Imaging Features with Histopathologic Correlation of CNS High-Grade Neuroepithelial Tumors with a BCOR Internal Tandem Duplication.

BACKGROUND AND PURPOSE: A new brain tumor entity occurring in early childhood characterized by a somatic BCL6 corepressor gene internal tandem duplication was recently described. The aim of this study was to describe the radiologic pattern of these tumors and correlate this pattern with histopathologic findings. MATERIALS AND METHODS: This retrospective, noninterventional study included 10 children diagnosed with a CNS tumor, either by ribonucleic acid-sequencing analysis or deoxyribonucleic acid methylation analysis. Clinical, radiologic, and histopathologic data were collected. A neuropathologist reviewed 9 tumor samples. Preoperative images were analyzed in consensus by 7 pediatric radiologists. RESULTS: All tumors were relatively large (range, 4.7-9.2 cm) intra-axial peripheral masses with well-defined borders and no peritumoral edema. All tumors showed mild and heterogeneous enhancement and marked restriction on DWI of the solid portions. Perfusion imaging showed a relatively lower CBF in the tumor than in the adjacent normal parenchyma. Nine of 10 tumors showed areas of necrosis, with the presence of hemorrhage in 8/10 and calcifications in 4/7. Large intratumoral macroscopic veins were observed in 9/10 patients. No intracranial or spinal leptomeningeal dissemination was noted at diagnosis. CONCLUSIONS: CNS tumors with a BCL6 corepressor gene internal tandem duplication present as large intra-axial peripheral masses with well-defined borders, no edema, restricted diffusion, weak contrast enhancement, frequent central necrosis, hemorrhage and calcifications, intratumoral veins, and no leptomeningeal dissemination at the time of diagnosis. Knowledge of these imaging characteristics may aid in histologic, genomic, and molecular profiling of brain tumors in young children.

Soft tissue sarcoma in children, adolescents and young adults: Outcomes according to compliance with international initial care guidelines.

BACKGROUND: Outcomes for adults with soft tissue sarcoma are better when managed at referral centers. Care guidelines advise for 5 main criteria: 1-Imaging before biopsy; 2-Tumor biopsy before surgery; 3-Multidiscipinary team discussion (MTD) before biopsy; 4-Biopsy in "expert centers"; 5-Somatic molecular biology feasible. The aim is to describe and assess the prognostic impact of initial management of STS according to the type of referring centers and the number of optimal criteria. METHODS: Monocentric retrospective analysis of the management of 127 youths (0-25 years) with localized STS treated from 2006 to 2015. RESULTS: Median age at diagnosis was 9.6 years (range: 025). Overall, only 41% patients had 5/5, 28% 3-4, 31% ≤2. No adequate imaging was performed before surgery/biopsy for 18% patients, no biopsy before treatment for 29%. Patients referred by "expert centers" had higher compliance to guidelines (P = 0.025). Upfront surgery was performed in 59/127 patients. Immediate re-operation was inversely related to the number of criteria (0% when 5 criteria vs. 14% for 3-4, 46% if ≤ 2; P < 0.001). For malignant tumors, outcome was better when 5 criteria were reached: 5 year EFS 90.8% (81.4-100.0%) vs. 71.6 for (60.4-84.9%; ≤4 criteria; p = 0.033), OS 93.6% (85.5-100%) vs. 79.5% (68.9-91.8%; p = 0.11), and LRFFS 90.6% (81.0-100.0) vs. 73.1% (62.0-86.3%; p = 0.047). CONCLUSION: Less than half of the youths with STS are initially managed according to international guidelines, highlighting the need for better information about optimal management. These results plead for immediate management in reference centers to reduce initial burden of therapy.

Postextubation pulmonary edema: an unusual cause of transient pulmonary edema.

We report a case of sudden onset of respiratory distress caused by pulmonary edema due to laryngospasm. The diagnosis was established by the clinical context and chest X-ray. A CT-scan was performed to narrow down the differential diagnosis and to confirm the diagnosis. Postextubation pulmonary edema due to laryngospasm is a rare entity with a typical clinical and radiographic presentation.

The role of magnetic resonance imaging in the diagnostic work-up of fetal ventriculomegaly.

The indication for fetal magnetic resonance imaging (MRI) remains a subject of debate, partly because of questions concerning its diagnostic accuracy compared to ultrasound, partly because of practical factors such as accessibility, high costs and available expertise. Most studies advocate an added value for MRI in cases diagnosed with central nervous system pathology. MRI is a good modality to detect small foci of brain hemorrhage, to depict callosal anomalies, to add information about normal and pathological cortical development, and is a more sensitive imaging method to detect white matter pathology. This manuscript discusses the role of MRI as an adjunct to ultrasound for cases diagnosed-- with cerebral ventriculomegaly.

Limbal transplantation after chemical injuries of the eye.

PURPOSE: To illustrate the benefit of limbal stem cell transplantation in three eyes with severe ocular surface failure due to chemical burns. METHODS: In two patients with monocular corneal scarring and vascularization after chronic chemical burns, a limbal tissue autograft was transferred from the unaffected fellow eye. A complete superficial keratectomy was performed on the host eye. One patient with bilateral ocular surface disorder received an eccentrically trephined corneolimbal allograft. To prevent immunologic rejection of the transplanted limbus, this patient was treated with systemic Ciclosporin A. RESULTS: Postoperatively the limbal autografts grew a normal epithelium on the recipient eye with less vascularization and scarring. Our two patients reported a significant reduction in symptoms (redness, pain, photophobia) and an improved visual acuity. The corneolimbal allograft has remained clear for five months postoperatively. CONCLUSION: In strictly unilateral conditions of limbal deficiency, transplantation of healthy limbal tissue from the normal fellow eye may result in a stable ocular surface and a quiet and comfortable eye. Transplantation of an eccentrically trephined corneolimbal allograft under systemic Ciclosporin A cover may be an option in the rehabilitation of patients with severe bilateral stem cell deficiencies.

[Current computer utilization by 2 surgical services: start-up problems]. / Huidig computergebruik in twee chirurgische diensten: de moeilijkheden bij het opstarten.

There are almost no hardware problems. Before buying a computer, one should carefully investigate the number of different tasks to be automated. The major problem is the kind of software to use for this tasks. It is obvious that the software possibilities determine the choice of the hardware type.