Brain Sciences (Apr 2023)

Pediatric Posterior Fossa ATRT: A Case Report, New Treatment Strategies and Perspectives

  • Luca Paun,
  • Alexandre Lavé,
  • Gianpaolo Jannelli,
  • Kristof Egervari,
  • Insa Janssen,
  • Karl Schaller,
  • André O. von Bueren,
  • Andrea Bartoli

DOI
https://doi.org/10.3390/brainsci13050712
Journal volume & issue
Vol. 13, no. 5
p. 712

Abstract

Read online

Posterior fossa atypical teratoid rhabdoid tumor (ATRT) is a rare childhood tumor usually associated with a dismal prognosis. Although upfront surgical gross total resection (GTR) has classically been the first line of treatment, new multimodal treatments, including two-stage surgery, are showing promising results in terms of overall survival (OS) and complication rate. We present a case of a 9-month-old child treated with two-staged surgery and chemotherapy. When deemed risky, multimodal treatments, including staged surgeries, can be a safe alternative to reduce surgical mortality and morbidity. At 23 months old, the patient had normal global development and no major impact on quality of life. We, therefore, discuss the most recent advancements from a treatment perspective, including molecular targeting.

Keywords