Craniopharyngioma

Heather Sonnemann BS (Ms. Sonnemann of the National Cancer Institute has no relevant financial relationships to disclose.)
Deric M Park MD (Dr. Park of the National Cancer Institute has no relevant financial relationships to disclose.)
Rimas V Lukas MD, editor. (Dr. Lukas has received honorariums from AstraZeneca as an advisory board member and AbbVie as a guest speaker.)
Originally released July 28, 1994; last updated May 15, 2017; expires May 15, 2020

This article includes discussion of craniopharyngioma, Rathke pouch tumors, hypophyseal duct tumors, adamantinomas. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Craniopharyngioma is a rare histologically low-grade (WHO grade 1) suprasellar tumor believed to originate from Rathke's pouch. It occurs in adamantinomatous and papillary subtypes. Consistent with its location, patients experience visual abnormalities, symptoms of increased intracranial pressure, and endocrine dysfunction. If the tumor is favorably located, the most effective treatment is adequate resection. In this article, the authors present the protean clinical manifestations, suspected pathogenesis, and currently available treatment options for craniopharyngioma, with emphasis on the importance of a multidisciplinary approach. Genetic analysis has shown that adamantinomatous and papillary craniopharyngiomas have distinct tumor biology. Adamantinomatous craniopharyngiomas demonstrate activating mutations in the gene encoding β-catenin. On the other hand, the majority of papillary craniopharyngiomas harbor the oncogenic BRAF V600E substitution, which can be used as a confirmatory test using immunochemistry and shows promise as targeted therapy in selected patients.

Key points

 

• Due to its suprasellar location, patients with craniopharyngioma often present with visual and endocrine symptoms.

 

• There are 2 craniopharyngioma subtypes: adamantinomatous and papillary.

 

• Adamantinomatous craniopharyngiomas have an activating mutation in the gene encoding β-catenin whereas papillary craniopharyngiomas carry the BRAF V600E mutation.

 

• Most patients are treated by surgery followed with irradiation.

 

• The role of chemotherapy remains unclear.

Historical note and terminology

Craniopharyngioma is a rare parasellar tumor of low histological grade that came into attention towards the end of 19th century. It was first reported by a German pathologist, Friedrich Albert von Zenker in 1857, who described a cystic suprasellar mass containing cholesterol crystals and squamous epithelium in an autopsy. In 1860, Luschka extensively studied squamous epithelial cells in the adenohypophysis.

Pioneering work by Jakob Erdheim in 1904 led to accurate histopathological characterization of craniopharyngiomas. The first successful surgical resection of a craniopharyngioma was performed by A E Halstead in 1909 by an intranasal approach. The current terminology of craniopharyngioma was introduced by Harvey Cushing in 1932. Prior to this, craniopharyngiomas had been called interpeduncular cyst, dysontogenetic cyst, craniobuccal cyst, Rathke's pouch tumor, hypophyseal duct tumor, Erdheim tumor, craniopharyngeal duct tumor, and adamantinomas (Lindholm and Nielsen 2009; Barkhoudarian and Law 2013).

The content you are trying to view is available only to logged in, current MedLink Neurology subscribers.

If you are a subscriber, please log in.

If you are a former subscriber or have registered before, please log in first and then click select a Service Plan or contact Subscriber Services. Site license users, click the Site License Acces link on the Homepage at an authorized computer.

If you have never registered before, click Learn More about MedLink Neurology  or view available Service Plans.