Nasopharyngeal Carcinoma in Pediatric and Adolescent Patients

Cancer is the second most common cause of death among children aged 1–14 years in the United States, surpassed only by accidents, and is the fourth most common cause of death among adolescents (aged 15–19 years) (Siegel et al. 2022). Nasopharyngeal carcinoma (NPC) is rare in children accounting for less than 1% of all childhood cancers with distinct features and clinical presentation than adult population (Sultan et al. 2010). NPC arises in the epithelial lining of the nasal cavity and pharynx and accounts for about one-third of all cancers of the upper airways in children (Ayan et al. 2003). Children with NPC almost always have the WHO type III undifferentiated variant of the disease, previously called lymphoepithelioma or Schmincke tumor, and it is associated with advanced locoregional spread and distant metastases (Ayan and Altun 1996). Treatment of NPC is multimodal, and currently, cisplatin-based induction chemotherapy followed by concomitant chemoradiation is the mostly used choice of management (Cheuk et al. 2011). Although multimodality treatment has increased the 5-year OS (OS) to more than 85%, late morbidities such as endocrinopathies, hearing loss, dental problems, lifelong dry mouth, and secondary cancers are still a major concern. Immune modulation with interferon has resulted in excellent outcome, and studies have been extended to investigate the impact of immunotherapy on survival, in combination with less toxic chemoradiotherapy (Buehrlen et al. 2012). Due to the limitation of clinical evidence available in the literature especially those from prospective clinical trials, it is difficult to put forth an evidence-based treatment guideline specifically for this group of patients, and treatment strategies usually follow the guidelines established for adults. In this chapter, we aim to make a comprehensive review of the pediatric and adolescent NPC (PANPC), differences between adult and pediatric patients, as well as recommendations in terms of diagnosis, staging, management, and follow-up.