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.
Background The current standard-of-care treatment for brain metastases (BM)≥20 is Whole Brain Radiotherapy (WBRT), which can cause neurocognitive decline detrimental to patients’ quality of life, especially if their functional status is good on presentation. The benefits of Gamma Knife Surgery (GKS) have been shown for BM≤10, but there is no consensus on the upper limit where GKS is no longer beneficial. We hypothesize that selected patients with ≥20 BM may benefit by replacing WBRT with GKS to preserve neurocognition without compromising intracerebral tumor control and overall survival, with additional treatments as needed. Methodology This is retrospective analysis of 31 patients with ≥20 BM who underwent single-session GKS between 2016–2021. Twenty-two patients had ECOG of 0 at the time of GKS. Median number of BM at GKS was 30 (20–79) with median total tumour volume 4cm3 (2–28 cm3). Median marginal dose was 20Gy (10-25Gy). Results Median overall survival following GKS was 14-months (95%CI 4-24months), justifying GKS in this population. 11/12 patients that died succumbed due to extracranial disease, while 1 patient, who was treated with WBRT before GKS, succumbed to intracranial tumor progression. Local tumor control achieved was achieved for 63% of patients at 2-years and distal tumor control in 24% of patients at 1.5-years without additional radiation treatment. Salvage GKS was given in seven patients and salvage WBRT in three. One local recurrence was surgically resected. Systemic treatment given to most patients probably contributed to intracranial tumor control. No patients developed significant neurocognitive deficits attributable to GKS during the follow-up period of median 7-months (Q1-Q3: 3-12months). Conclusion Most patients treated with GKS for ≥20 BM have sufficient survival time to benefit from the treatment. Local and distal recurrences can be managed with systemic treatment, salvage GKS, or WBRT, resulting in intracerebral tumor control in vast majority of cases.
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