Craniopharyngioma in Children and Adults – Does the Age Matter?

Abstract

<h3>Purpose/Objective(s)</h3> To investigate the long-term results of treatment of children and adults with craniopharyngioma and to identify factors related to survival. <h3>Materials/Methods</h3> A retrospective analysis of all consecutive patients (pts) treated due to craniopharyngioma in years 1979–2019 was performed. Event-free survival (EFS) was defined as no tumor progression or pts` death after the end of treatment (radiotherapy – RT or the last surgery in non-irradiated pts). Standard statistical tests were used. <h3>Results</h3> The group consisted of 97 pts (41 children, 56 adults) with median age of 26 years (range 2-71) and small predominance of males (56%). The most common symptoms were visual disturbances (54%), headaches (54%), endocrine disorders (20%) and nausea/vomiting (22%). The diagnosis was based on histopathological examination in the majority of pts (84%) with remaining 16 cases diagnosed based on characteristic radiological features. Surgery was the primary treatment in 94% with majority having one (44%) or two (26%) surgeries, while 3, 4 and 5 surgeries were conducted in case of 14%, 6% and 4% of pts, respectively. The most common deficits observed after surgery were visual disturbances (45%) and endocrine disorders (62%). RT was applied in 89% pts with 63% receiving irradiation as part of salvage treatment. Conventional RT (CRT) and stereotactic RT (SRT) was applied in 58 and 28 pts, respectively. Median total dose (TD) in CRT was 54 Gy and 16 Gy in SRT. Ten patients in CRT group had stereotactic boost (median TD 6 Gy). The most common side effects of RT were endocrine disorders (14%), visual disturbances (7%), vasculopathies (6%) and deterioration in general status (8%). Secondary tumors within RT volume were observed in 3 pts (2 high grade gliomas, 1 meningioma). Median follow-up was 13 years from the diagnosis and during that time 29 pts died. Five- & 10-year overall survival (OS) was 90% & 78%, respectively. Univariate analysis showed that adults had worse OS compared to children (p=0.001) with 5- & 10-year OS of 85% & 68% vs 97% & 92% for adult vs children, respectively. Also, pts who did not receive RT had inferior outcome with 5- and 10- year OS of 82% & 34% vs 92% & 84% (p=0.0006). In multivariate analysis, only age was an independent factor affecting OS (p=0.005). Median EFS was 8.4 years. Five- and 10- year EFS for the whole group was 78% and 62%. Univariate analysis showed that adults had worse EFS compared to children (p=0.048). RT was also found in univariate analysis to have positive impact on EFS (p=0.021) which was also confirmed in multivariate Cox regression model (p=0.037). The local effect of RT was evaluated in case of 78 pts with stagnation & regression observed in 35 & 37 pts. The comparison of CRT with SRT showed inferior local control in SRT group (p=0.023). However, in SRT arm, in 5 out of 8 failures TD applied was ≤15 Gy. <h3>Conclusion</h3> Adjuvant RT should not be omitted as it is associated with better local control. Meticulous follow up is required to assure effective salvage treatment in case of progression.