Evidence of neural crest cell origin of a DICER1 mutant CNS sarcoma in a child with DICER1 syndrome and NRAS-mutant neurocutaneous melanosis

Neuropathol Appl Neurobiol. 2022 Oct;48(6):e12830. doi: 10.1111/nan.12830. Epub 2022 Jul 5.
No abstract available

Keywords: DICER1 mutant CNS sarcoma; DNA methylation profiling; NRAS; neural crest cell; neurocutaneous melanosis; pseudo-meissnerian corpuscle.

MeSH terms

  • Child
  • DEAD-box RNA Helicases
  • GTP Phosphohydrolases
  • Humans
  • Magnetic Resonance Imaging
  • Melanosis* / genetics
  • Membrane Proteins
  • Neural Crest
  • Neurocutaneous Syndromes* / genetics
  • Ribonuclease III / genetics
  • Sarcoma*

Substances

  • Membrane Proteins
  • DICER1 protein, human
  • Ribonuclease III
  • GTP Phosphohydrolases
  • NRAS protein, human
  • DEAD-box RNA Helicases

Supplementary concepts

  • Neurocutaneous melanosis