Neurosurgical treatment of craniopharyngioma in adults and children: early and long-term results in a large case series

Clinical article

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Object

Craniopharyngioma accounts for 2%–5% of all primary intracranial neoplasms. The optimal management of craniopharyngioma remains controversial. The authors evaluated the early results of surgery and the longterm risk of tumor recurrence in a large series of patients undergoing resection of craniopharyngiomas.

Methods

Between 1990 and 2008, 112 consecutive patients (57 male and 55 female patients with a mean [± SEM] age of 33.3 ± 1.8 years) underwent resection of craniopharyngiomas at the authors' hospital. Recurrence or growth of residual tumor tissue during follow-up was assessed using MR imaging.

Results

There were 3 perioperative deaths (2.7%). Severe adverse events were more frequent in patients who underwent operations via the transcranial route (37%) than the transsphenoidal approach (5.6%; p < 0.001). Magnetic resonance imaging showed radical resection of the tumor in 78 (71.6%) of the remaining 109 patients. Previous surgery and maximum tumor diameter were associated with persistence of disease after surgery. Craniopharyngioma recurred in 26 (24.5%) of 106 patients. Presence of residual tumor on the first postoperative MR imaging, male sex, and no postoperative radiation therapy were associated with a risk of tumor recurrence. Quality-of-life data were assessed in the 91 patients who attended the authors' institution for follow-up visits. Among them, 8.8% patients were partially or completely dependent on others for daily living activities before surgery. This percentage increased to 14.3% at the last follow-up visit. The 5- and 10-year overall survival rates were 94.4% (95% CI 90.0%–98.8%) and 90.3% (95% CI 83.4%–97.3%), respectively.

Conclusions

Complete surgical removal of craniopharyngioma can be achieved with reasonable safety in more than 70% of patients. Recurrence of craniopharyngioma may occur even after apparent radical excision. Prompt management of residual or recurring disease by radiotherapy, repeat surgery, or a combination of both is usually successful in controlling further tumor growth.

Abbreviations used in this paper: GH = growth hormone; GKS = Gamma Knife surgery; HR = hazard ratio; IQR = interquartile range; QOL = quality of life.

Article Information

Address correspondence to: Pietro Mortini, M.D., Department of Neurosurgery, Istituto Scientifico San Raffaele, Via Olgettina 60, 20132 Milano, Italy. email: mortini.pietro@hsr.it.

Please include this information when citing this paper: published online January 7, 2010; DOI: 10.3171/2010.11.JNS10670.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    Preoperative (A and B) and postoperative (C and D) T1-weighted contrast-enhanced MR images in a 38-year-old woman. The preoperative coronal (A) and sagittal (B) images show a suprasellar and retrosellar craniopharyngioma. A frontoorbitozygomatic approach was used in this case. The postoperative coronal (C) and sagittal (D) images obtained 2 months after surgery show radical resection of the tumor. The patient suffered from postoperative panhypopituitarism and diabetes insipidus. Visual function, which was impaired before surgery, markedly improved after surgery. She also showed improvement of preexisting anorexia and memory loss.

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    Kaplan-Meier analysis of recurrence-free survival in patients undergoing operations for craniopharyngioma. The median follow-up duration for the 106 patients on neuroradiological follow-up was 67 months (IQR 30–112.5 months). The recurrence-free survival rates at 5 and 10 years were 75.7% (95% CI 66.7%–84.7%) and 69.3% (95% CI 58.4%–80.1%), respectively.

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    Kaplan-Meier analysis of recurrence-free survival according to the absence (solid line) or presence (dashed line) of residual tumor after surgery. The recurrence-free survival rate at 5 years was 85.6% (95% CI 76.8%–94.4%) in 80 patients with no residual tumor after surgery, compared with 46.3% (95% CI 25.7%–66.8%; p < 0.001, log-rank test) in 26 patients with residual tumor after surgery.

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    Kaplan-Meier analysis of overall survival in 112 patients undergoing operations for craniopharyngioma. The overall survival rates at 5 and 10 years were 94.4% (95% CI 90.0%–98.8%) and 90.3% (95% CI 83.4%–97.3%), respectively.

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