Abstract

Advanced techniques and treatment methods have been found to be associated with improved survival rates in adults with nasopharyngeal carcinoma (NPC); however, not much is known about associations in pediatric patients. To investigate whether advanced imaging diagnosis, radiotherapy (RT) technology, and treatment modality are associated with survival in pediatric patients with NPC. In this retrospective cohort study, 810 pediatric patients ages 21 years and younger with nonmetastatic NPC diagnosed from 1989 to 2020 at a single cancer center in China were included. Data were analyzed from April through December 2021. Patients were divided into 3 groups by initial treatment date (ie, 1989-2002, 2003-2011, and 2012-2020). Associations between advances in technology and treatment and survival were investigated. Comparisons of advances vs older technology and treatments included those in imaging diagnosis (magnetic resonance imaging [MRI] vs computed tomography [CT] and positron emission tomography [PET]-CT with MRI vs CT), radiotherapy (RT) techniques (intensity-modulated RT [IMRT] or TomoTherapy [TOMO] vs 2-dimensional conventional radiotherapy [2D-CRT] or 3-dimensional conventional radiotherapy [3D-CRT]), and treatment methods (concurrent chemoradiotherapy [CCRT] vs RT alone, induction chemotherapy [IC] with CCRT vs RT alone, and CCRT or RT with adjuvant chemotherapy [AC] vs RT alone). The primary end point was progression-free survival (PFS). Secondary end points were overall survival (OS), distant metastasis-free survival, and locoregional recurrence-free survival. Cox and competing-risks regression were used to estimate hazard ratios (HRs) and 95% CIs for associations between variables and survival. Among 810 pediatric patients with NPC, the median (IQR) age was 18 (15-20) years, and there were 577 [71.2%] male patients. This included 122 patients in the 1989 to 2002 period, 212 patients in the 2003 to 2011 period, and 476 patients in the 2012 to 2020 period. The 5-year PFS and OS rates increased, respectively, from 65.9% (95% CI, 56.6%-73.7%) and 69.9% (95% CI, 60.7%-77.4%) in 1989 to 2002 to 79.8% (95% CI, 73.7%-84.7%) and 86.2% (95% CI, 80.6%-90.3%) in 2003 to 2011, then 88.1% (95% CI, 84.2%-91.1%) and 95.0% (95% CI, 91.5%-97.0%) in 2012 to 2020. The 5- year cumulative incidence of distant metastasis rate was similar in the 3 periods (1989-2002: 11.7% [95% CI, 7.0%- 19.4%]; 2003-2011: 18.0% [95% CI, 13.4%-24.0%]; 2012-2020: 10.4% [95% CI, 7.6%-14.1%], while the 5-year cumulative incidence of locoregional recurrence rate decreased from 22.5% (95% CI, 15.9%-31.3%) in the first period to 2.9% (95% CI, 1.3%-6.3%) in the second period, remaining stable in the third period, at 4.3% (95% CI, 2.4%-7.6%). Advances in imaging diagnosis (MRI vs CT: hazard ratio [HR], 0.25 [95% CI, 0.17-0.38]; PET-CT with MRI vs CT: HR, 0.41 [95% CI, 0.27-0.62]), radiotherapy techniques (IMRT or TOMO vs 2D-CRT or 3D-CRT: HR, 0.42 [95% CI, 0.30-0.59]), and treatment methods (CCRT vs RT alone: HR, 0.55 [95% CI, 0.32-0.96]; IC with CCRT vs RT alone: HR, 0.59 [95% CI, 0.38-0.91]; CCRT or RT with AC vs RT alone: HR, 0.48 [95% CI, 0.25-0.91]) were associated with improved PFS. This study found that advanced techniques and treatment methods were associated with improved survival rates in pediatric patients with NPC, but distant failure remained a key challenge.

Highlights

  • Nasopharyngeal carcinoma (NPC) is considered a rare type of malignant neoplasm globally, but it is endemic in Southeastern Asia and Eastern Asia, especially in some provinces in southeastern China, with a peak incidence in individuals aged 40 to 60 years.1 a subpeak is found among individuals between the ages of 10 and 20 years.2 Owing to the hidden location of nasopharyngeal carcinoma (NPC), the nonspecific nature of nasal and auditory symptoms, and the difficulty of clinical examination of the nasopharynx, most individuals have locoregionally advanced stages at diagnosis.3 Compared with adults, pediatric patients with NPC seem more likely to have advanced stages with more lymph node metastasis.4 In endemic areas, most pathological cases in pediatric patients are nonkeratinizing subtypes, which are always associated with Epstein-Barr virus (EBV) infection

  • The 5- year cumulative incidence of distant metastasis rate was similar in the 3 periods

  • This study found that advanced techniques and treatment methods were associated with improved survival rates in pediatric patients with NPC, but distant failure remained a key challenge

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Summary

Introduction

Nasopharyngeal carcinoma (NPC) is considered a rare type of malignant neoplasm globally, but it is endemic in Southeastern Asia and Eastern Asia, especially in some provinces in southeastern China, with a peak incidence in individuals aged 40 to 60 years. a subpeak is found among individuals between the ages of 10 and 20 years. Owing to the hidden location of NPC, the nonspecific nature of nasal and auditory symptoms, and the difficulty of clinical examination of the nasopharynx, most individuals have locoregionally advanced stages at diagnosis. Compared with adults, pediatric patients with NPC seem more likely to have advanced stages (ie, stages III and IV) with more lymph node metastasis. In endemic areas, most pathological cases in pediatric patients are nonkeratinizing subtypes, which are always associated with Epstein-Barr virus (EBV) infection. Nasopharyngeal carcinoma (NPC) is considered a rare type of malignant neoplasm globally, but it is endemic in Southeastern Asia and Eastern Asia, especially in some provinces in southeastern China, with a peak incidence in individuals aged 40 to 60 years.. Owing to the hidden location of NPC, the nonspecific nature of nasal and auditory symptoms, and the difficulty of clinical examination of the nasopharynx, most individuals have locoregionally advanced stages at diagnosis.. Pediatric patients with NPC seem more likely to have advanced stages (ie, stages III and IV) with more lymph node metastasis.. External radiotherapy (RT) is the primary curative treatment method, and with RT alone, the 10-year disease-specific survival rate among pediatric patients with NPC patients was reported to be 98% with stage I disease but 60% with stage II disease.

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