Efficacy and safety of radical resection of primary and recurrent craniopharyngiomas in 86 children

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>Optimal treatment of primary and recurrent craniopharyngiomas remains controversial. Radical resection and limited resection plus radiation therapy yield similar rates of disease control and overall survival. The data are much less clear for recurrent tumors. The authors report their experience with radical resection of both primary and recurrent craniopharyngiomas in children and compare the outcomes between the 2 groups.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>A retrospective analysis was performed in 86 children younger than 21 years of age who underwent a total of 103 operations for craniopharyngioma between 1986 and 2008; these were performed by the senior author. The goal was resection with curative intent in all patients. Two patients were lost to follow-up and were excluded from analysis. The mean age at the time of surgery was 9.6 years, and the mean follow-up was 9.0 years.</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>All 57 children with primary tumors underwent gross-total resection (GTR). A GTR was achieved in significantly fewer children with recurrent tumors (18 [62%] of 29). There were 3 perioperative deaths (3%). Tumor recurred after GTR in 14 (20%) of 71 patients. Overall survival and progression-free survival were significantly better in patients with primary tumors at time of presentation to the authors' institution. There were no significant differences in the neurological, endocrinological, visual, or functional outcomes between patients with primary and those with recurrent tumors. Factors negatively affecting overall survival and progression-free survival include subtotal resection (recurrent tumors only), tumor size ≥ 5 cm, or presence of hydrocephalus or a ventriculoperitoneal shunt. Prior radiation therapy and increasing tumor size were both risk factors for incomplete resection at reoperation.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>In the hands of surgeons with experience with craniopharyngiomas, the authors believe that radical resection at presentation offers the best chance of disease control and potential cure with acceptable morbidity. While GTR does not preclude recurrence and is more difficult to achieve in recurrent tumors, especially large and previously irradiated tumors, radical resection is still possible in patients with recurrent craniopharyngiomas with morbidity similar to that of primary tumors.</jats:p></jats:sec>

Surgical management of giant pediatric craniopharyngiomas

“…As we have discovered in our analysis of our entire series of 86 children, prior RT has a markedly negative effect on achieving GTR, severely limiting the treatment options for irradiated, recurrent tumors. 19 Furthermore, recurrent tumors of giant size were also associated with significantly worse OS compared with primary tumors-a finding that underscores the need for long-term surveillance of children with craniopharyngiomas. Preventing recurrent tumors from reaching large size-becoming adherent to an increasing number of vital structures in the suprasellar region and beyond-is imperative to ensure improved functional outcomes and survival.…”

“…Of note, some patients in this series were included in other reports on various aspects of the management of craniopharyngiomas in children. [19][20][21]92,93 Twenty-six patients had tumors greater than or equal to 5 cm in largest diameter in any plane on imaging and were included in this study. Gross-total resection was the primary aim of treatment in all children; GTR was defined as lack of residual tumor by visual inspection intraoperatively and no residual soft-tissue mass or enhancement suspicious for tumor on postoperative imaging as determined by an independent neuroradiologist.…”

“…Prior work by our group demonstrated decreased overall survival (OS) in patients with larger craniopharyngiomas. 19 Other authors report increased mortality, 94 increased neurological and hypothalamic morbidity, 14,22,94 decreased extent of resection and local disease control, 14,16,23,27,28,39,46,68,74,86,91 and higher rates of tumor recurrence 14,22,29,34,83,91 in larger tumors. Some authors, however, report tumor size or volume to have no impact on extent of resection, 2,4,50,88,95 rate of recurrence 49,63 or hypothalamic morbidity.…”

Craniopharyngioma recurrence: the impact of tumor topography

“…Table 1 summarizes outcomes for the largest CP surgical series in which the results were compared between primary and recurrent cases. 3,5,7,8,[10][11][12][13]15,27,29,32 Although comparative evaluation among the cases in these series is complicated by their variable epidemiological composition and lack of validated parameters defining total removal, good outcome, and perioperative mortality, a uniform tendency toward a different outcome for primary and recurrent CPs can be recognized. The rate of radical excision falls dramatically for recurrent CPs, and operative mortality substantially increases for recurrent tumors.…”

Emerging technology in intracranial neuroendoscopy: application of the NICO Myriad

“…Craniopharygiomas are challenging to resect through an open 12,13 or transsphenoidal approach. 7,9,20,29 Many surgeons have demonstrated successful resection of craniopharyngiomas through an extended endoscopic endonasal transsphenoidal approach.…”