Cerebellopontine angle craniopharyngioma in familial adenomatous polyposis.

Surg Neurol Int

Department of Neurosurgery, Salford Royal NHS Foundation Trust, Salford, United Kingdom.

Published: September 2024

AI Article Synopsis

  • Craniopharyngiomas are usually benign tumors located in the sellar and suprasellar regions, but rare instances of ectopic tumors can occur, particularly associated with familial adenomatous polyposis (FAP).
  • The text discusses a case of an ectopic cerebellopontine angle (CPA) craniopharyngioma in a patient with FAP, marking it as the fifth such documented case.
  • This case underlines the importance of considering craniopharyngioma in differential diagnoses for CPA tumors, especially in patients with FAP, suggesting a potential link to genetic mutations.

Article Abstract

Background: Craniopharyngiomas are benign tumors arising in the sellar and suprasellar regions. Although ectopic tumors do occur, it is usually due to local spread or recurrent tumors. Purely ectopic cerebellopontine angle (CPA) or 4 ventricle tumors are extremely rare and have been found to be significantly associated with familial adenomatous polyposis (FAP), a genetic disorder.

Case Description: Only four cases of ectopic CPA craniopharyngioma associated with FAP have been reported to date. Here, we present the 5 case of ectopic CPA craniopharyngioma on a background of FAP. The previously described cases have been elaborated as well.

Conclusion: CPA tumor with a background of FAP should raise a differential diagnosis of craniopharyngioma, and similarly, a CPA primary ectopic craniopharyngioma may raise suspicion of underlying APC gene mutation.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11450800PMC
http://dx.doi.org/10.25259/SNI_315_2024DOI Listing

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