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Update on management of craniopharyngiomas

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Abstract

Purpose/introduction

Craniopharyngiomas are locally-aggressive tumors arising along the hypothalamic-pituitary axis. Treatment is nuanced as a result of their proximity and adherence to vital neurovascular structures and responsiveness to surgery, radiation and, in some cases, chemotherapy.

Methods

We reviewed the literature discussing the current state of knowledge regarding craniopharyngioma biology and therapy.

Results

Recent advances in endoscopic endonasal surgery (EEA) have made surgery a safer and more effective option. While cure may be achieved with gross total resection (GTR), when felt to be too risky, a subtotal resection followed by radiation is often a more prudent strategy, particularly in children with hypothalamic invasion. Data on long-term outcome are mostly derived from older studies in which a craniotomy, rather than EEA, was performed. Long-term EEA outcome studies are lacking. Enhanced knowledge of the biological basis of papillary CPs has led to novel medical treatments for BRAFv600E variants that appear to be effective.

Conclusion

Endoscopic technology has improved surgical results for craniopharyngiomas and expanded the indications for the transsphenoidal approach. The goal of CP surgery goal is maximal safe resection to achieve cure, but subtotal resection and radiation may be equally effective. Early diagnosis of specific variants will facilitate enrollment in promising medical trials.

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Data availability

Data sharing not applicable to this article as no datasets were generated or analysed during the current study.

Abbreviations

EOR:

Extent of resection

CP:

Craniopharyngioma

aCP:

Adamantinomatous craniopharyngioma

pCP:

Papillary craniopharyngioma

MRI:

Magnetic resonance imaging

HPA:

Hypothalamic pituitary axis

GTR:

Gross total resection

STR:

Subtotal resection

EEA:

Endonasal endoscopic approach

TCA:

Transcranial approach

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Acknowledgements

The authors acknowledge the medical artist Matthew Holt.

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  1. Department of Neurological Surgery, Weill Cornell Medicine, NewYork-Presbyterian Hospital, 525 East 68th St, Box #99, New York, NY, 10065, USA

    Fraser Henderson Jr. & Theodore H. Schwartz

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  1. Fraser Henderson Jr.
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FH and TS designed and conceived of this manuscript. FH performed the literature review and compiled the primary manuscript. FH and TS analyzed data. FH and TS compiled the figures and tables. All authors critically revised the manuscript and approved the manuscript as submitted.

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Correspondence to Theodore H. Schwartz.

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Henderson, F., Schwartz, T.H. Update on management of craniopharyngiomas. J Neurooncol 156, 97–108 (2022). https://doi.org/10.1007/s11060-021-03906-4

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