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Craniopharyngiomas presenting as incidentalomas: results of KRANIOPHARYNGEOM 2007

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Abstract

Purpose

Childhood-onset craniopharyngiomas (CP) are diagnosed due to clinical symptoms (symCP) or incidentally (incCP). We investigated clinical manifestations and outcome in incCPs and symCPs.

Methods

IncCP were discovered in 4 (3 m/1 f) and symCP in 214 (101 m/113 f) CP recruited 2007–2014 in KRANIOPHARYNGEOM 2007. Age, sex, height, body mass index (BMI), tumor volume, degree of resection, pre- and postsurgical hypothalamic involvement/lesions, pituitary function and outcome were compared between both subgroups.

Results

Reasons for imaging in incCP were cerebral palsy, head trauma, nasal obstruction, and tethered-cord syndrome, whereas headache (44%), visual impairment (25%), and growth retardation (17%) lead to imaging in symCP. Tumor volume at diagnosis was smaller in incCP (median 2.39 cm3; range 0.14–4.10 cm3) when compared with symCP (15.86 cm3; 0.002–286.34 cm3). Age, gender, BMI, height, hydrocephalus, tumor location, and hypothalamic involvement at diagnosis of incCP were within the range of these parameters in symCP. Complete resections were achieved more frequently (3/4 patients) in incCP when compared with symCP (20%). Surgical hypothalamic lesions were distributed similar in incCP and symCP. Irradiation was performed only in symCP (33%). No noticeable differences were observed concerning survival rates, endocrine deficiencies, BMI, height, functional capacity and quality of life of the 4 incCP cases when compared with the symCP cohort.

Conclusions

IncCP are rare (1.8%) and characterized by lack of endocrine deficiencies, resulting in normal height and BMI, no hydrocephalus, and smaller tumor volume at diagnosis when compared with symCPs. Outcome of the observed incCP is similar with symCP.

Clinical trial registration number: NCT01272622.

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Acknowledgements

The authors want to thank all participating colleagues for recruiting patients in KRANIOPHARYNGEOM 2007, and the patients and their parents for participating in this study.

Funding

This study was funded by a grant (HLM; DKS2014.13) of the German Childhood Cancer Foundation, Bonn, Germany.

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Authors and Affiliations

  1. Department of Pediatrics and Pediatric Hematology/Oncology, University Children’s Hospital, Klinikum Oldenburg AöR, Rahel-Straus-Strasse 10, 26133, Oldenburg, Germany

    Svenja Boekhoff & Hermann L. Müller

  2. Department of Neuroradiology, University Hospital Würzburg, 97080, Würzburg, Germany

    Brigitte Bison

  3. Institute of Biostatistics and Clinical Research, University of Münster, 48149, Münster, Germany

    Maria Eveslage

  4. Department of Pediatrics, Faculty of Medicine, Srinakharinwirot University, 26120, Bangkok, Thailand

    Panjarat Sowithayasakul

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  1. Svenja Boekhoff
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  5. Hermann L. Müller
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Contributions

SB researched the data and wrote the manuscript. BB did neuroradiological assessment of all imaging. BB is the neuroradiologist, who performs reference-assessment of imaging in all patients recruited in KRANIOPHARYNGEOM 2007. She prepared the imaging data and their presentation and reviewed/edited the manuscript. ME supervised statistical analyses and reviewed/edited the manuscript. PS contributed to the analytical plan and discussion and reviewed/edited the manuscript. HLM initiated and conducted the multicenter trials HIT-Endo and KRANIOPHARYNGEOM 2000/2007, contributed to the analytical plan and discussion and reviewed/edited the manuscript.

Corresponding author

Correspondence to Hermann L. Müller.

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Conflict of interest

HLM 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 other authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in our study were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. The study KRANIOPHARYNGEOM 2007 (Clinical trial registration number: NCT01272622) was approved by the local standing-committee on ethical practice of the Medizinische Fakultät, Julius-Maximilians-Universität Würzburg, Germany (approval: 94/06), and written parental and/or patient consent was obtained in all cases.

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11102_2019_983_MOESM1_ESM.pdf

Supplementary material 1 Supplemental Figure 1 Parental-assessed health-related quality of life (QoL) as measured by the Pediatric Quality of Life (PEDQOL) [24] questionnaire in patients diagnosed and recruited with incidentaloma craniopharyngioma (incCP) and symptomatic craniopharyngioma (symCP) in the trial KRANIOPHARYNGEOM 2007 between 2007 and 2014. Parental assessments by PEDQOL at the time points 3, 12, and 36 months after CP diagnosis are depicted for the PEDQOL domains autonomy (Suppl. Fig. 1A), emotional stability (Suppl. Fig. 1B), body image (Suppl. Fig. 1C), cognition (Suppl. Fig. 1D), physical function (Suppl. Fig. 1E), social function (friends) (Suppl. Fig. 1F), and social function (family) (Suppl. Fig. 1G). PEDQOL provides negative ratings, i.e. a high score is equivalent to more negative self or parental QoL assessment. Individual PEDQOL scores for incCP are depicted as circle for case 1, triangle for case 3, and square for case 4. PEDQOL scores for sympCP are shown as boxplots. The horizontal line in the middle of the box depicts the median. The top and bottom edges of the box respectively mark the 25th and 75th percentiles. Whiskers indicate the range of values that fall within 1.5 box-lengths. (PDF 363 kb)

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Boekhoff, S., Bison, B., Eveslage, M. et al. Craniopharyngiomas presenting as incidentalomas: results of KRANIOPHARYNGEOM 2007. Pituitary 22, 532–541 (2019). https://doi.org/10.1007/s11102-019-00983-7

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