Craniopharyngioma in Children Younger Than 6 Years Old—What Is the Best Management? A Clinical and Surgical Analysis

Objective: Craniopharyngiomas represent one of the most common tumors of the hypothalamo–pituitary region in childhood. Despite their benign histology, the proximity to noble structures makes their management an ongoing challenge due to the possible neuro-endocrinological symptoms, visual impairment and eventual obstructive hydrocephalus that influence importantly the quality of life of the patient. This is particularly true in little children. Even though there are different surgical techniques to treat this type of tumor, the timing and approach of choice in children younger than 6 years old is still debated. The aim of this work is to analyze the best management from a clinical and surgical point of view.

Materials and Methods: For this retrospective study, the databank of Meyer Children’s Hospital IRCCS the was used. 129 consecutive pediatric patients (0–18 years old) with a craniopharyngioma were referred to our center between January 1994 and January 2024. Of these 22 patients were younger than 6 years old upon diagnosis. All patients underwent endocrinological, ophthalmogical and radiological examination upon admission. Standard follow-up appointments at the outpatient clinic were given to the patients.

Results: The mean follow-up was of 10 years ± 1.4 years. All of the tumors had sellar and suprasellar components. The most common presenting symptoms were headaches (67.8%), visual impairment (30% - not reliable due to the very young age of the children who are thus less compliant during the ophthalmologic evaluation), and nausea/vomiting (39%). Most patients (74%) had resolution of their presenting symptoms by their first postoperative visit. Postoperatively, incidence of panhypopituitarism or diabetes insipidus developed in 84% and 91% of patients, respectively. 5 patients underwent only an Ommaya reservoir positioning, in 1 of these patients an endoscopic fenestration of the tumoral cyst was performed before putting in the Ommaya reservoir. 4 patients underwent first an endoscopic fenestration and not long after a subfrontal approach for craniopharyngioma removal. 5 patients were operated via a transsphenoidal endonasal approach for removal of the tumor while 12 patients were operated on through a subfrontal approach. We noticed no significant differences in terms of extensive resection and outcome between the patients operated via a subfrontal approach and those operated by an endoscopic approach. Recurrence rate and need for adjuvant radiation is quite similar in both groups.

Conclusion: Considering the very young age of these group of patients, it is important to bear in mind that the choice of the treatment should aim to preserve the hypothalamic function of the child. Thus, the least invasive approach is often the best approach. Given the similarity in terms of outcome between the craniotomic and endoscopic approach we believe that the latter should be the treatment of choice. However, this depends on the expertise of the surgeon.

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Artikel online veröffentlicht:
07. Februar 2025