comparing the outcomes and toxicities of intensity-modulated radiotherapy versus two-dimensional conventional radiotherapy for the treatment of children and adolescent nasopharyngeal carcinoma. J Cancer Res Clin Oncol. 2017;143(8):1563–72.

    Article  PubMed  PubMed Central  Google Scholar 

  43. 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;9:274.

    Article  PubMed  PubMed Central  Google Scholar 

  44. Guo Q, Cui X, Lin S, Lin J, Pan J. Locoregionally advanced nasopharyngeal carcinoma in childhood and adolescence: analysis of 95 patients treated with combined chemotherapy and intensity-modulated radiotherapy. Head Neck. 2016;38(Suppl 1):E665–72.

    Article  PubMed  Google Scholar 

  45. Li Y, Tang LQ, Liu LT, Guo SS, Liang YJ, Sun XS, et al. Induction chemotherapy plus concurrent chemoradiotherapy versus concurrent chemoradiotherapy alone in locoregionally advanced nasopharyngeal carcinoma in children and adolescents: a matched cohort analysis. Cancer Res Treat. 2018;50(4):1304–15.

    Article  PubMed  PubMed Central  Google Scholar 

  46. DeRenzo C, Lam C, Rodriguez-Galindo C, Rapkin L, Gottschalk S, Venkatramani R. Salvage regimens for pediatric patients with relapsed nasopharyngeal carcinoma. Pediatr Blood Cancer. 2019;66(1):e27469.

    Article  PubMed  Google Scholar 

  47. Sahraoui S, Acharki A, Benider A, Bouras N, Kahlain A. Nasopharyngeal carcinoma in children under 15 years of age: a retrospective review of 65 patients. Ann Oncol. 1999;10(12):1499–502.

    Article  CAS  PubMed  Google Scholar 

  48. Uzel O, Yoruk SO, Sahinler I, Turkan S, Okkan S. Nasopharyngeal carcinoma in childhood: long-term results of 32 patients. Radiother Oncol. 2001;58(2):137–41.

    Article  CAS  PubMed  Google Scholar 

  49. Zubizarreta PA, D’Antonio G, Raslawski E, Gallo G, Preciado MV, Casak SJ, et al. Nasopharyngeal carcinoma in childhood and adolescence: a single-institution experience with combined therapy. Cancer. 2000;89(3):690–5.

    Article  CAS  PubMed  Google Scholar 

  50. Altun M Aİ, Dizdar Y, et al.. Nasopharyngeal carcinoma of children, adolescents and young adults. 40th Congress of the International Society of Pediatric Oncology. 40th Congress of the International Society of Pediatric Oncology, Berlin, Germany Oct 2–6, 2008; SIOP Abstract Book 2008; 142, D118 (abstract). 2008.

    Google Scholar 

  51. Ingersoll L, Woo SY, Donaldson S, Giesler J, Maor MH, Goffinet D, et al. Nasopharyngeal carcinoma in the young: a combined M.D. Anderson and Stanford experience. Int J Radiat Oncol Biol Phys. 1990;19(4):881–7.

    Article  CAS  PubMed  Google Scholar 

  52. Sherif RS, Attalla EM, Elshemey WM, Madian NG. The risk of secondary cancer in nasopharyngeal carcinoma paediatric patients due to intensity modulated radiotherapy and mega-voltage cone beam computed tomography. J Med Imaging Radiat Oncol. 2017;61(3):402–9.

    Article  PubMed  Google Scholar 

  53. Radhakrishnan V, Kumar P, Totadri S, Ganesan P, Selvaluxmy G, Ganesan T, et al. Pediatric nasopharyngeal carcinoma: experience from a tertiary cancer center in India. Indian J Cancer. 2016;53(3):377–80.

    CAS  PubMed  Google Scholar 

  54. Al Mousa A, Abu-Hijlih R, Salem A, Sultan I, Mula-Hussain L, Ismael T, et al. Induction methotrexate, cisplatin, and 5-fluorouracil versus cisplatin and 5-fluorouracil followed by radiotherapy in pediatric nasopharyngeal carcinoma: a retrospective analysis in a tertiary cancer center. J Pediatr Hematol Oncol. 2017;39(8):e437–e42.

    Article  PubMed  CAS  Google Scholar 

  55. Khalil EM, Anwar MM. Treatment results of pediatric nasopharyngeal carcinoma, NCI, Cairo University experience. J Egypt Natl Canc Inst. 2015;27(3):119–28.

    Article  PubMed  Google Scholar 

  56. Das A, Mallick I, Arun P, Midha D, Sen S, Krishnan S, et al. Encouraging outcomes with manageable toxicity using neoadjuvant chemotherapy and intensity-modulated radiotherapy in advanced pediatric nasopharyngeal carcinoma: single-center experience from a developing country. J Pediatr Hematol Oncol. 2017;39(4):318–9.

    Article  PubMed  Google Scholar 

  57. Nikitovic M, Popovic-Vukovic M, Stanic D, Bokun J, Paripovic L, Ilic V, et al. Treatment outcome of childhood nasopharyngeal carcinoma: a single institution experience. Int J Pediatr Otorhinolaryngol. 2018;113:168–72.

    Article  PubMed  Google Scholar 

  58. Sahai P, Mohanti BK, Sharma A, Thakar A, Bhasker S, Kakkar A, et al. Clinical outcome and morbidity in pediatric patients with nasopharyngeal cancer treated with chemoradiotherapy. Pediatr Blood Cancer. 2017;64(2):259–66.

    Article  CAS  PubMed  Google Scholar 

  59. Yan Z, Xia 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  PubMed  Google Scholar 

  60. Uezono H, Indelicato DJ, Rotondo RL, Sandler ES, Katzenstein HM, Dagan R, et al. Proton therapy following induction chemotherapy for pediatric and adolescent nasopharyngeal carcinoma. Pediatr Blood Cancer. 2019;66(12):e27990. https://doi.org/10.1002/pbc.27990.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Radiation Oncology, Acibadem Mehmet Ali Aydinlar University, School of Medicine, Istanbul, Turkey

    Enis Ozyar & Teuta Zoto Mustafayev

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  1. Enis Ozyar
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  2. Teuta Zoto Mustafayev
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Correspondence to Enis Ozyar .

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Editors and Affiliations

  1. Department of Radiation Oncology, Sun Yat-sen University Cancer Center, Guangzhou, China

    Jun Ma

  2. Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, USA

    Nancy Y. Lee

  3. Department of Radiation Oncology, Shanghai Proton and Heavy Ion Center, Shanghai, China

    Jiade J. Lu

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Ozyar, E., Mustafayev, T.Z. (2021). Special Consideration in Pediatric Nasopharyngeal Cancer. In: Ma, J., Lee, N.Y., Lu, J.J. (eds) Nasopharyngeal Cancer. Practical Guides in Radiation Oncology. Springer, Cham. https://doi.org/10.1007/978-3-030-65037-7_12

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