Dataset Information

Treatment and outcome of the Dutch Childhood Craniopharyngioma Cohort study; first results after centralization of care.

ABSTRACT:

Introduction

Childhood craniopharyngioma (cCP) has excellent survival, but quality of life may be severely hampered by hypothalamic dysfunction. We aimed to evaluate treatment and hypothalamic outcomes of a Dutch cCP cohort, and evaluate the effect of centralization of care.

Methods

A retrospective cohort study was performed, including cCP patients diagnosed between 2004-2021. Treatment characteristics and hypothalamic outcomes were evaluated and compared before and since centralization of care in May 2018.

Results

We included 87 cCP patients. Cyst drainage/fenestration was performed in 29.9%, limited resection in 27.6%, near total resection in 16.1%, and gross total resection (GTR) in 25.4%. Radiotherapy was given in 46.0%. After a median follow-up of 6.5 years, hypothalamic obesity (HO) was present in 24.7% and panhypopituitarism with diabetes insipidus in 71.3%. Higher BMI SDS at diagnosis and Muller grade II at last MRI of follow-up were associated to overweight/obesity. No association was found between extensiveness of resection and overweight/obesity at last follow-up. When comparing before and after centralization of care, rates of GTR remained similar, but BMI outcomes changed; mean ΔBMI SDS 1 year after diagnosis from 1.12 (SD 1.15) to 0.81 (SD 1.24), and HO after 1 year decreased from 33.3% to 12.0% (p = 0.067), and after 2 years from 28.6% to 6.7% (p = NS).

Conclusion

In our nationwide cohort, GTR was performed in a relatively low percentage of patients and extensiveness of resection was no longer associated to HO at follow-up. A trend towards improvement of BMI is observed since centralization of care, which needs further exploration.

SUBMITTER: Van Schaik J

PROVIDER: S-EPMC10708930 | biostudies-literature | 2023 Jun

REPOSITORIES: biostudies-literature

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Treatment and outcome of the Dutch Childhood Craniopharyngioma Cohort study: First results after centralization of care.

Van Schaik Jiska J Schouten-van Meeteren Antoinette Y N AYN Vos-Kerkhof Evelien E Janssens Geert O GO Porro Giorgio L GL Fiocco Marta M Bakker Boudewijn B Tissing Wim J E WJE Hoving Eelco W EW van Santen Hanneke M HM

Neuro-oncology 20231201 12

<h4>Background</h4>Childhood craniopharyngioma (cCP) has excellent survival, but quality of life may be severely hampered by hypothalamic dysfunction. We aimed to evaluate treatment and hypothalamic outcomes of a Dutch cCP cohort, and evaluate the effect of centralization of care.<h4>Methods</h4>A retrospective cohort study was performed, including cCP patients diagnosed between 2004 and 2021. Treatment characteristics and hypothalamic outcomes were evaluated and compared before and since centra ...[more]

PMID: 37381692

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