The association of ectopic craniopharyngioma in the fourth ventricle with familial adenomatous polyposis: illustrative case

Yoshiki Arakawa,Susumu Miyamoto,Sachiko Minamiguchi,Yasuhide Takeuchi,Hidenori Kawasaki,Shinji Kosugi,Hiroya Uemura,Yohei Mineharu,Hiroki Natsuhara,Kazumichi Yoshida,Masahito Hoki,Masahiro Tanji,Akihiko Sugimoto,Masako Torishima

doi:10.3171/case21572

Yoshiki Arakawa, Susumu Miyamoto + Show 12 more

Open Access

https://doi.org/10.3171/case21572

Copy DOI

Abstract

BACKGROUNDCraniopharyngioma (CP) often arises in the sellar and suprasellar areas; ectopic CP in the posterior fossa is rare. Familial adenomatous polyposis (FAP) is a genetic disorder involving the formation of numerous adenomatous polyps in the gastrointestinal tract, and it is associated with other extraintestinal manifestations.OBSERVATIONSThe authors reported the case of a 63-year-old woman with FAP who presented with headache and harbored a growing mass in the fourth ventricle. Magnetic resonance imaging (MRI) findings revealed a well-circumscribed mass with high intensity on T1-weighted images and low intensity on T2-weighted images and exhibited no contrast enhancement. Gross total resection was performed and histopathology revealed an adamantinomatous CP (aCP). The authors also reviewed the previous reports of ectopic CP in the posterior fossa and found a high percentage of FAP cases among the ectopic CP group, thus suggesting a possible association between the two diseases.LESSONSAn ectopic CP may be reasonably included in the differential diagnosis in patients with FAP who present with well-circumscribed tumors in the posterior fossa.

Highlights

Craniopharyngioma (CP) often arises in the sellar and suprasellar areas; ectopic CP in the posterior fossa is rare
The common site of CP is around the sella turcica of the pituitary stalk, primary ectopic CP occurring in other locations in the cranium has been reported, including in the nasopharynx, sphenoid bone, and third ventricle.[7,8,9]
The high incidence of Familial adenomatous polyposis (FAP) among patients with ectopic CP in the posterior fossa suggests an association between these two rare diseases

Summary

BACKGROUND

Craniopharyngioma (CP) often arises in the sellar and suprasellar areas; ectopic CP in the posterior fossa is rare. Familial adenomatous polyposis (FAP) is a genetic disorder involving the formation of numerous adenomatous polyps in the gastrointestinal tract, and it is associated with other extraintestinal manifestations. The authors reviewed the previous reports of ectopic CP in the posterior fossa and found a high percentage of FAP cases among the ectopic CP group, suggesting a possible association between the two diseases. An ectopic CP arising from other sites is rare; CP of the posterior fossa is even rarer and has only been reported in 19 cases so far.[2,3] there have only been five reports of CP arising from the fourth ventricle.[4,5]. Unauthenticated | Downloaded 02/08/22 09:47 AM UTC on ectopic CP and discuss the possible relationship between these rare diseases

Illustrative Case

Discussion

Findings

Present case