Craniopharyngiomas presenting as incidentalomas: results of KRANIOPHARYNGEOM 2007
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Childhood-onset craniopharyngiomas (CP) are diagnosed due to clinical symptoms (symCP) or incidentally (incCP). We investigated clinical manifestations and outcome in incCPs and symCPs.
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.
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.
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.
KeywordsCraniopharyngioma Incidentaloma Hypothalamus Pituitary Quality of life
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.
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.
This study was funded by a grant (HLM; DKS2014.13) of the German Childhood Cancer Foundation, Bonn, Germany.
Compliance with ethical standards
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.
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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