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Related Experiment Video

Updated: Jan 15, 2026

Role of Diffusion MRI Tractography in Endoscopic Endonasal Skull Base Surgery
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Ectopic craniopharyngiomas.

Georgia Ntali1, Taufiq Khan2, Niki Karavitaki3

  • 1Department of Endocrinology, Diabetes and Metabolism, "D. Ikkos", European Reference Network on Rare Endocrine Conditions, Endo-ERN, Evangelismos Hospital, Athens 10676, Greece; Department of Metabolism and Systems Science, College of Medicine and Health, University of Birmingham, IBR Tower, Level 2, Birmingham B15 2TT, United Kingdom; Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham B15 2TT, United Kingdom; Department of Endocrinology, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2GW, United Kingdom.

Best Practice & Research. Clinical Endocrinology & Metabolism
|October 8, 2025
PubMed
Summary

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This summary is machine-generated.

Craniopharyngiomas (CPs) are rare tumors that can occur outside the typical brain region. This review examines the distinct causes, diagnosis, and treatment of these ectopic CPs.

Area of Science:

  • Neuro-oncology
  • Developmental Biology
  • Pathology

Background:

  • Craniopharyngiomas (CPs) are rare, World Health Organization (WHO) grade 1 tumors originating from embryonic remnants in the sellar/parasellar region.
  • Ectopic CPs can occur in atypical locations such as the nasopharynx, orbit, posterior fossa, brain parenchyma, and spine.
  • These ectopic tumors present either as primary growths in unusual sites or as secondary recurrences after treatment in the orthotopic location.

Purpose of the Study:

  • To review the pathogenesis, clinical presentation, diagnosis, and management of primary and secondary ectopic craniopharyngiomas.
  • To analyze a comprehensive dataset of 97 published ectopic CP cases (42 primary, 55 secondary) from 2000-2025.

Main Methods:

  • Literature review of published cases of ectopic craniopharyngiomas.
Keywords:
adamantinomatouscraniopharyngiomaectopicpapillary

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  • Analysis of case series focusing on ectopic tumor characteristics and outcomes.

    • Main Results:

    • Primary ectopic CPs arise from embryologically misplaced tissue or genetic tumor development outside the sella.
    • Secondary ectopic CPs result from tumor cell dissemination via surgical tracts, cerebrospinal fluid (CSF), or meningeal seeding.
    • Ectopic craniopharyngiomas present unique diagnostic and therapeutic challenges.

    Conclusions:

    • Understanding the distinct pathogenesis of primary versus secondary ectopic CPs is crucial for effective management.
    • Ectopic craniopharyngiomas require tailored diagnostic and therapeutic strategies due to their atypical locations and origins.
    • Further research into the specific mechanisms and optimal treatments for these rare tumors is warranted.