Fluorescence in situ hybridization analyses of chromosome band 1p36 in neuroblastoma detect two classes of alterations

Chromosomal alterations in 1p36 were investigated in 196 neuroblastoma tumors using fluorescence in situ hybridization. Additionally, by using the same technique, it was determined whether MYCN was amplified in 149 of these. The most frequent finding was a deletion in 1p36, leading to monosomy of this region (29 cases, 15%). Furthermore, we found tumors with at least two intact copies of chromosome 1 and additional 1p36-deleted copies. Altogether, 21 tumors (11%) displayed this imbalance of 1p36. Similar to the cases with deletion, imbalances were predominantly found in stage 4 tumors (81%), and they were significantly associated with an increased patient age (P = 0.01). Nearly all 1p-deleted tumors showed amplification of MYCN (24/27 analyzed samples, 89%), whereas only 8 of 21 (38%) with imbalance did. Eight cases with imbalance were investigated for loss of heterozygosity (LOH) using microsatellite markers in 1p35-36. Only 4 displayed 1p36 LOH, whereas the remaining 4 were heterozygous. Both patients with deletion of 1p and with imbalance had a poor outcome [3-year rate of event-free-survival (EFS): 33 +/- 15% and 41 +/- 15%], which was significantly worse compared to the outcome of patients without 1p alterations (3-year EFS: 70 +/- 5%; P = 0.01 and P = 0.0059). We conclude that besides monosomic short arm deletions, imbalance of 1p36 is a strong marker of a poor prognosis in neuroblastoma and not necessarily associated with MYCN amplification and LOH.     

Lymphocytic adenohypophysitis mimicking a pituitary macroadenoma

A 24-year-old woman developed headache and rapidly progressive visual disturbances during the last trimester of her first pregnancy. Magnetic resonance tomography (MRI) of the brain documented an intra- and suprasellar mass lesion. For preservation of vision, transsphenoidal microsurgical decompression was performed. Immediately postoperatively, visual acuity improved and hemianopia resolved. Histological examination yielded the diagnosis of primary lymphocytic adenohypophysitis. This is a rare inflammatory pituitary disease. There are no typical clinical, laboratory, or radiological findings that allow precise preoperative diagnosis. Even though this autoimmune disorder is principally steroid-responsive, an improvement of visual disturbances under steroid therapy cannot be predicted. Therefore, surgery is justified not only to establish the diagnosis, but also to restore vision by decompression of the optic nerves and the chiasm.     

Ganglioside Profiles in Human Gliomas: Quantification by Microbore High Performance Liquid Chromatography and Correlation to Histomorphology and Grading

The composition and the content of gangliosides changes during physiological growth and differentiation as well as in neoplastic cell transformation. In order to determine if ganglioside profiles correlate with brain tumour malignancy, the ganglioside distribution was determined in 31 gliomas of astrocytic origin and in non-tumour tissue by a recently developed microbore high performance liquid chromatography (HPLC) method. Glioma malignancy was graded according to the grading system proposed by the World Health Organization (WHO) in 1993. In general, an increase of GD3 and a decrease of normal brain gangliosides correlated with a higher grade of malignancy. Pilocytic astrocytomas Grade I had a distinctive ganglioside profile, histologically as well as biochemically. Although they are low-grade gliomas, the pilocytic astrocytomas exhibited a GD3 content comparable to anaplastic gliomas and could only be biochemically distinguished from other tumour grades by relatively high type "b" ganglioside levels. Thus, ganglioside composition not only reflects anaplasia but can also be used to indicate biological characteristics of tumours of different histogenetic origin.     

The fronto-orbital osteotomy as plastic-reconstructive approach to the anterior and middle skull base.

INTRODUCTION: A combined extra-intracranial access for the operative exploration of tumours of the anterior and middle skull base is indicated when the tumour extends intracranially and simultaneously into the nasal cavity, the paranasal sinuses or the orbit. METHODS: Two standardized modifications of the fronto-orbital osteotomy, the fronto-orbito-nasal and the fronto-orbito-zygomatic osteotomy, allow safe removal of skull base tumours in these locations. In extensive skull base tumours, a modified bilateral fronto-orbital-zygomatic osteotomy can be used. RESULTS: Between February 1993 and July 2000 skull base tumours in 111 patients were resected using the presented methods. The most frequent tumour type was meningioma in 29 cases. Complications were encountered in 13 cases (11.7%). CONCLUSION: The advantages over other approaches are good extra- and intracranial overview and minimal cerebral trauma. Additional transfacial incisions are not usually necessary. Exact repositioning of the fronto-orbital segments leads to optimal aesthetic results.     

Structure, use, and risks of biomaterial repositories of embryonal tumors

Availability of statistically sufficient numbers of tumor samples and other biomaterials in high quality together with corresponding clinical data is crucial for biomedical research. Tumor repositories from individual scientists are mostly not sufficient to satisfy these criteria, especially since pediatric tumors are rare. In 2000 three centralized tumor repositories (neuroblastoma in Cologne, nephroblastoma in Würzburg, hepatoblastoma, brain tumors in Bonn) have been established by the "German Competence Net Pediatric Oncology und Hematology". The aim was to collect biomaterial including tumor samples, normal tissue, and blood in high quality for research and diagnostic purposes at a central institution. Informed consent of the parents or patients is a prerequisite for scientific use of the samples and is requested by the therapy trial. The samples are collected according to accepted standards and shipped in the specially designed Tumorbox. The tumor repository organizes the distribution of the samples to the cooperating diagnostic laboratories. The number of collected tumor samples has increased over the years. In 2000, samples from 200 patients were collected while the patient number increased to 321 in 2005. Over the years the tumor repositories collected more than 7,150 samples (fresh frozen tumor, fresh frozen normal tissue, and blood). Through links with clinical trial databases the samples can be connected with clinical data. 12 of 14 applications for tumor material to be used in specific scientific projects have been approved by an independent supervisory board. The establishment of central tumor repositories represents a major step for biomedical research activities and quality control in pediatric oncology.     

Delayed bilateral craniectomy for treatment of traumatic brain swelling in children: case report and review of the literature

Introduction Head injury is the leading cause of accidental death in children. Recent reports have shown the benefit of decompressive craniectomy in children and the role of early timing has been emphasized. However, there is still a lack of data to determine the optimal time for performing craniectomy.Case report In contrast to most reports in the literature, this case report demonstrates successful bilateral decompressive craniectomy in a 10-year-old girl with multiple posttraumatic intracranial lesions and massive traumatic brain swelling on the 8th posttraumatic day.Conclusions Various pathophysiological mechanisms in the genesis of posttraumatic brain swelling make different treatment strategies necessary. Continuous monitoring of intracranial pressure (ICP), as well as serial cranial computed tomography (CCT), can help to differentiate between these mechanisms. Furthermore, repeated clinical and neurophysiological investigations are important for the timing of craniectomy.A commentary on this paper is available at