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

• Published in: Journal of neurosurgery. Case lessons Vol. 3; no. 1
• Main Authors: Uemura, Hiroya, Tanji, Masahiro, Natsuhara, Hiroki, Takeuchi, Yasuhide, Hoki, Masahito, Sugimoto, Akihiko, Minamiguchi, Sachiko, Kawasaki, Hidenori, Torishima, Masako, Kosugi, Shinji, Mineharu, Yohei, Arakawa, Yoshiki, Yoshida, Kazumichi, Miyamoto, Susumu
• Format: Journal Article
• Language: English
• Published: American Association of Neurological Surgeons 01.01.2022
• Subjects: Case Lesson
• ISSN: 2694-1902; 2694-1902
• DOI: 10.3171/CASE21572

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.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.The 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.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.An ectopic CP may be reasonably included in the differential diagnosis in patients with FAP who present with well-circumscribed tumors in the posterior fossa.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.