ESPE Abstracts

Purpose: Childhood-onset craniopharyngiomas (CP) are diagnosed due to clinical symptoms (symCP) or incidentally (incCP). In childhood-onset CP, studies on history before CP diagnosis have revealed symptoms and complaints related to CP, which were documented in CP patients’ records with a median duration of 5 months prior to CP diagnosis. We investigated clinical manifestations and outcome in incCPs and symCPs.

Methods: IncCP were discovered in 4 (3m/1f) and symCP in 214 (101m/113f) 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. The Pediatric Quality of Life (PEDQOL) and the German daily life ability scale Fertigkeitenskala Münster-Heidelberg (FMH) questionnaires were used to analyze health-related quality of life (QoL) and functional capacity, respectively.

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. Based on our findings, we conclude that headache, visual impairment, growth retardation, neurologic symptoms, weight gain, symptoms of central diabetes insipidus, and disturbances of pubertal development are frequent symptoms observed in CP at the time of diagnosis. A long duration of history is typical with regard to reduced growth rates and weight gain. IncCP cases occur with low frequency especially in CP patients with tumors of low tumor volume and without hydrocephalus at the time of diagnosis. In incCP, outcome was similar to symCP patients.