Surgical Management of Craniopharyngiomas in Children: Meta-analysis and Comparison of Transcranial and Transsphenoidal Approaches

Neurosurgery 69:630–643, 2011 DOI: 10.1227/NEU.0b013e31821a872d

Controversy persists regarding the optimal treatment of pediatric craniopharyngiomas.

OBJECTIVE: We performed a meta-analysis of reported series of transcranial (TC) and transsphenoidal (TS) surgery for pediatric craniopharyngiomas to determine whether comparisons between the outcomes in TS and TC approaches are valid.

METHODS: Online databases were searched for English-language articles reporting quantifiable outcome data published between 1990 and 2010 pertaining to the surgical treatment of pediatric craniopharyngiomas. Forty-eight studies describing 2955 patients having TC surgery and 13 studies describing 373 patients having TS surgery met inclusion criteria.

RESULTS: Before surgery, patients who had TC surgery had less visual loss, more frequent hydrocephalus and increased intracranial pressure, larger tumors, and more suprasellar disease. After surgery, patients in the TC group had lower rates of gross total resection (GTR), more frequent recurrence after GTR, higher neurological morbidity, more frequent diabetes insipidus, less improvement, and greater deterioration in vision. There was no difference in operative mortality, obesity/hyperphagia, or overall survival percentages.

CONCLUSION: Directly comparing outcomes after TC and TS surgery for pediatric craniopharyngiomas does not appear to be valid. Baseline differences in patients who underwent each approach create selection bias that may explain the improved rates of disease control and lower morbidity of TS resection. Although TS approaches are becoming increasingly used for smaller tumors and those primarily intrasellar, tumors more amenable to TC surgery include large tumors with significant lateral extension, those that engulf vascular structures, and those with significant peripheral calcification.

Surgical management of giant pediatric craniopharyngiomas

J Neurosurg Pediatrics 6:000–000, 2010.DOI: 10.3171/2010.8.PEDS09385

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.