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  • Updated 07.11.2019
  • Released 07.28.1994
  • Expires For CME 07.11.2022

Craniopharyngioma

Introduction

This article includes discussion of craniopharyngioma, Rathke pouch tumors, hypophyseal duct tumors, and 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 (Sekin et al 2002; 12; 29; 75). 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 (09; 75).

Key points

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

• There are two 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 1929 (71). Prior to this, craniopharyngioma was referred to as interpeduncular cyst, dysontogenetic cyst, craniobuccal cyst, Rathke’s pouch tumor, hypophyseal duct tumor, Erdheim tumor, craniopharyngeal duct tumor, and adamantinomas (54; Barkhoudarian and Law 2013).

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