Treatment and outcomes of high-risk neuroblastoma in Southeast Asia: a single-institution experience and review of the literature.

Introduction: In Europe and North America, the majority of children with high-risk neuroblastoma survive the disease. Elsewhere, the treatment outcomes are poor. Methods: A retrospective review of children treated for high-risk neuroblastoma in a single institution in Singapore from 2007 to 2019 was carried out. Treatment consisted of intensive chemotherapy, surgery aimed at gross total resection of residual disease after chemotherapy, consolidation with high-dose therapy followed by autologous stem cell rescue, and radiotherapy to the primary and metastatic sites followed by maintenance treatment with either cis-retinoic acid or anti-disialoganglioside monoclonal antibody therapy. Survival data were examined on certain clinical and laboratory factors. Results: There were 57 children (32 male) treated for high-risk neuroblastoma. Their mean age was 3.9 (range 0.7-14.9) years. The median follow-up time was 5.5 (range 1.8-13.0) years for the surviving patients. There were 31 survivors, with 27 patients surviving in first remission, and the five-year overall survival and event-free survival rates were 52.5% and 47.4%, respectively. On log-rank testing, only the group of 17 patients who were exclusively treated at our centre had a survival advantage. Their five-year overall survival rate compared to patients whose initial chemotherapy was done elsewhere was 81.6% versus 41.1% (P = 0.011), and that of event-free survival was 69.7% versus 36.1% (P = 0.032). Published treatment results were obtained from four countries in Southeast Asia with five-year overall survival rates from 13.5% to 28.2%. Conclusion: Intensified medical and surgical treatment for high-risk neuroblastoma proved to be effective, with superior survival rates compared to previous data from Southeast Asia.

Idiopathic normal pressure hydrocephalus: correlating magnetic resonance imaging biomarkers with clinical response.

Idiopathic Normal Pressure Hydrocephalus (NPH) is a debilitating condition of the elderly. The patient is typically "wet, wobbly and wonky", to different degrees of the triad. The diagnosis is supported by the radiologic finding of dilated ventricles, determined by an elevated Evan's Index (EI) without a demonstrable cause. Patients with newly diagnosed NPH typically respond to ventriculo-peritoneal shunting (VPS). NPH-related dementia is possibly the only surgically reversible dementia. An elevated cerebrospinal fluid (CSF) fl ow rate (FR) is associated with a positive response to shunting. However, post-shunting EI and FRs are unpredictable. Of late, intracranial apparent diffusion coefficient (ADC) quantification via Diffusion Weighted Imaging (DWI) has been emerging as a possible marker in NPH diagnosis. A local study, conducted on a national level, to study the relationship of EI, FR and ADC to pre- and post-shunt clinical measurements has just ended. This review seeks to reconcile the current thinking of NPH, magnetic resonance imaging (MRI) quantification and clinical evaluation, and in the process shed some light on major pathophysiological determinants of the disease.

Value of quantitative MRI biomarkers (Evans' index, aqueductal flow rate, and apparent diffusion coefficient) in idiopathic normal pressure hydrocephalus.

PURPOSE: To define the value of Evans' index (EI), aqueductal flow rate (FR), and apparent diffusion coefficient (ADC) in the diagnosis of normal pressure hydrocephalus (NPH) and to assess the ability of these markers preoperatively to predict shunt response. To shed some light as to the mechanisms responsible for the symptoms of NPH. MATERIALS AND METHODS: Preoperative EI, FR, and ADC readings in nine cases of clinically diagnosed NPH were compared with those of age- and gender-matched controls. Similar pre- and postoperative readings of responders and nonresponders were subsequently compared. RESULTS: Compared with the controls, all measurements were statistically significant except for peak systolic flow rate (pSfr), which was near statistical significance. Comparison of pre- and postoperative readings of responders and nonresponders revealed a decrease in ADC in all responders (P = 0.032). Subdural hemorrhage was found in all nonresponders (P = 0.012). CONCLUSION: For patients presenting with signs and symptoms of NPH, readings on MRI greater than 0.3, 10 mL/min, -9.0 mL/min, and 10.65 x 10(-4) mm(2)/s for EI, peak diastolic flow rate (pDfr), pSfr, and ADC, respectively, add further weight to the diagnosis. The strong correlation between shunt response and ADC decline support our hypothesis that water accumulation in the cerebrum is the major cause for the symptoms of NPH. The presence of subdural hemorrhage in all nonresponders raises suspicion of decreased compliance as the other major cause.

A developmental theory of the superior sagittal sinus(es) in craniopagus twins.

INTRODUCTION: Based on the study of the vascular anatomy of craniopagus twins, and particularly that of Ladan and Laleh, a theory is hypothesised regarding the embryological formation of the sagittal sinuses. THEORY AND DISCUSSION: The superior sagittal sinus develops in contact with the calvarium bone within the inter-periosteal leptomeningeal space following a signal from the midline of the telencephalic vesicle derived from a single prosencephalic one. The position of the falx cerebri and most likely in the deepest part, the corpus callosum, points to the position of this midline.