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[Children with craniopharyngioma, a 'benign' brain tumor with a high morbidity]
M J Taphoorn1, C van Es, R H Gooskens
1Afd. Neurologie, Universitair Medisch Centrum, GA Utrecht. m.j.b.taphoorn@neuro.azu.nl
Insights
Craniopharyngioma, a benign brain tumor in children, often leads to significant long-term morbidity despite successful treatment. Multidisciplinary care is crucial for managing these effects and improving quality of life.
Area of Science:
- Pediatric Oncology
- Pediatric Neurosurgery
- Endocrinology
Background:
- Craniopharyngioma is a histologically benign tumor primarily affecting children.
- While neurosurgical treatment and radiotherapy offer a fair prognosis, central brain location and treatment adverse effects cause substantial morbidity.
Observation:
- Three pediatric cases of craniopharyngioma are presented, highlighting symptoms like increased intracranial pressure and hydrocephalus.
- Patients experienced significant endocrine, visual, hypothalamic, and intellectual disturbances post-treatment.
- Despite treatment, tumor recurrence was not observed in the follow-up periods (up to 4-6 years).
Findings:
- Craniopharyngioma treatment, including surgery and radiotherapy, results in considerable long-term morbidity in pediatric patients.
- The observed morbidity negatively impacts the patients' quality of life.
- Complete tumor removal in one case, without radiotherapy, still led to significant neurological and endocrine deficits.
Implications:
- A multidisciplinary approach to craniopharyngioma management is essential for pediatric patients.
- Early observation of treatment-related problems can help prevent further damage.
- While morbidity cannot be entirely avoided, proactive management can improve outcomes and quality of life for affected children.
Abstract:
The craniopharyngioma is a histologically benign tumour, mainly occurring in childhood. Neurosurgical treatment, in some of these patients in combination with external radiotherapy, results in a fair prognosis for children with craniopharyngioma. However, the central localisation of this tumour in the brain, damage of surrounding structures due to tumour growth, as well as the adverse effects of treatment will result in substantial morbidity in many patients. The first patient, a 13-year-old boy, presented with symptoms and signs of increased intracranial pressure. Following operation he was treated with radiotherapy, and up to 4 years after the operation there was no tumour recurrence. However, he experienced endocrine, visual, hypothalamic and intellectual disturbances due to the disease and its treatment. The second patient, a 6-year-old girl, presented with hydrocephalus due to the tumour, but also had growth retardation. After operation and radiotherapy, she had visual, endocrine, and intellectual dysfunction, but there were no signs of tumor recurrence. The third patient, an 12-year-old boy, already had growth retardation several years before he presented with neurological dysfunction. He received no postoperative radiation, as all tumour tissue could be removed. He had visual, hypothalamic, endocrine and intellectual disturbances but until nearly 6 years after the operation, there was no tumour recurrence. The morbidity due to the tumour and its treatment has a negative impact on the patient's quality of life. A multidisciplinary treatment and follow-up of these children cannot avoid this morbidity but may result in a timely observation of problems and thereby prevent unnecessary damage.