REVIEW Adult Craniopharyngioma: Case Series, Systematic Review, and Meta-Analysis Charlotte Dandurand, MD * Amir Ali Sepehry, BA, MSc, PhD ‡ Mohammad Hossein Asadi Lari § Ryojo Akagami, MD, BSc, MHSc, FRCSC * Peter Gooderham, MD, FRCSC * ∗ Faculty of Medicine, Division of Neurosurgery, The University of British Columbia, Vancouver, British Columbia, Canada; ‡ Faculty of Medicine, Division of Neurology, The University of British Columbia, Vancouver, British Columbia, Canada; § Faculty of Medicine, Department of Cellular and Physiological Sciences, The University of British Columbia, Vancouver, British Columbia, Canada The study abstract was published because it was selected for the presentation at the 52nd Annual Congress of the Canadian Neurological Sciences Federation taking place in Victoria, British Columbia, Canada from June 20 to 23, 2017. Correspondence: Peter Gooderham, MD, FRCSC, Department of Surgery, The University of British Columbia, 3100—950 W 10th Ave, Vancouver, BC V5Z 1M9, Canada. E-mail: pagooderham@gmail.com Received, February 17, 2017. Accepted, October 26, 2017. Published Online, December 18, 2017. Copyright C  2017 by the Congress of Neurological Surgeons BACKGROUND: The optimal therapeutic approach for adult craniopharyngioma remains controversial. Some advocate for gross total resection (GTR), while others advocate for subtotal resection followed by adjuvant radiotherapy (STR + XRT). OBJECTIVE: To conduct a systematic review and meta-analysis assessing the rate of recur- rence in the follow-up of 3 yr in adult craniopharyngioma stratifed by extent of resection and presence of adjuvant radiotherapy. METHODS: MEDLINE (1946-July 1, 2016) and EMBASE (1980-June 30, 2016) were systemati- cally reviewed. From1975 to 2013, 33 patients were treated with initial surgical resection for adult onset craniopharyngioma at our center and were reviewed for inclusion in this study. RESULTS: Data from 22 patients were available for inclusion as a case series in the systematic review. Eligible studies (n = 21) were identifed from the literature in addition to a case series of our institutional experience. Three groups were available for analysis: GTR, STR + XRT, and STR. The rates of recurrence were 17%, 27%, and 45%, respectively. The risk of developing recurrence was signifcant for GTR vs STR (odds ratio [OR]: 0.24, 95% confdence interval [CI]: 0.15-0.38) and STR + XRT vs STR (OR: 0.20, 95% CI: 0.10-0.41). Risk of recurrence after GTR vs STR + XRT did not reach signifcance (OR: 0.63, 95% CI: 0.33-1.24, P = .18). CONCLUSION: This is the frst and largest systematic review focusing on the rate of recur- rence in adult craniopharyngioma. Although the rates of recurrence are favoring GTR, diference in risk of recurrence did not reach signifcance. This study provides guidance to clinicians and directions for future research with the need to stratify outcomes per treatment modalities. KEY WORDS: Craniopharyngioma, Recurrence, Recurrence rate, Meta-analysis, Neurosurgery Neurosurgery 83:631–641, 2018 DOI:10.1093/neuros/nyx570 www.neurosurgery-online.com C raniopharyngiomas are rare, slow growing, benign (WHO grade I) epithelial tumors, believed to be derived from cell remnants of Rathke’s pouch. 1- 5 They make up between 2% and 5% of all primary intracranial tumors 2, 3 , 6 with an overall incidence rate of 0.5 to 2 cases per million per year. 5, 7 , 8 Half of all cases occur in adulthood with a peak incidence between the ages of 40 and 44 and a second small peak in the sixth decade. 9 , 10 There are 2 distinct histopathological subtypes of craniopharyngioma: adamantinomatous and ABBREVIATIONS: CI, confdence interval; GTR, gross total resection; OR, odds ratio; STR, subtotal resection; UBC, University of British Columbia Neurosurgery Speaks! Audio abstracts available for this article at www.neurosurgery-online.com. papillary. The papillary type occurs almost exclusively in adults. Recent reports show a difference in pathological behavior between the 2 subtypes. 10- 12 Malignant behavior is often observed due to infiltration of, adherence to, and pressure on surrounding critical structures of the sellar region, notably the pituitary gland, hypothalamus, optic nerve, blood vessels, and third ventricle. 10 This may result in considerable morbidity and mortality due to the disease itself or its treatments. 1, 3 , 7 The optimal therapeutic approach remains controversial. 3 , 13, 14 Some authors suggest that gross total resection (GTR) should be the primary goal if it can be achieved with limited associated morbidity. 3 , 7, 15- 17 Other authors advocate less aggressive subtotal resection followed by adjuvant radiotherapy (STR + XRT). 3 , 18, 19 There are competing arguments to NEUROSURGERY VOLUME 83 | NUMBER 4 | OCTOBER 2018 | 631 Downloaded from https://academic.oup.com/neurosurgery/article/83/4/631/4756079 by guest on 10 September 2021