Pediatric primary intramedullary spinal cord glioblastoma

Spinal cord tumors in pediatric patients are rare, representing less than 1% of all central nervous system tumors. Two cases of pediatric primary intramedullary spinal cord glioblastoma at ages 14 and 8 years are reported. Both patients presented with rapid onset paraparesis and quadraparesis. Magnetic resonance imaging in both showed heterogeneously enhancing solitary mass lesions localized to lower cervical and upper thoracic spinal cord parenchyma. Histopathologic diagnosis was glioblastoma. Case #1 had a small cell component (primitive neuroectodermal tumor-like areas), higher Ki67, and p53 labeling indices, and a relatively stable karyotype with only minimal single copy losses involving regions: Chr8;pter-30480019, Chr16;pter-29754532, Chr16;56160245–88668979, and Chr19;32848902-qter on retrospective comparative genomic hybridization using formalin-fixed, paraffin-embedded samples. Case #2 had relatively bland histomorphology and negligible p53 immunoreactivity. Both underwent multimodal therapy including gross total resection, postoperative radiation and chemotherapy. However, there was no significant improvement in neurological deficits, and overall survival in both cases was 14 months.This report highlights the broad histological spectrum and poor overall survival despite multi modality therapy. The finding of relatively unique genotypic abnormalities resembling pediatric embryonal tumors in one case may highlight the value of genome-wide profiling in development of effective therapy. The differences in management with intracranial and low-grade spinal cord gliomas and current management issues are discussed

Pituitary surgery: experience from a large network in Central Switzerland

PRINCIPLES: During the past years our group built a care network for patients with pituitary tumours with referrals from the midlands and the central part of Switzerland, comprising about 1.6 million inhabitants. The purpose of this retrospective observational study with longitudinal data is to review the experience of pituitary surgery and the operative outcome within this Swiss-wide largest network. METHODS: A total of 182 patients operated at the Neurosurgical Department of the Kantonsspital Aarau 2005-2010 were included in this study. The follow-up was 3.6+/-1.6 years. RESULTS: The following lesions were found: non-functioning adenoma (n = 114; 63%); macroprolactinoma (n = 18; 10%); microprolactinoma (n = 11; 6%); acromegaly (n = 11; 6%), Cushing's disease (n = 7; 4%); Rathke's cleft cyst (RCC; n = 9; 5%); others (n = 12; 7%). Intraoperative MRI (iMRI) was used in 115 (63%) patients. Preoperatively, hypopituitarism was found in 105 (58%) patients. Postoperative recovery of defunct axes was detected in 48%. Visual field and visual acuity deficits due to optic pathway compression by tumour were detected in 48% and 41% of the patients, respectively. Postoperative recovery of visual function was seen in 89%. The increase of total resection rate by iMRI was statistically significant (p = 0.0007). Recurrent tumour growth was seen in 5 (3%) patients during follow-up. CONCLUSIONS: Transsphenoidal surgery is the primary treatment for most sellar lesions. The use of iMRI may lead to higher gross total resection rates. In Switzerland close cooperation between specialised centres is a very positive experience both to support operative case loads and to optimise patient follow-up