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22.08.2019 | Ausgabe 5/2019

Pituitary 5/2019

Craniopharyngiomas presenting as incidentalomas: results of KRANIOPHARYNGEOM 2007

Zeitschrift:
Pituitary > Ausgabe 5/2019
Autoren:
Svenja Boekhoff, Brigitte Bison, Maria Eveslage, Panjarat Sowithayasakul, Hermann L. Müller
Wichtige Hinweise

Electronic supplementary material

The online version of this article (https://​doi.​org/​10.​1007/​s11102-019-00983-7) contains supplementary material, which is available to authorized users.

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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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Zusatzmaterial
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)
11102_2019_983_MOESM1_ESM.pdf
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