Differentiating MYCN-amplified RB1 wild-type retinoblastoma from biallelic RB1 mutant retinoblastoma using MR-based radiomics: a retrospective multicenter case-control study

Sci Rep. 2024 Oct 23;14(1):25103. doi: 10.1038/s41598-024-76933-6.

Abstract

MYCN-amplified RB1 wild-type (MYCNampRB1+/+) retinoblastoma is a rare and aggressive subtype, often resistant to standard therapies. Identifying unique MRI features is crucial for diagnosing this subtype, as biopsy is not recommended. This study aimed to differentiate MYCNampRB1+/+ from the most prevalent RB1-/- retinoblastoma using pretreatment MRI and radiomics. Ninety-eight unilateral retinoblastoma patients (19 MYCN cases and 79 matched controls) were included. Tumors on T2-weighted MR images were manually delineated and validated by experienced radiologists. Radiomics analysis extracted 120 features per tumor. Several combinations of feature selection methods, oversampling techniques and machine learning (ML) classifiers were evaluated in a repeated fivefold cross-validation machine learning pipeline to yield the best-performing prediction model for MYCN. The best model used univariate feature selection, data oversampling (duplicating MYCN cases), and logistic regression classifier, achieving a mean AUC of 0.78 (SD 0.12). SHAP analysis highlighted lower sphericity, higher flatness, and greater gray-level heterogeneity as predictive for MYCNampRB1+/+ status, yielding an AUC of 0.81 (SD 0.11). This study shows the potential of MRI-based radiomics to distinguish MYCNampRB1+/+ and RB1-/- retinoblastoma subtypes.

Keywords: MYCN-amplification; MRI; radiomics; Retinoblastoma.

Publication types

  • Multicenter Study

MeSH terms

  • Case-Control Studies
  • Child
  • Child, Preschool
  • Diagnosis, Differential
  • Female
  • Humans
  • Infant
  • Machine Learning
  • Magnetic Resonance Imaging* / methods
  • Male
  • Mutation
  • N-Myc Proto-Oncogene Protein* / genetics
  • Radiomics
  • Retinal Neoplasms / diagnostic imaging
  • Retinal Neoplasms / genetics
  • Retinal Neoplasms / pathology
  • Retinoblastoma Binding Proteins* / genetics
  • Retinoblastoma* / diagnostic imaging
  • Retinoblastoma* / genetics
  • Retinoblastoma* / pathology
  • Retrospective Studies
  • Ubiquitin-Protein Ligases* / genetics

Substances

  • N-Myc Proto-Oncogene Protein
  • RB1 protein, human
  • MYCN protein, human
  • Retinoblastoma Binding Proteins
  • Ubiquitin-Protein Ligases