A Hemorrhagic Brain Mass in a Child With Encephalocraniocutaneous Lipomatosis

J Pediatr Hematol Oncol. 2024 Oct 1;46(7):e541-e543. doi: 10.1097/MPH.0000000000002947. Epub 2024 Aug 27.

Abstract

Encephalocraniocutaneous lipomatosis (ECCL) is a rare genetic condition with well-described skin, ocular, and central nervous system findings. Several case reports have been documented demonstrating the presence of low-grade gliomas in patients with ECCL and the association with certain FGFR1 mutations. We report on a case of diffuse low-grade glioma, mitogen activated protein kinase pathway altered in a patient with ECCL, who was found to have a distinct FGFR1 mutation.

Publication types

  • Case Reports

MeSH terms

  • Brain Neoplasms / complications
  • Brain Neoplasms / genetics
  • Brain Neoplasms / pathology
  • Eye Diseases / etiology
  • Eye Diseases / pathology
  • Female
  • Glioma / complications
  • Glioma / genetics
  • Glioma / pathology
  • Humans
  • Lipomatosis* / complications
  • Lipomatosis* / genetics
  • Lipomatosis* / pathology
  • Male
  • Mutation
  • Neurocutaneous Syndromes* / complications
  • Neurocutaneous Syndromes* / genetics
  • Neurocutaneous Syndromes* / pathology
  • Primary Immunodeficiency Diseases / complications
  • Primary Immunodeficiency Diseases / genetics
  • Primary Immunodeficiency Diseases / pathology
  • Receptor, Fibroblast Growth Factor, Type 1 / genetics

Substances

  • Receptor, Fibroblast Growth Factor, Type 1
  • FGFR1 protein, human

Supplementary concepts

  • Encephalocraniocutaneous lipomatosis