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Griffith R Harsh, IV, MD, MBA
Lawrence D Recht, MD
Karen J Marcus, MD
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Deputy Editor
April F Eichler, MD, MPH


Craniopharyngiomas are rare solid or mixed solid-cystic tumors that arise from remnants of Rathke's pouch along a line from the nasopharynx to the diencephalon [1]. Historically, these have also been referred to as Rathke pouch tumors or hypophyseal duct tumors.

The epidemiology, pathology, diagnosis, management, and outcomes after treatment of craniopharyngiomas are reviewed here.


In the United States, there are an estimated 350 new cases of craniopharyngioma diagnosed each year, constituting approximately 1 to 3 percent of all brain tumors [2,3]. Craniopharyngiomas comprise about 5 to 10 percent of brain tumors in children [3,4]. In other parts of the world, craniopharyngiomas appear to be more common, with higher incidence rates reported in Japan and some parts of Africa [5,6]. Craniopharyngiomas are approximately equally common in males and females.

There is a bimodal age distribution, with one peak in children between 5 and 14 years, and a second peak in adults between 50 and 75 years of age [2]. Adamantinomatous craniopharyngiomas are more common in the pediatric age group, while papillary craniopharyngiomas are seen predominantly in adults [7]. (See 'Pathology' below.)


Craniopharyngiomas are epithelial tumors that usually arise in the pituitary stalk in the suprasellar region, adjacent to the optic chiasm. A small percentage arise within the sella [8,9], and a few tumors have been described within the optic system or the third ventricle [10-12].


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Literature review current through: Sep 2016. | This topic last updated: Aug 11, 2016.
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