Relative survival of patients with non-malignant central nervous system tumours: a descriptive study by the Austrian Brain Tumour Registry.

BACKGROUND: Unlike malignant primary central nervous system (CNS) tumours outcome data on non-malignant CNS tumours are scarce. For patients diagnosed from 1996 to 2002 5-year relative survival of only 85.0% has been reported. We investigated this rate in a contemporary patient cohort to update information on survival. METHODS: We followed a cohort of 3983 cases within the Austrian Brain Tumour Registry. All patients were newly diagnosed from 2005 to 2010 with a histologically confirmed non-malignant CNS tumour. Vital status, cause of death, and population life tables were obtained by 31 December 2011 to calculate relative survival. RESULTS: Overall 5-year relative survival was 96.1% (95% CI 95.1-97.1%), being significantly lower in tumours of borderline (90.2%, 87.2-92.7%) than benign behaviour (97.4%, 96.3-98.3%). Benign tumour survival ranged from 86.8 for neurofibroma to 99.7% for Schwannoma; for borderline tumours survival rates varied from 83.2 for haemangiopericytoma to 98.4% for myxopapillary ependymoma. Cause of death was directly attributed to the CNS tumour in 39.6%, followed by other cancer (20.4%) and cardiovascular disease (15.8%). CONCLUSION: The overall excess mortality in patients with non-malignant CNS tumours is 5.5%, indicating a significant improvement in survival over the last decade. Still, the remaining adverse impact on survival underpins the importance of systematic registration of these tumours.

Anatomical accuracy of abdominal lesion localization. Retrospective automatic rigid image registration between FDG-PET and MRI.

UNLABELLED: Software-based image registration can improve the diagnostic value of imaging procedures and is an alternative to hybrid scanners. The aim of this study was to evaluate the anatomical accuracy of automatic rigid image registration of independently acquired datasets of positron emission tomography with 18F-deoxyglucose and abdominal magnetic resonance imaging. PATIENTS, METHODS: Analyses were performed on 28 abdominal lesions from 20 patients. The PET data were obtained using a stand-alone PET camera in 14 cases and a hybrid PET/CT scanner in 9 cases. The abdominal T1- and T2-weighted MRI scans were acquired on 1.5 T MRI scanners. The mean time interval between MRI and PET was 7.3 days (0-28 days). Automatic rigid registration was carried out using a self-developed registration tool integrated into commercial available software (InSpace for Siemens Syngo). Distances between the centres of gravity of 28 manually delineated neoplastic lesions represented in PET and MRI were measured in X-, Y-, and Z-direction. The intra- (intraclass correlation 0.94) and inter- (intraclass correlation 0.86) observer repeatability were high. RESULTS: The average distance in all MRI sequences was 5.2±7.6 mm in X-direction, 4.0±3.7 mm in Y-direction and 6.1±5.1 mm in Z-direction. There was a significantly higher misalignment in Z-direction (p<0.05). The misalignment was not significantly different for the registration of T1- and T2- weighted sequences (p=0.7). CONCLUSION: The misalignment between FDG-PET and abdominal MRI registered using an automated rigid registration tool was comparable to data reported for software-based fusion between PET and CT. Although this imprecision may not affect diagnostic accuracy, it is not sufficient to allow for pixel-wise integration of MRI and PET information.

Histopathological prognostic factors in medulloblastoma: high expression of survivin is related to unfavourable outcome.

Standard postoperative treatment of medulloblastoma consists of craniospinal irradiation and chemotherapy. Currently, only clinical factors are used for therapy stratification. To optimise treatment and patient outcome, biological prognostic markers are needed. In the present study we tested the prognostic influence of four histopathological parameters considered in recent publications as prognostic factors in medulloblastoma. We analysed a series of 82 Austrian medulloblastoma patients who were treated according to the consecutive HIT protocols for medulloblastoma conducted by the German Society of Paediatric Haematology and Oncology. Histological subtype and immunohistochemical expression of erbB-2, TRKC, and survivin were determined on paraffin embedded tumour tissue and correlated with patient outcome. Statistical analysis showed a significant correlation of high expression levels of survivin with decreased survival. None of the other investigated histopathological factors correlated significantly with patient outcome. Our data indicate that high survivin expression is related to unfavourable clinical outcome in medulloblastoma patients.

[Radiation exposure of health personnel and patients in the heart catheterization laboratory in during vascular brachytherapy]. / Untersuchungen zur Strahlenexposition von Beschäftigten und Patienten im Herzkatheterlabor und bei der vaskulären Brachytherapie.

Interventional radiological measures can lead to high radiation exposures for medical staff. In order to determine the radiation exposure to staff and patients, the resulting radiation exposures were directly measured for 52 measures at an cardiac catheterization laboratory with a new dosimetry system DIS (Direct Ion Storage). Beside the measurement of body dose behind the lead apron, measurements of radiation doses were performed in front of the lead apron and at the wrist of the physician. These measurements were taken as an approximation of the radiation exposure of the non-shielded body parts. The patients dose was estimated by placing a dosemeter close to the head of the patient and from the dose-area product. The mean value of body dose from 52 measurements for the physician behind the lead apron was 1.9 microSv per procedure with a range of 0-9 microSv. In front of the lead apron, a mean value of 53.9 microSv (3-233 microSv) per procedure was obtained. The mean value of partial body dose at the physician's wrist was determined to be 163.2 microSv (12-603 microSv) per procedure. It could be shown that measures combined with interventions lead to higher exposures compared with measures without interventions. For the medical technician, the mean value behind the lead apron was 3.9 microSv (0-58 microSv) per procedure. For the patient, a mean value of 800 microSv (119-8642 microSv) was measured close to the head. The mean dose to the skin of the patient at radiation entrance was determined to be 307 mGy (70-1190 mGy). From this data, the radiation dose per year for the physician performing 1000 measures, was determined to be 1.9 mSv/year. This is below the new dose limit of 20 mSv/year. Also the estimations for the dose to the eye lens and the hands of the physician show no conflicts with actual dose limits when obeying all radiation regulations. The results for vascular brachytherapy did not show significantly higher exposures, compared with conventional measures including interventions.

Glomangioma of the nasal cavity and paranasal sinuses.

The glomangioma, a benign vascular tumour, derived from the cutaneous glomus bodies, should not be confused with paragangliomas, which are occasionally also referred to as glomus tumours. Up to now, only eleven cases of a glomangioma of the nasal cavity and the paranasal sinuses have been published. We report the case of a patient with a glomangioma of the ethmoidal aircell system. The tumour was completely removed under endoscopic-microscopic vision via endonasal access. A tumour recurrence was not observed over a period of eighteen months. The clinical signs, diagnosis, therapy and histological features will be discussed with reference to the literature.

Determination of the cellular retinoic-acid-binding protein in dysplastic epithelia of the cervix uteri, differentiated into apo and holo forms.

PURPOSE: The appearance of the cervical mucosa is regulated by different factors including retinoic acid. Hormone-dependent alteration of the cervix uteri mucosa is accompanied by a decrease or increase of cytoplasmatic retinoic-acid-binding protein (CRABP). To elucidate whether this hormone-dependent alteration of CRABP is preserved in the case of neoplasms of the cervix uteri, we measured the level of total and apo-CRABP in normal and neoplastically transformed cervical cells. METHODS: In a prospective pilot study, standardised biopsies of normal epithelium and cervical intra-epithelial neoplasm grade 3 (CIN III) were taken from 24 patients. A newly developed method was used to determine the intra-epithelial level of apo- and total CRABP. RESULTS: The concentration of total CRABP in normal squamous epithelium compared with that in intra-epithelial neoplasm grade 3 is very significantly lower in the CIN III areas (normal: 3.66 +/- 1.46 pmol/ mg wet weight +/- SD; CIN III 1.43 +/- 0.59 pmol/mg P < 0.01). In addition CRABP in the apo form is lower in normal than in neoplastic epithelium (Wilcoxon test for paired non-parametric values: P < 0.05; mean for all patients: normal: 1.65 + 0.82 pmol/mg; CIN III: 1.14 +/- 0.23 pmol/mg). CONCLUSION: From our results we conclude that, in neoplastically transformed cells, the hormone-dependent CRABP cycle is interrupted. Whether this has consequences for the further development of the neoplastic cells has to be elucidated.

[Investigation of the frequency of heterozygotes for galactose-1-phosphate-uridyl-transferase-deficiency(galactosemia) in the Vienna area. Comparison with the frequency of homozygotes found by newborn screening (author's transl)]. / Untersuchung über die Häufigkeit von Heterozygoten für Galaktose-1-Phosphat-Uridyl-Transferase-Mangel (Galaktosmie) im Raum von Wien. Vergleich mit der durch Neugeborenen-Screening gefundenen Homozygoten-Häufigkeit

By routine newborn screening for galactose blood level elevations a great difference in the frequency of Galactosemia by transferase deficiency was observed between Eastern- and Western-Austria. This made enzymatic heterozygousity determinations desirable. At the same time it should be examined whether the frequency of homozygotes found by screening correlates with the frequency derived from the heterozygousity frequency. In a first step the region of Vienna was studied. 377 unselected blood samples were examined following BEUTLER-BALUDA's method. As technical control samples of 9 homozygotes and 12 heterozygotes as found by screening were examined but not included in the frequency study. Arranged following age groups a slight but significant decrease of enzym activity with increasing age could be confirmed (23,89 versus 21,99 U/g Hb). This, increasing the overlapping between normals and heterozygotes of different ages possibly should be kept in mind in individual heterzygousity determinations. The enzymatically determined frequency of heterozygotes was found to be twice as high as the frequency calculated from the frequency of biochemically detected but enzymatically proven homozygotes (1:53 versus 1:99). A comparison of the heterozygousity frequencies found in literature with the results of the most efficient (technically most reliable) screening programs shows that this discrepancy is great and general. Bases on heterozygousity frequencies the frequency of homozygotes found by screening programs seems to be at least 50% too low. Analysis of theoretical possibilities for loss of homozygotes by screening programs makes this explanation highly improbable. It seems more probable that the enzymatically determined heterozygotes frequency is too high by including still undefined allels or allel combinations which result in about half normal activity but not in disturbances resembling classical Galactosemia.

Purification of filamentous plant viruses by thin layer centrifugation.

The construction of a modified thin layer ultracentrifuge rotor is described. This rotor was used in the purification of five filamentous plant viruses, viz. TMV, SCMV, PVX, SGV and YMC. The purification and concentration of these viruses in their monomeric forms is hazardous when conventional "tube" rotors are used since they invariably result in dissociation and aggregation of the virus particles. Using the thin layer rotor these infective agents may be concentrated in volumes of fluid equal to approximately 1% of the starting suspension and not as pellets obtained after ultracentrifugation is conventional "tube" rotors. Electron microscopy revealed that the virus particles concentrated by thin layer centrifugation were not aggregated and that only few fragments of the virus filaments were present in the final preparations.