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Craniopharyngioma

Nature Reviews Disease Primers volume 5, Article number: 75 (2019) Cite this article

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Abstract

Craniopharyngiomas are rare malformational tumours of low histological malignancy arising along the craniopharyngeal duct. The two histological subtypes, adamantinomatous craniopharyngioma (ACP) and papillary craniopharyngioma (PCP), differ in genesis and age distribution. ACPs are diagnosed with a bimodal peak of incidence (5–15 years and 45–60 years), whereas PCPs are restricted to adults mainly in the fifth and sixth decades of life. ACPs are driven by somatic mutations in CTNNB1 (encoding β-catenin) that affect β-catenin stability and are predominantly cystic in appearance. PCPs frequently harbour somatic BRAFV600E mutations and are typically solid tumours. Clinical manifestations due to increased intracranial pressure, visual impairment and endocrine deficiencies should prompt imaging investigations, preferentially MRI. Treatment comprises neurosurgery and radiotherapy; intracystic chemotherapy is used in monocystic ACP. Although long-term survival is high, quality of life and neuropsychological function are frequently impaired due to the close anatomical proximity to the optic chiasm, hypothalamus and pituitary gland. Indeed, hypothalamic involvement and treatment-related hypothalamic lesions frequently result in hypothalamic obesity, physical fatigue and psychosocial deficits. Given the rarity of these tumours, efforts to optimize infrastructure and international collaboration should be research priorities.

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Fig. 1: Features of craniopharyngioma.
Fig. 2: Protumorigenic effects of senescence and SASP in mouse and human ACP.
Fig. 3: Neuroradiological characteristics of craniopharyngioma and other sellar masses.
Fig. 4: CP with hypothalamic involvement: preoperative grading on MRI.
Fig. 5: Craniopharyngioma hypothalamic damage: postoperative grading on MRI.

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Acknowledgements

H.L.M. is supported by the German Childhood Cancer Foundation, Bonn, Germany (grant DKS2014/13). The authors thank C. L. Andoniadou, Centre for Craniofacial and Regenerative Biology, King’s College London, London, UK, for help in preparing Fig. 2.

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Authors and Affiliations

  1. Department of Pediatrics and Pediatric Hematology/Oncology, University Children’s Hospital, Klinikum Oldenburg AöR, Oldenburg, Germany

    Hermann L. Müller

  2. Department of Radiation Oncology, St. Jude Children’s Research Hospital, Memphis, TN, USA

    Thomas E. Merchant

  3. Department of Neuroradiology, University Hospital, Würzburg, Germany

    Monika Warmuth-Metz

  4. Developmental Biology and Cancer Programme, Birth Defects Research Centre, UCL Great Ormond Street Institute of Child Health, University College London, London, UK

    Juan-Pedro Martinez-Barbera

  5. Service de Neurochirurgie, Hôpital Necker-Enfants Malades, Sorbonne Paris Cité, Paris, France

    Stephanie Puget

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Contributions

Introduction (H.L.M.); Epidemiology (H.L.M.); Mechanisms/pathophysiology (J.-P.M.-B.); Diagnosis, screening and prevention (H.L.M. and M.W.-M.); Management (T.E.M. and S.P.); Quality of life (H.L.M., T.E.M. and S.P.); Outlook (H.L.M.); Overview of the Primer (H.L.M.).

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Correspondence to Hermann L. Müller.

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Competing interests

H.L.M. has received reimbursement of participation fees for scientific meetings and continuing medical education events from the following companies: Ferring, Lilly, Pfizer, Sandoz/Hexal, Novo Nordisk, Ipsen and Merck Serono. He has received reimbursement of travel expenses from Ipsen and lecture honoraria from Pfizer. The remaining authors declare no competing interests.

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Nature Reviews Disease Primers thanks E. T. Hidalgo, J. M. Pascual, R. Prieto, T. Schwartz and the other, anonymous, reviewer(s) for their contribution to the peer review of this work.

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Müller, H.L., Merchant, T.E., Warmuth-Metz, M. et al. Craniopharyngioma. Nat Rev Dis Primers 5, 75 (2019). https://doi.org/10.1038/s41572-019-0125-9

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