Increased Incidence and Improved Survival of Ewing and Ewing-Like Sarcomas in Children and Young Adolescents in the Netherlands Since the 1990s: A Population-Based Study.

@article{schepwijnveen2026increased,
  title = {{Increased Incidence and Improved Survival of Ewing and Ewing-Like Sarcomas in Children and Young Adolescents in the Netherlands Since the 1990s: A Population-Based Study.}},
  author = {Hanna J M Schep-Wijnveen and Lianne M Haveman and Maya Schulpen and Marijn H Geyskens and Otto Visser and Lizz van der Heijden and Arthur J A T Braat and Laura S Hiemcke-Jiwa and Raquel Dávila Fajardo and Simone A J Ter Horst and Sheila C E J Terwisscha van Scheltinga and Rutger R G Knops and Jos A M Bramer and Hendrik W B Schreuder and Michiel A J van de Sande and Johannes H M Merks and Henrike E Karim-Kos},
  journal = {Cancer medicine},
  year = {2026},
  month = nov,
  doi = {10.1002/cam4.72429},
  url = {https://europepmc.org/article/MED/42967889},
  note = {CC BY-NC},
}

TY  - JOUR
TI  - Increased Incidence and Improved Survival of Ewing and Ewing-Like Sarcomas in Children and Young Adolescents in the Netherlands Since the 1990s: A Population-Based Study.
AU  - Hanna J M Schep-Wijnveen
AU  - Lianne M Haveman
AU  - Maya Schulpen
AU  - Marijn H Geyskens
AU  - Otto Visser
AU  - Lizz van der Heijden
AU  - Arthur J A T Braat
AU  - Laura S Hiemcke-Jiwa
AU  - Raquel Dávila Fajardo
AU  - Simone A J Ter Horst
AU  - Sheila C E J Terwisscha van Scheltinga
AU  - Rutger R G Knops
AU  - Jos A M Bramer
AU  - Hendrik W B Schreuder
AU  - Michiel A J van de Sande
AU  - Johannes H M Merks
AU  - Henrike E Karim-Kos
PY  - 2026
DA  - 2026/11/01
JO  - Cancer medicine
DO  - 10.1002/cam4.72429
UR  - https://europepmc.org/article/MED/42967889
AB  - Against the background of therapeutic improvements and centralization of care, we evaluated epidemiological trends in Ewing and Ewing-like sarcomas (ES) among children and young adolescents in the Netherlands over three decades. Data on 464 pediatric patients (0-17 years) diagnosed with ES in the Netherlands between 1990 and 2018 were obtained from the Netherlands Cancer Registry, with follow-up until February 2024. Incidence trends were evaluated by calculating the average annual percentage change (AAPC). A Cox regression model was used to assess the effect of sex, age, stage at diagnosis, and tissue-specific tumor site on changes in 5-year observed overall survival (OS) over time. Between 1990 and 2018, on average 16 pediatric patients were diagnosed with ES yearly. The age-standardized incidence rate increased by 2.1% annually, rising from 3.7 per million person-years in 1990-1999 to 5.2 in 2010-2018. Over time, the use of local therapy combined with chemotherapy increased from 75% in 1990-1999 to 94% in 2010-2018. Five-year OS improved from 56% (1990-1999) to 70% (2010-2018), and the 10-year OS increased from 51% to 65% in these periods. Multivariable analyzes confirmed a lower risk of mortality from ES in 2010-2018 (HR 0.4, 95% CI 0.3-0.6) compared to 1990-1999. ES incidence increased alongside improved survival, likely influenced by various factors, including advancements in diagnostics, treatment, supportive care, and follow-up. Nevertheless, patients with metastatic disease and older children continue to experience higher mortality risks, highlighting the importance of ongoing research and targeted treatments to further improve pediatric ES outcomes.
SN  - 2045-7634
ER  -