Ganglioglioma and gangliocytoma: a review for pathologists

Gianfranco E Umeres-Francia1, Melissa Mejia-Bautista1, Pouya Jamshidi1

  • 1Department of Pathology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.

Insights

Gangliogliomas and gangliocytomas are rare brain tumors often causing epilepsy in young people. Molecular insights reveal the MAPK/ERK pathway is crucial for their development and treatment.

Area of Science:

  • Neuro-oncology
  • Molecular Pathology
  • Pediatric Neurology

Background:

  • Ganglioglioma and gangliocytoma are rare neuroepithelial tumors.
  • These tumors predominantly affect children and young adults, often presenting with epilepsy.
  • Recent molecular profiling has elucidated key pathways involved in their pathogenesis.

Purpose of the Study:

  • To provide a comprehensive review of ganglioglioma and gangliocytoma.
  • To summarize current knowledge on epidemiology, etiology, and clinical presentation.
  • To discuss diagnostic, therapeutic, and prognostic aspects.

Main Methods:

  • Literature review of epidemiology, etiology, clinical presentation, imaging, pathology, and treatment.
  • Synthesis of current understanding based on molecular profiling and clinical outcomes.
  • Analysis of diagnostic criteria and prognostic factors.

Main Results:

  • These tumors are characterized by specific molecular alterations, particularly in the MAPK/ERK pathway.
  • Diagnosis integrates clinical, radiological, and histopathological findings.
  • Complete surgical resection is associated with the best prognosis.

Conclusions:

  • Understanding the molecular basis of these tumors is critical for targeted therapies.
  • Multidisciplinary diagnosis and management are essential.
  • Further research into novel therapeutic strategies is warranted.

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