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Long-term results of treatment for craniopharyngioma in children
R M Villani1, G Tomei, L Bello
1Institute of Neurosurgery, University of Milan, Ospedale Maggiore Policlinico, IRCCS, Italy.
Insights
This study reports on 27 children treated for craniopharyngioma, showing that radical tumor removal leads to better functional outcomes and fewer recurrences. A new functional evaluation scale aids in assessing long-term patient well-being.
Area of Science:
- Pediatric Neurosurgery
- Pediatric Oncology
- Endocrinology
Background:
- Craniopharyngioma is a rare brain tumor affecting children.
- Treatment aims for maximal tumor removal while preserving neurological function.
- Long-term outcomes require comprehensive functional assessment.
Purpose of the Study:
- To report treatment outcomes for pediatric craniopharyngioma.
- To evaluate a novel functional assessment scale for long-term follow-up.
- To correlate surgical extent with tumor recurrence and functional status.
Main Methods:
- Retrospective analysis of 27 children with craniopharyngioma.
- Surgical approaches included subfrontal pterional, transsphenoidal, and transcallosal/transcortical.
- Patients were followed for a mean of 7 years using a proposed functional evaluation scale.
Main Results:
- Radical tumor removal (70.8%) was associated with lower recurrence rates (17.6%) compared to limited surgery plus radiotherapy (42.8%).
- Complete excision correlated with high functional performance (85% low score on the scale).
- Progressive improvement in visual, endocrine, and psychosocial functions was observed; 81% achieved endocrine balance.
Conclusions:
- Radical surgical resection is crucial for improved outcomes in pediatric craniopharyngioma.
- The proposed functional evaluation scale is effective for routine patient assessment.
- While many patients regain social function, long-term disabilities like short stature and hormonal disturbances persist.
Abstract:
Results of the treatment of 27 children with craniopharyngioma are reported. A subfrontal pterional approach was used in 55.6% of cases, a transsphenoidal and a transcallosal or transcortical approach in 25.9% and 18.5% of cases, respectively. Radical removal was the goal of surgery and was achieved in 70.8% of cases treated as primary surgery. The operative mortality was 3.7% and was due to hypothalamic failure. Most (81.4%) patients were followed up, for a mean of 7 years. Patients were evaluated according to a functional evaluation scale and outcome categories proposed by us. The scale takes account of tumor (recurrences and their eventual evolution); visual functions; endocrine functions (mainly hypothalamic), attainment of endocrine balance and drug regimen; headache; and psychosocial function. Recurrences were observed in 17.6% of patients treated with radical surgery and in 42.8% of those treated with limited surgery plus radiotherapy. A progressive amelioration of visual, endocrine and neuropsychosocial functions from the intervention to follow-up was observed in the majority of patients. Complete tumor excision was associated in 85% of cases with a low score on the functional scale, reflecting a high functional performance. Adequate substitution therapy maintained endocrine balance in 81% of patients. Since the intervention a progressive decrease in the number and dosages of medications has been observed. The majority of patients were again able to lead a normal social life. Small stature, obesity, headache, and emotional and sexual disturbances were frequent cause of long-term disability even despite adequate drug regimens. The functional evaluation scale we propose is a simple and effective tool that can be easily used during routine evaluation of patients with craniopharyngioma.