Familial Nasopharyngeal Carcinoma 6

Nasopharyngeal Cancer in Pediatric 23and Adolescent PatientsEnis Ozyar and Inci AyanCONTENTS23.1 Introduction 29523.2 Etiological and EpidemiologicalFeatures 29523.3 Treatment Strategy 29723.4 Radiation Therapy 29723.5 Systemic Treatments 30023.6 Treatment-Induced Adverse Effects 30523.6.1 Acute Toxicities 30523.6.2 Late Toxicities 30523.7 Summary 306References 30623.1IntroductionNasopharyngeal carcinoma (NPC) is a rare disease inchildren with distinct epidemiological and etiologicalfeatures, histopathological characteristics, and clinicalpresentation. The incidence of pediatric NPC varieswidely around the world, and both genetic and environmentalfactors contribute to the development ofthe disease. Children with NPC almost always have theundifferentiated variant of the disease, which is associatedwith advanced locoregional spread and distantmetastases. Currently, cisplatin-based induction chemotherapyfollowed by high-dose radiotherapy is thetreatment of choice. Although multimodality treatmenthas increased the 5-year survival to 70%–90%,late morbidity is a major concern. Immune-modulationwith interferon has resulted in excellent outcome, andstudies have been extended to investigate the impactof immunotherapy on survival, in combination withless toxic chemoradiotherapy.The aim of this chapter is to address the currentmanagement strategy of pediatric NPC, as well asdiscuss the future direction of research for improvedtreatment of the disease.23.2Etiological and Epidemiological FeaturesEnis Ozyar, MDProfessor of Radiation Oncology, Acibadem Maslak HospitalAcibadem University,Department of Radiation OncologyMaslak, 34457, TurkeyInci Ayan MDProfessor of Pediatric Oncology, Acibadem Maslak HospitalAcibadem University, Department of Pediatrics, Maslak,34457, TurkeyNPC is one of the few malignancies in childhood thatemerge from the epithelium, and constitutes 1%–5%of all pediatric cancers and 20%–50% of all primarynasopharyngeal malignant tumors in children (Ayanet al. 2003). Several studies indicated the distinctclinical features of pediatric NPC when comparedwith its adult counterpart (Ayan and Altun 1996).Young patients have a higher rate of lymph node