RARE-05. Legal degree of disability in childhood-craniopharyngioma survivors during long-term follow-up – results of the HIT-ENDO study

BACKGROUND: Cranioparyngiomas are rare low-grade embryonic malformational tumors of the sellar/parasellar region. The prognosis after diagnosis during childhood and adolescence is influenced by endocrine and hypothalamic long-term sequelae. A legal status of the degree of disability (GdB), according to the German Social Code Book V that is worthy of support, provides financial means for psychosocial rehabilitation and participation of craniopharyngioma survivors. The aim of this study was to determine the association of clinical/psychosocial characteristics and quality of life (QoL) indicators with the resulting GdB. PATIENTS AND METHODS: HIT-Endo is a German registry study on craniopharyngioma patients aged ≤ 18 years at diagnosis and included before the year 2000. In a sample of 108 patients, the degree of disability and the association with endocrine, ophthalmological, neuropsychological and psychosocial parameters was analyzed after a mean follow-up period of 16 years (95% CI: 9.8-36.4). RESULTS: 44 patients (41%) did not receive a GdB, three patients (3%) received a GdB of 30-40, 43 patients (40%) a GdB of 50-90 and 18 patients (17%) the maximum GdB of 100. Higher GdB were associated with lower education, higher body mass index standard deviation and a higher degree of visual impairment and hypothalamic involvement of the craniopharyngeoma. Patients with a GdB of 100 reported loss in physical and cognitive function, as well as fatigue, dyspnea, and pain conditions, and limitations in social and occupational contexts. They further had a lower functional capacity (German daily life ability scale (FMH)) compared to those with a smaller GdB. CONCLUSION: The GdB is associated with psychosocial and physical impairments and reflects the long-term consequences of craniopharyngioma during childhood and adolescence. A low functional capacity (assessed by FMH) may indicate the eligibility for a high GdB in later life of craniopharyngioma survivors.