Long-term sequelae and quality of life after childhood-onset
craniopharyngioma: results of a Spanish multicenter study.
Abstract
Purpose: We aim to describe the characteristics of patients
with childhood-onset craniopharyngioma and to analyze factors that
impair quality of life (QoL) in this population. Methods:
multicenter national study including patients treated between 2008-2022,
from 2 to 25 years of age diagnosed with craniopharyngioma. QoL was
assessed once during patient’s follow-up by age-adapted versions
Pediatric Quality of Life Inventory (PedsQL TM)
questionnaire. Results: Sixty-six patients were included.
Median age at diagnosis was 5 years (IQR 3-8), while median follow-up
was 7.4 years (IQR 2.8-9.7). Most craniopharyngioma were suprasellar
(93.9%) and 59.7% had hypothalamic involvement (HI). All patients
underwent surgery, 44.4% received radiotherapy and 23.6%, intra-cystic
therapy. Most frequent long-term complications were visual deficit
(72.7%) and endocrine impairment (94.5%). Patients exhibited
hypothyroidism requiring hormone replacement (92.4%), hypocortisolism
(80.3%), diabetes insipidus (86.4%) and/or growth hormone therapy
(50%). When parents evaluated QoL, PedsQL TM median
score was 53.8 points out of 100 (IQR 41-71.6). Higher scores were noted
when patients assessed their own QoL [median-score 64.8 (IQR
57.3-81.8)], observing statistical-significant differences (p=0.019).
QoL was impaired by repeated surgeries (r -0.44; p=0.014), HI
[median-score 51.5 (IQR 39-63.8) vs 76.4 (59-84.8); p=0.001],
radiotherapy [median-score 51.9 (IQR 38.1-61.3) vs 63.8 (IQR 49-82.5);
p=0.02] and longer follow-up (r-0.3; p=0.01). Conclusion: In
our study, most patients had significant comorbidities and low overall
QoL scores, which was mainly affected by repeated surgery, radiation,
and HI. The complex management of these patients requires
multidisciplinary teams that can warrant the hypothalamic preservation
and prompt intervention to prevent and identify sequelae.