Craniopharyngioma treatment: an updated summary of important clinicopathological concepts

ABSTRACT

Introduction

Craniopharyngiomas (CPs) are benign histological tumors that may develop at different positions along the hypothalamic-pituitary axis. Their close, heterogenous relationship to the hypothalamus makes surgical removal challenging even though this remains the primary treatment strategy.

Areas covered

This article presents a critical overview of the pathological and clinical concepts regarding CPs that should be considered when planning treatment. Thus, we have performed a comprehensive review of detailed CP reports published between 1839 and 2020.

Expert opinion

CP surgery should pursue maximal tumor resection while minimizing the risk of injuring the hypothalamus. Therefore, surgical strategies should be individualized for each patient. Accurate assessment of presenting symptoms and preoperative MRI has proven useful to predict the type of CP-hypothalamus relationship that will be found during surgery. CPs with dense and extensive adhesions to the hypothalamus should be highly suspected when MRI shows the hypothalamus positioned around the mid-third of the tumor and an amputated upper portion of the pituitary stalk. Symptoms related to functional impairment of the infundibulo-tuberal area of the third ventricle floor, such as obesity/hyperphagia, Fröhlich’s syndrome, diabetes insipidus, and/or somnolence, also indicate risky CP-hypothalamic adhesions. In these cases, limited tumor removal is strongly advocated followed by radiation therapy.

KEYWORDS:

• Craniopharyngioma
• hypothalamus
• hypothalamic syndrome
• infundibulo-tuberal syndrome
• pituitary gland
• pituitary syndrome
• third ventricle

Acknowledgments

The authors wish to especially thank Crystal Smith and Liliya Gusakova, reference librarians at the National Library of Medicine, National Institutes of Health (Bethesda, MD), as well as Lucretia Maclure and Jack Eckert, librarians at the Francis Countway Medical Library at Harvard Medical School (Boston, MA) and Melissa Grafe, head of the Medical Historical Library of the Cushing/Whitney Medical Library, Yale University (New Haven, CT) for their invaluable assistance during the process of searching and retrieving the original articles and monographs used in this study. We are also indebted to Eduard Winter from the Pathologisch-anatomische Sammlung im Narrenturm-NHM, Vienna, Austria, for allowing us access to the brain tumor specimens stored at the Narrenturm. Finally, we are grateful to George Hamilton for his critical review of the language and style of the manuscript.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Disclosure

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.