Opposite Incidence Trends for Differentiated and Medullary Thyroid Cancer in Young Dutch Patients over a 30-Year Time Span

SIMPLE SUMMARY: Thyroid cancer is a rare disease in childhood; however, its incidence is rising. Thyroid cancer consists of three main types: Papillary thyroid cancer (PTC), follicular thyroid cancer (FTC), and medullary thyroid cancer (MTC). The aim of our retrospective study was to investigate the incidence and survival trends of these three thyroid cancer types in Dutch children, adolescents, and young adults over a 30-year life span. In total, 839 patients aged 0–24 years had been diagnosed with thyroid cancer between 1990 and 2019. The incidence of PTC increased significantly over time, the incidence of FTC showed a stable trend, while the incidence of MTC decreased significantly. Overall, the 10-year survival rates over the last decades were high (>95%) for PTC, FTC, and MTC in young individuals. ABSTRACT: Thyroid cancer is the most common endocrine malignancy in children. A rising incidence has been reported worldwide. Possible explanations include the increased use of enhanced imaging (leading to incidentalomas) and an increased prevalence of risk factors. We aimed to evaluate the incidence and survival trends of thyroid cancer in Dutch children, adolescents, and young adults (0–24 years) between 1990 and 2019. The age-standardized incidence rates of differentiated thyroid cancer (DTC, including papillary and follicular thyroid cancer (PTC and FTC, respectively)) and medullary thyroid cancer (MTC), the average annual percentage changes (AAPC) in incidence rates, and 10-year overall survival (OS) were calculated based on data obtained from the nationwide cancer registry (Netherlands Cancer Registry). A total of 839 patients aged 0–24 years had been diagnosed with thyroid carcinoma (PTC: 594 (71%), FTC: 128 (15%), MTC: 114 (14%)) between 1990 and 2019. The incidence of PTC increased significantly over time (AAPC +3.6%; 95%CI +2.3 to +4.8), the incidence rate of FTC showed a stable trend ((AAPC −1.1%; 95%CI −3.4 to +1.1), while the incidence of MTC decreased significantly (AAPC: −4.4% (95%CI −7.3 to −1.5). The 10-year OS was 99.5% (1990–1999) and 98.6% (2000–2009) in patients with DTC and 92.4% (1990–1999) and 96.0% (2000–2009) in patients with MTC. In this nationwide study, a rising incidence of PTC and decreasing incidence of MTC were observed. For both groups, in spite of the high proportion of patients with lymph node involvement at diagnosis for DTC and the limited treatment options for MTC, 10-year OS was high.