RARE-01. MANAGEMENT AND OUTCOMES OF PAEDIATRIC CRANIOPHARYNGIOMA: A 15-YEAR EXPERIENCE IN SINGAPORE. (4th December 2020)

• Record Type: Journal Article
• Authors: Lim, Mervyn Jun Rui; Liu, Sherry Jiani; Ho, Cindy Wei Li; Teo, Kejia; Lwin, Sein; Chou, Ning; Yeo, Tseng Tsai; Kimpo, Miriam; Nga, Vincent Diong Weng
• Is Part Of: Neuro-oncology. Volume 22(2020)Supplement 3
• Page Start: iii441
• Page End: iii442
• Publication Date: 2020-12-04
• Subjects: Brain Neoplasms -- Periodicals; Brain -- Tumors -- Periodicals; Brain -- Cancer -- Periodicals; Nervous system -- Cancer -- Periodicals; 616.99481
• DOI: 10.1093/neuonc/noaa222.713
• Languages: English
• ISSNs: 1522-8517

Abstract

Abstract: BACKGROUND: Craniopharyngiomas are rare embryonic malformations of the sellar region with high survival rates but high morbidity due to long-term sequelae caused by the location of the tumour. We summarise our institution's experience on the management and outcomes of paediatric craniopharyngiomas in Singapore. METHODS: This was a retrospective review of all paediatric patients (18 years and below) with histologically diagnosed craniopharyngioma managed by the National University Hospital, Singapore from January 2002 to June 2017. Data on clinical presentation, imaging, treatments, and outcomes were extracted from the electronic medical records using a standardized data collection form. Data analysis was conducted using RStudio (Version 1.2.5033). Institutional ethics approval was obtained for the study. RESULTS: We identified 12 cases of paediatric craniopharyngiomas. The majority of cases were male (8, 66.7%) and the median age at presentation was 6.0 (IQR 3.8 – 9.5). Initial surgical management was tumour excision (11, 91.7%) or insertion of a reservoir into the cyst cavity (1, 8.3%). All cases had diabetes insipidus, 10 (83.3%) had endocrine dysfunction, and 8 (66.7%) had visual impairment on long term follow up. 7 (58.3%) cases had recurrence, and 3 (25.0%) had demised. Cox-regression showed that females (HR=33.9, p=0.049), and Chinese race (HR=13.3, p=0.034) were at higher risk for recurrence, but age at diagnosis and residual tumor on post-operative MRI was not significant. CONCLUSION: The management of craniopharyngioma is complex as it is complicated by high recurrence rates and significant long-term morbidity. Further research on treatment strategies focusing on maintaining quality of life is important.