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OBJECT - Pediatric cerebellar astrocytomas with pilomyxoid features include classic pilomyxoid astrocytomas (PMAs) and intermediate pilomyxoid tumors (IPTs). Since the original description of PMA in 1999, most reports in the literature have described PMAs arising from the hypothalamic/chiasmatic region. To the authors' knowledge, PMAs arising from the posterior fossa have not been discussed in the neurosurgical literature. Intermediate pilomyxoid tumors, or tumors with pathological features of both pilocytic astrocytoma (PA) and PMA, have only recently been described. In this article, the authors present 2 cases that fall within the spectrum of pediatric cerebellar PMA-including a classic PMA and an intermediate pilomyxoid tumor. The authors compare the radiological presentation, surgical results, and postoperative course to findings in a cohort of 15 patients with cerebellar PAs.
METHODS - Between 2003 and 2010, 2 patients with pilomyxoid-spectrum astrocytomas underwent treatment at Vanderbilt Children's Hospital. One was a 22-month-old girl who presented with progressive gait disturbance and falls. The other was a 4-year-old girl who presented with ataxia and generalized weakness. In a retrospective review of pediatric cerebellar neoplasms resected by the senior author during this period, these tumors comprised 4% of cerebellar neoplasms and approximately 10% of cerebellar glial neoplasms.
RESULTS - Both patients were treated with midline suboccipital craniotomy for resection. In both cases, tumor invasion anteriorly into the brainstem prevented gross-total resection. the patient in Case 1 was placed on chemotherapy following pathological diagnosis and later developed definitive evidence of leptomeningeal dissemination (LD) 3 years after the operation. The patient in Case 2 was placed on chemotherapy after exhibiting progressive evidence of local recurrence (findings were negative for LD) 12 months following resection.
CONCLUSIONS - Pediatric patients with cerebellar pilomyxoid-spectrum astrocytomas appear to suffer higher rates of local recurrence and LD than pediatric patients with cerebellar PAs.
OBJECT - The use of intraventricular endoscopy to achieve diagnosis or to resect accessible intraventricular or paraventricular tumors has been described in the literature in both adults and children. Traditionally, these techniques have not been used in patients with small ventricles due to the perceived risk of greater morbidity. The authors review their experience with the effectiveness and safety of endoscopic brain tumor management in children with small ventricles.
METHODS - Between July 2002 and December 2009, 24 children with endoscopically managed brain tumors were identified. Radiological images were reviewed by a radiologist blinded to study goals and clinical setting. Patients were categorized into small-ventricle and ventriculomegaly groups based on frontal and occipital horn ratio. Surgical success was defined a priori and analyzed between groups. Trends were identified in selected subgroups, including complications related to pathological diagnosis and surgeon experience.
RESULTS - Six children had small ventricles and 18 had ventriculomegaly. The ability to accomplish surgical goals was statistically equivalent in children with small ventricles and those with ventriculomegaly (83% vs 89%, respectively, p = 1.00). There were no complications in the small-ventricle cohort, but in the ventriculomegaly cohort there were 2 cases of postoperative hemorrhages and 1 case of infection. All hemorrhagic complications occurred in patients with high-grade tumor histopathological type and were early in the surgeon's endoscopic career.
CONCLUSIONS - Based on our experience, endoscopy should not be withheld in children with intraventricular tumors and small ventricles. Complications appear to be more dependent on tumor histopathological type and surgeon experience than ventricular size.