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International benchmarking of stage at diagnosis for six childhood solid tumours (the BENCHISTA project): a population-based, retrospective cohort study.

Lancet Child Adolesc Health

February 2025

Developmental Biology and Cancer Research & Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London, UK. Electronic address:

Background: International variation in childhood cancer survival might be explained by differences in stage at diagnosis, among other factors. As part of the BENCHISTA project, we aimed to assess geographical variation in tumour stage at diagnosis through the application, by population-based cancer registries working with clinicians, of the international consensus Toronto Childhood Cancer Stage Guidelines.

Methods: This population-based, retrospective cohort study involved 67 cancer registries from 23 European countries, Australia, Brazil, Japan, and Canada.

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Incidence and survival of European adolescents and young adults diagnosed with sarcomas: EUROCARE-6 results.

Eur J Cancer

January 2025

Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.

Background: Epidemiological data for sarcoma in adolescents and young adults (AYAs) and across age groups are limited. We aim to: 1) update sarcoma incidence, survival, and changes over time in European AYAs; 2) provide an updated comparison of sarcoma survival in AYAs versus children and mature adults.

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Rhabdomyosarcoma (RMS) is a family of phenotypically myogenic paediatric cancers consisting of two major subtypes: fusion-positive (FP) RMS, most commonly involving the PAX3::FOXO1 fusion gene, formed by the fusion of paired box 3 (PAX3) and forkhead box O1 (FOXO1) genes, and fusion-negative (FN) RMS, lacking these gene fusions. In humans, DNA methylation patterns distinguish these two subtypes as well as mutation-associated subsets within these subtypes. To investigate the biological factors responsible for these methylation differences, we profiled DNA methylation in RMS tumours derived from genetically engineered mouse models (GEMMs) in which various driver mutations were introduced into different myogenic lineages.

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Article Synopsis
  • Rhabdomyosarcoma (RMS) is a rare and aggressive pediatric cancer that commonly affects children and adolescents, with alveolar rhabdomyosarcoma (ARMS) often developing in areas like the trunk and head/neck.
  • An 11-year-old girl diagnosed with ARMS presented with a perianal mass, showing aggressive metastasis to the pleura, lymph nodes, and even bones, which is less common.
  • The case underscores the difficulties in diagnosing and treating ARMS, stressing the importance of early detection, advanced imaging techniques, and the need for ongoing research to develop better treatment options.
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Pediatric Meningeal Diseases: What Radiologists Need to Know.

Tomography

December 2024

Department of Radiology, Nemours Children's Health, 1600 Rockland Rd., Wilmington, DE 19803, USA.

Evaluating altered mental status and suspected meningeal disorders in children often begins with imaging, typically before a lumbar puncture. The challenge is that meningeal enhancement is a common finding across a range of pathologies, making diagnosis complex. This review proposes a categorization of meningeal diseases based on their predominant imaging characteristics.

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