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Craniopharyngioma in children: trends from a third consecutive single-center cohort study
Hani J Marcus1,2, Fahid T Rasul3, Ziad Hussein1,4
11Department of Neurosurgery, National Hospital for Neurology and Neurosurgery, London.
Insights
Pediatric craniopharyngioma management has shifted to less invasive surgeries, improving outcomes. Current methods show significantly reduced morbidity compared to previous treatments, maintaining oncological control.
Area of Science:
- Pediatric neurosurgery
- Pediatric oncology
Background:
- Craniopharyngioma management in children has evolved, with a trend towards less invasive surgical techniques.
- The goal is to balance effective tumor control with reduced treatment-related morbidity.
Purpose of the Study:
- To evaluate the safety and effectiveness of current pediatric craniopharyngioma management strategies.
- To compare current approaches with historical management methods at the authors' institution.
Main Methods:
- A 14-year database (2005-2018) of pediatric craniopharyngioma patients (≤17 years) was reviewed.
- Data on presentation, investigation, treatment, and outcomes were extracted retrospectively.
- Morbidity was assessed across visual, pituitary, hypothalamic, neurological, and cognitive domains.
Main Results:
- 59 children diagnosed with craniopharyngioma were identified.
- Surgical approaches included cyst drainage, craniotomy, and transsphenoidal resection, with increased use of image guidance and endoscopy.
- Adjuvant therapies included proton beam and conventional radiotherapy.
- Significantly reduced morbidity was observed in the current cohort compared to the previous one (1996-2004).
Conclusions:
- Pediatric craniopharyngioma management increasingly utilizes less invasive neurosurgical procedures with image guidance or endoscopy.
- Targeted radiotherapy plays a growing role for residual disease.
- These advances achieve comparable tumor control with significantly reduced morbidity and mortality.
Objective:
The management of children with craniopharyngioma has evolved over time, with a trend toward less invasive neurosurgical approaches as surgeons have sought to balance oncological control and treatment-related morbidity. To this end, the aim of this study was to evaluate the safety and effectiveness of the current management of children with craniopharyngioma compared to the previous management methods used at the authors' treatment center.
Methods:
A prospectively maintained database was searched over a 14-year period between January 1, 2005, and December 31, 2018, to identify all children 17 years of age or younger with a new diagnosis of craniopharyngioma. A retrospective case note review was performed for each child to extract data on the presentation, investigation, treatment, and outcome of their illness. Morbidity was assessed in the same fashion as in previous cohorts, according to the following categories: visual loss, pituitary dysfunction, hypothalamic dysfunction, neurological deficits, and cognitive impairment.
Results:
In total, 59 children were identified with craniopharyngioma during the study period. A total of 92 operations were performed, including cyst drainage (35/92; 38.0%), craniotomy and resection (30/92; 32.6%), and transsphenoidal resection (16/92; 17.4%). Approximately two-thirds of all operations were performed using image guidance (66/92; 71.7%) and one-third were performed using endoscopy (27/92; 29.3%). The majority of children had adjuvant therapy comprising proton beam therapy (18/59; 30.5%) or conventional radiotherapy (16/59; 27.1%). The median follow-up duration was 44 months (range 1-142 months), and approximately one-half of the children had no evidence of residual disease on MRI studies (28/59; 47.5%). Of the remaining 31 children, there was a reduction in the volume of residual disease in 8 patients (8/59; 13.6%), stable residual disease in 18 (18/59; 30.5%), and tumor growth in 5 patients (5/59; 8.5%). There was significantly reduced morbidity (p < 0.05) in all categories in the current cohort compared with our last cohort (1996-2004).
Conclusions:
The authors' institutional experience of pediatric craniopharyngioma confirms a trend toward less invasive neurosurgical procedures, most of which are now performed with the benefit of image guidance or endoscopy. Moreover, the authors have identified an expanding role for more targeted radiotherapy for children with residual disease. These advances have allowed for tumor control comparable to that achieved in previous cohorts, but with significantly reduced morbidity and mortality.

