The clinical manifestations of HH are correlated with the topology of the HH in relation to the hypothalamus. Our results suggest that patients with Type IV HH have the best outcome from surgery and neurosurgeons should be cautious about performing surgery on patients with Type II and Type III HH.
Shunt dependency syndrome is a rare but serious complication of shunting an arachnoid cyst. This condition is similar to the slit ventricle syndrome, but also has some differences. ICP monitoring may confirm the diagnosis when there are no significant radiological findings. Achievement of a shunt-free state might be the ultimate goal for all shunted patients.
BackgroundMedulloblastoma (MB) has been classified into four core subgroups according to the transcriptional profile in recent years. However, some disagreement among researchers remains regarding the prognoses and most effective treatments of the different subgroups with different age distributions.ObjectiveThe objective of this study was to analyze MB prognosis in children population based on the classification of four molecular subgroups.MethodsFrom January 2011 to January 2013, 84 consecutive MB patients aged underwent tumor removal at Beijing Tiantan Hospital. A total of 55 patients who ranged in age from 4 to 18 years underwent detailed follow-up. Molecular subgrouping was performed using RT-PCR.ResultsThe 2-year progression-free survival (PFS) and overall survival (OS) rates for the entire cohort were 76.2 ± 5.8 and 81.8 ± 5.2%, respectively. Univariate analysis revealed that the Group 4 patients had a better survival (2-year OS, 90.6 ± 5.2%) than the SHH subgroup (P = 0.002) and Group 3 patients (P = 0.008). Only two of the 23 non-metastasized Group 4 patients relapsed, and chemotherapy did significantly affect these patients (PFS, P = 0.685). One out of five WNT patients had tumor relapse and died at last. Large cell/anaplastic (LC/A) histology and chemotherapy were independent risk factors in multivariate analysis.ConclusionIn our study, the non-metastasized Group 4 patients had an excellent prognosis. The SHH subgroup and Group 3 patients had worst prognoses. LC/A histology had a dismal prognosis in our cohorts, which warrants intensive treatment.
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