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Childhood craniopharyngioma: 20-year institutional experience in Western Australia

Authors

  • Shoshana R Rath,

    1. Department of Endocrinology and Diabetes, Princess Margaret Hospital for Children, Perth, Western Australia, Australia
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  • Sharon Lee,

    1. Department of Neurosurgery, Princess Margaret Hospital for Children, Perth, Western Australia, Australia
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  • Rishi S Kotecha,

    1. Haematology and Oncology, Princess Margaret Hospital for Children, Perth, Western Australia, Australia
    2. Telethon Institute for Child Health Research, Centre for Child Health Research, Perth, Western Australia, Australia
    3. School of Paediatrics and Child Health, University of Western Australia, Perth, Western Australia, Australia
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  • Mandy Taylor,

    1. Department of Radiation Oncology, Sir Charles Gardiner Hospital, Perth, Western Australia, Australia
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  • Reimar C Junckerstorff,

    1. School of Pathology and Laboratory Medicine, University of Western Australia, Perth, Western Australia, Australia
    2. Section of Neuropathology, PathWest Laboratory Medicine, Royal Perth Hospital, Perth, Western Australia, Australia
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  • Catherine SY Choong

    Corresponding author
    1. School of Paediatrics and Child Health, University of Western Australia, Perth, Western Australia, Australia
    • Department of Endocrinology and Diabetes, Princess Margaret Hospital for Children, Perth, Western Australia, Australia
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  • Conflicts of interest: None declared.

Correspondence: Dr Catherine Choong, Department of Endocrinology and Diabetes, Princess Margaret Hospital for Children, GPO Box D184, Perth, WA 6840, Australia. Fax: +61 8 9340 8605; email: catherine.choong@health.wa.gov.au

Abstract

Aim

A retrospective audit was undertaken to evaluate modes of presentation and treatment outcomes for craniopharyngioma in a single paediatric institution over a 20-year period.

Methods

A search of the neurosurgical and histopathological databases for patients under 21 years of age treated for craniopharyngioma between 1990 and 2010 was performed at our institution. The clinical records of eligible patients were reviewed and information regarding presentation, medical and surgical management and post-treatment outcome were extracted and collated.

Results

Of 10 evaluable patients, the commonest presenting symptoms were headache and visual impairment. Clinical and biochemical evaluation undertaken prior to surgery revealed visual dysfunction in 70% and pituitary deficit in 30%. Gross total resection was achieved in 40% but was curative in only 20%. The remaining 80% required further surgical and/or radiotherapeutic intervention. Seven patients had radiation therapy with stabilisation in 70%. Multiple Pituitary Hormone Deficiency evolved in all patients over time, while visual impairment worsened in 30% post-operatively and improved in 20%. Obesity was present in 50% after a mean follow-up interval of 5.6 years and was apparent within 1 year of initial surgery in 30%. Although neurocognitive, psychological and behavioural problems were noted for some patients during medical review, only 20% of patients were formally assessed.

Conclusions

Craniopharyngioma is associated with significant long-term morbidity. Attention to an integrated care pathway that includes standardised neurocognitive and psychological and behavioural assessment would facilitate early appropriate intervention and support leading to an improved quality of life for children with craniopharyngioma.

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