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Childhood craniopharyngioma—current concepts in diagnosis, therapy and follow-up

Nature Reviews Endocrinology volume 6, pages 609–618 (2010)Cite this article

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Abstract

Craniopharyngiomas have an overall incidence of 0.5–2.0 new cases per million of the population per year, and ∼30–50% of all cases represent childhood craniopharyngioma. These partly cystic embryogenic malformations of the sellar region are presumably derived from Rathke cleft epithelium. Many of the typical manifestations at primary diagnosis are nonspecific and include headache, visual impairment, polyuria and/or polydypsia, growth retardation and weight gain. Total resection is the treatment of choice in patients with favorable tumor localization, with the intention to maintain hypothalamic–pituitary and optical nerve functions. When the tumor localization is unfavorable, a limited resection followed by local irradiation is recommended. The overall survival rates are high (91–98%). High recurrence rates after complete resection and high progression rates after incomplete resection have been observed, although the risk of recurrence or progression is less after complete resection than partial resection. Irradiation of the tumor is protective and the appropriate time point of irradiation after incomplete resection is currently under investigation in a randomized trial. Long-term sequelae substantially reduce the quality of life of ∼50% of long-term survivors, notably extreme obesity owing to hypothalamic involvement.

Key Points

  • The combination of headache, visual impairment, pathological low growth rate, weight gain and polydipsia and/or polyuria should arouse suspicion of craniopharyngioma in the differential diagnosis

  • A pathological low growth rate demonstrable as early as 12 months of age can be an early manifestation of the disease; an increase in weight tends to occur as a late manifestation

  • The combination of solid, cystic and calcified craniopharyngioma components is an important radiological clue to the tumor diagnosis on MRI and CT

  • Quality of life is frequently impaired in long-term survivors due to sequelae caused by the anatomical proximity of craniopharyngioma to the optic nerve and/or chiasma, pituitary gland and hypothalamus

  • Extreme obesity owing to hypothalamic involvement has a major negative impact on quality of life

  • For unfavorably localized craniopharyngiomas with hypothalamic involvement, a radical neurosurgical treatment strategy is not recommended owing to the high risk of adverse sequelae

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Figure 1: Clinical manifestations before diagnosis of craniopharyngioma in 138 children and adolescents.
Figure 2: Height and weight before and after diagnosis of childhood craniopharyngioma.
Figure 3: MRI of different sellar masses with different sequelae and long-term prognosis.
Figure 4: Kaplan–Meier analyses of 3-year event-free survival rates (mean ± SD) in relation to the degree of resection among 117 patients with craniopharyngioma followed prospectively in the KRANIOPHARYNGEOM 2000 trial.
Figure 5: Study design of the randomized trial KRANIOPHARYNGEOM 2007.97

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Acknowledgements

H. L. Müller is supported by a grant of the Deutsche Kinderkrebsstiftung, Bonn, Germany.

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  1. Department of Pediatrics and Pediatric Hematology and Oncology, Zentrum für Kinder- und Jugendmedizin, Klinikum Oldenburg, Rahel-Straus-Strasse 10, 26133, Oldenburg, Germany

    Hermann L. Müller

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Müller, H. Childhood craniopharyngioma—current concepts in diagnosis, therapy and follow-up. Nat Rev Endocrinol 6, 609–618 (2010). https://doi.org/10.1038/nrendo.2010.168

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