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Surgical management of giant pediatric craniopharyngiomas
Robert E Elliott1, Jeffrey H Wisoff
1Department of Neurosurgery, New York University School of Medicine, New York, New York, USA.
Insights
Radical resection of giant craniopharyngiomas in children offers excellent disease control. While functional outcomes are acceptable, neurological complications are slightly higher than with smaller tumors, impacting survival in recurrent cases.
Area of Science:
- Pediatric neurosurgery
- Pediatric oncology
- Cranial base surgery
Background:
- Giant craniopharyngiomas (≥5 cm) are associated with worse outcomes in children.
- Previous studies suggest larger tumor size correlates with poorer functional results and survival.
Purpose of the Study:
- To evaluate oncological, endocrinological, and functional outcomes after radical resection of giant craniopharyngiomas in pediatric patients.
- To assess the impact of tumor size on treatment success and patient survival.
Main Methods:
- Retrospective analysis of 26 children (<18 years) who underwent radical resection of giant craniopharyngiomas between 1986 and 2006.
- Data collected included tumor characteristics, surgical approach, and postoperative outcomes.
Main Results:
- Gross-total resection (GTR) was achieved in 77% of patients, with higher success in primary tumors (100%) than recurrent (33%).
- Disease control was achieved in 84% of patients, significantly higher with GTR (95%) vs. subtotal resection (STR) (50%).
- Postoperative complications included new-onset diabetes insipidus (63.2%), visual deficits (16-28%), and neurological deficits (19% significant, 19% mild/moderate).
Conclusions:
- Radical resection of giant craniopharyngiomas in children yields excellent disease control and acceptable functional outcomes.
- Neurological complication rates are slightly elevated compared to smaller tumors.
- Recurrent, large, and previously irradiated tumors have diminished survival rates following radical resection.
Object:
Prior work by the authors' group and reports of other authors suggest worse functional outcomes and decreased survival in children with larger craniopharyngiomas. The purpose of this study was to assess the oncological, endocrinological, and functional outcomes in children who underwent radical resection of giant craniopharyngiomas (defined as 5 cm or greater in largest diameter).
Methods:
Between 1986 and 2006, 26 children under the age of 18 (14 boys, 12 girls; mean age 10.5 years) underwent radical resection of giant craniopharyngiomas performed by the senior author. Data were retrospectively collected to assess the outcome of surgical treatment.
Results:
Twenty (77%) of 26 patients underwent gross-total resection (GTR) confirmed by intraoperative inspection and postoperative imaging. All primary tumors (17 of 17) and 3 (33%) of 9 recurrent tumors were treated with GTR. There was no operative mortality, and 18 of 26 patients (69%) were alive at a mean follow-up of 8.9 years (median 9.3 years). Disease control was achieved in 21 (84%) of the 25 patients followed up for more than 6 months and was more successful in patients who underwent GTR (95%) than in those who underwent STR (50%, p = 0.03). New-onset diabetes insipidus (DI) occurred in 63.2% of patients (73% of patients had DI postoperatively). New or worsened deficits in visual acuity and visual fields occurred in 16% and 28%, respectively, of the 25 patients for whom postoperative visual data were available. Five patients (19%) experienced significant, permanent neurological deficits, and 5 (19%) had mild to moderate deficits. New or worsened hypothalamic disturbance occurred in 35% and 22% of patients, respectively, but obesity developed in only 15%.
Conclusions:
In this retrospective series, radical resection of giant craniopharyngiomas in children was found to lead to excellent rates of disease control with acceptable or good functional outcomes but slightly higher rates of neurological complications compared with rates in patients with smaller tumors. Radical resection is less successful in recurrent tumors that reach very large sizes, especially previously irradiated tumors, with resultant diminished survival.
