Low-grade glioma: A rare second tumor in retinoblastoma survivors

Pediatr Blood Cancer. 2021 Feb;68(2):e28770. doi: 10.1002/pbc.28770. Epub 2020 Oct 16.

Abstract

Retinoblastoma survivors with a germline RB1 mutation are at elevated risk for secondary (nonocular) malignancy, but their risk for low-grade glioma (LGG) is unknown. We performed a retrospective review of the Memorial Sloan Kettering Cancer Center and the NCI databases that revealed that three of the 837 5-year survivors of hereditary retinoblastoma were diagnosed with an LGG and a fourth patient (but unilateral and without a germline mutation) was identified at another center. Retinoblastoma survivors may be at increased risk for LGG.

Keywords: low-grade glioma; retinoblastoma; second cancers; survivorship.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, N.I.H., Intramural

MeSH terms

  • Adolescent
  • Cerebellum / pathology
  • Female
  • Genetic Predisposition to Disease / genetics
  • Glioma / genetics*
  • Glioma / pathology
  • Humans
  • Male
  • Neoplasms, Second Primary / diagnosis
  • Neoplasms, Second Primary / genetics*
  • Retinal Neoplasms / genetics*
  • Retinal Neoplasms / pathology
  • Retinal Neoplasms / therapy
  • Retinoblastoma / genetics*
  • Retinoblastoma / pathology
  • Retinoblastoma / therapy
  • Retinoblastoma Binding Proteins / genetics*
  • Temporal Lobe / pathology
  • Ubiquitin-Protein Ligases / genetics*
  • Young Adult

Substances

  • RB1 protein, human
  • Retinoblastoma Binding Proteins
  • Ubiquitin-Protein Ligases