Characteristics and Outcomes in Pediatric Versus Adult Craniopharyngiomas: A Systematic Review and Meta-analysis

 

Objective: Craniopharyngiomas are benign tumors that arise from the embryonic remnants of Rathke's pouch and account for 1.2 to 4.6% of all intracranial tumors. They have a bimodal distribution with the adamantinomatous subtype more common in children while the papillary subtype is more common in adults. Treatment can be highly variable depending on the extent of the tumor and can include surgical resection by either a transcranial or endoscopic endonasal approach, chemotherapy, and/or radiation. Yet few have focused on the differences between adult and pediatric craniopharyngiomas. This study aims to analyze the differences in treatments and outcomes between these two populations.

Methods: The Preferred Reporting of Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were followed for this systematic review with meta-analysis to identify original studies reporting patient outcome data undergoing surgery for craniopharyngioma resection between January 1, 1990, and January 1, 2021, in the Web of Science, MEDLINE, and Scopus databases. Studies before 1990 were excluded to take into account the increasing utilization of magnetic resonance imaging (MRI), evolving microsurgical techniques, and endoscopic skull base surgery. To reduce the risk of publication bias, studies that reported fewer than 10 patients were excluded.

Results: The search yielded 1,184 unique articles of which 99 articles (n = 4,232) met inclusion criteria for quantitative analysis. Pathological subtype upon presentation varied significantly as children were more likely to harbor adamantinomatous tumors (92.67%) compared to adults (69.04%), who saw a greater rate of papillary tumors (24.36%) than children (5.70%; p < 0.001). Children presented with significantly larger tumor size (3.61 ± 0.86 cm) compared to adults (2.86 ± 0.44 cm, p < 0.001). Adults were more likely to receive a transsphenoidal resection (51.74 vs. 26.18%, p < 0.001), although no significant difference was found with analysis of extent of resection between adult and pediatric craniopharyngiomas. Postoperatively, pediatric craniopharyngiomas were less likely to be complicated by CSF leak or meningitis (8.64 vs. 13.52%, p = 0.004). No significant difference was found between rates of post-operative vascularized reconstruction, lumbar drain use, or stroke/vascular injury. Compared to adults, children had significantly worse outcomes by most functional measures, including higher rates of panhypopituitarism (63.10 vs. 39.71%, p < 0.001), permanent diabetes insipidus (64.20 vs. 54.93%, p<0.001), cognitive deterioration and behavioral changes (18.47 vs. 8.88%, p = 0.001), obesity (36.65 vs. 10.87%, p < 0.001), hypothalamic dysfunction (14.53 vs. 6.55%, p = 0.003), and cranial nerve or neurological defects (7.75 vs. 4.72%, p = 0.038). No significant difference was found in rates of vision deterioration. Craniopharyngiomas were more likely to recur in children compared to adults (30.33 vs. 26.03%, p = 0.017), although mean time to recurrence was not significantly different (34.53 ± 24.13 vs. 34.13 ± 20.98, p = 0.443). Kaplan–Meier analysis of 5-year progression-free survival rates yielded 78.6% for adults and 72.4% for children (p = 0.476). Analysis of overall survival found 90.1% survival at 5 years for adults and 98.2% for children (p = 0.079).

Conclusion: Adult and pediatric craniopharyngiomas appear to have disparities in outcomes. Pediatric patients frequently require multimodality treatment and are best managed with a multidisciplinary team and an individualized approach.

Publication History

Article published online:
15 February 2022

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