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

Abstract Background 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 and 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 performe... Mehr ...

Verfasser: Van Schaik, Jiska
Schouten-van Meeteren, Antoinette Y N
Vos-Kerkhof, Evelien
Janssens, Geert O
Porro, Giorgio L
Fiocco, Marta
Bakker, Boudewijn
Tissing, Wim J E
Hoving, Eelco W
van Santen, Hanneke M
Dokumenttyp: Artikel
Erscheinungsdatum: 2023
Reihe/Periodikum: Neuro-Oncology ; volume 25, issue 12, page 2250-2261 ; ISSN 1522-8517 1523-5866
Verlag/Hrsg.: Oxford University Press (OUP)
Sprache: Englisch
Permalink: https://search.fid-benelux.de/Record/base-27443768
Datenquelle: BASE; Originalkatalog
Powered By: BASE
Link(s) : http://dx.doi.org/10.1093/neuonc/noad112

Abstract Background 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 and 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 body mass index (BMI) SDS at diagnosis and Muller grade II at last magnetic resonance imaging of follow-up were associated with 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 = .067), and after 2 years from 28.6% to 6.7% (P = NS). Conclusions In our nationwide cohort, GTR was performed in a relatively low percentage of patients and extensiveness of resection was no longer associated with HO at follow-up. A trend toward improvement of BMI is observed since centralization of care, which needs further exploration.