Ectopic craniopharyngiomas

Best Practice & Research Clinical Endocrinology & Metabolism, vol. 39, pp. 102047

Abstract

Craniopharyngiomas (CPs) are rare embryonic malformational tumours of the sellar/parasellar region, classified by the World Health Organization (WHO) 5th edition as grade 1 tumours. They may arise anywhere on the path of embryonic cell migration between the midline sphenoid bone and the floor of the sella turcica. On rare occasions, they can be found remotely in areas like the nasopharynx, the orbit, the posterior fossa, the brain parenchyma and the spine. Such ectopic CPs may present as primary in an atypical location, or as secondary, which represent distant recurrence after initial surgical excision in an orthotopic location. The pathogenesis of primary and secondary ectopic CPs is considered distinct, reflecting differences in developmental biology versus tumour dissemination. Primary ectopic CPs are thought to arise either from embryologically misplaced tissue or from genetically driven tumourigenesis outside the sella. Pathogenesis of secondary ectopic CPs reflects tumour cell dissemination through surgical tract implantation, cerebrospinal fluid (CSF) seeding or meningeal seeding. Ectopic cases pose unique diagnostic and therapeutic challenges. In this review which encompasses 97 cases (42 primary and 55 secondary ectopic CPs) published during the period, 2000-2025, we discuss the pathogenesis, clinical presentation, diagnosis and management of these distinctive tumours.

Authors 4

1. Georgia Ntali Aachen

   University Hospitals Birmingham NHS Foundation Trust · Queen Elizabeth Hospital Birmingham · Evangelismos Hospital · Endo-ERN · University of Birmingham

   Affiliation as printed

   Department 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. Electronic address: georgiantali@yahoo.com

2. Taufiq Khan

   University Hospitals Birmingham NHS Foundation Trust

   Affiliation as printed

   Department of Neurosurgery, University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2GW, United Kingdom. Electronic address: taufiq.khan@uhb.nhs.uk

3. Niki Karavitaki corresponding

   University Hospitals Birmingham NHS Foundation Trust · Queen Elizabeth Hospital Birmingham · University of Birmingham

   Affiliation as printed

   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. Electronic address: n.karavitaki@bham.ac.uk

4. Georgios Tsermoulas

   University Hospitals Birmingham NHS Foundation Trust · University of Birmingham

   Affiliation as printed

   Department of Metabolism and Systems Science, College of Medicine and Health, University of Birmingham, IBR Tower, Level 2, Birmingham B15 2TT, United Kingdom; Department of Neurosurgery, University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2GW, United Kingdom. Electronic address: georgios.tsermoulas@nhs.net