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Microglia and monocytes play a critical role in immune responses to cerebral ischemia. Previous studies have demonstrated that interferon regulatory factor 4 (IRF4) and IRF5 direct microglial polarization after stroke and impact outcomes. However, IRF4/5 are expressed by both microglia and monocytes, and it is not clear if it is the microglial (central) or monocytic (peripheral) IRF4-IRF5 regulatory axis that functions in stroke. In this work, young (8–12 weeks) male pep boy (PB), IRF4 or IRF5 flox, and IRF4 or IRF5 conditional knockout (CKO) mice were used to generate 8 types of bone marrow chimeras, to differentiate the role of central (PB-to-IRF CKO) vs. peripheral (IRF CKO-to-PB) phagocytic IRF4-IRF5 axis in stroke. Chimeras generated from PB and flox mice were used as controls. All chimeras were subjected to 60-min middle cerebral artery occlusion (MCAO) model. Three days after the stroke, outcomes and inflammatory responses were analyzed. We found that PB-to-IRF4 CKO chimeras had more robust microglial pro-inflammatory responses than IRF4 CKO-to-PB chimeras, while ameliorated microglial response was seen in PB-to-IRF5 CKO vs. IRF5 CKO-to-PB chimeras. PB-to-IRF4 or IRF5 CKO chimeras had worse or better stroke outcomes respectively than their controls, whereas IRF4 or 5 CKO-to-PB chimeras had similar outcomes compared to controls. We conclude that the central IRF4/5 signaling is responsible for microglial activation and mediates stroke outcomes. [ABSTRACT FROM AUTHOR]

Purpose: Choroid plexus tumors (CPT) are relatively rare CNS tumors that primarily occur in children. They are classified as low-grade choroid plexus papilloma, including atypical ones, and high-grade choroid plexus carcinoma based on histological characteristics. There has been extensive academic research regarding these complex tumors. The goal of this work was to identify the 100 most-cited articles pertaining to CPTs in order to better understand the most impactful studies to date. Methods: In August 2023, Elsevier's Scopus database was searched for the 100 most-cited articles about CPT. To look for trends, articles were classified as either basic science or clinical, and the earliest 50 articles were separated from the latest 50 articles and then were compared. Various bibliometric parameters were summarized and compared using Pearson's chi-square exact test and Wilcoxon rank sum test/Mann–Whitney U test. Results: The 100 most-cited articles were published between 1955 and 2016 in 53 different scientific journals, originating from 16 distinct countries. Over 75% of the articles were clinical in nature, and overall mean (range) values were as follows: citation count 78.5 (42–371), citation rate per year 3.4 (0.9–12), number of authors 6.2 (1–28). Newer articles had statistically higher citation rate (P < 0.01) and number of authors (P < 0.01) compared to their older counterparts. Additionally, while there was no significant difference in article focus (P = 0.64), there was a difference in study design (P < 0.01). Conclusion: This study used citation number as a surrogate for article impact and identified the 100 most-cited CPT articles. New mutational analyses have allowed for further subgrouping and positive trends in collaboration shine hope for improvement in treatment outcomes and long-term survival. [ABSTRACT FROM AUTHOR]

Purpose: Surgical resection of pineal region tumors is challenging because of close proximity to the vein of Galen draining system and the quadrigeminal plate. Surgical resection usually is performed through the narrow corridor by piecemeal resection and en bloc resection is difficult in cases of large tumors. Moreover, in cases of hypervascular tumors, surgical resection through the narrow corridor could entail massive intraoperative bleeding. The effectiveness of neoadjuvant chemotherapy and second-look surgery for pineal region tumors for maximal safe resection was evaluated. Methods: Retrospective institutional review of pediatric patients with pineal region tumors who underwent second look surgery after neoadjuvant chemotherapy was performed. Results: Nine patients underwent surgical resection after neoadjuvant chemotherapy over the period of September 2017 to February 2022. The mean age was 7.7 years (ranged from 1.4 to 15.3 years). Three patients underwent partial resection via open craniotomy, and 6 underwent endoscopic biopsy as an initial surgery. The histopathological diagnoses were germ cell tumors in 5 patients (yolk sac tumors in 2, germinoma in 2, choriocarcinoma in 1), /rhabdoid tumor (AT/RT) in 2, medulloblastoma in 1, and high-grade glioneuronal tumor in 1. After several courses of chemotherapy, the second-look surgery was performed. The tumor volume was reduced in 8 patients (89%) after chemotherapy except for 1 case of growing teratoma syndrome. The tumor was extended laterally to the ambient cistern in 2 patients, and posteriorly to the tentorial surface of the cerebellum in 3 patients. The lesion was approached through occipital transtentorial approach in 8 patients and infratentorial supracerebellar approach in 1. Intraoperatively, the high vascularity of the tumor was not observed in all cases. Gross total resection (8 patients, 89%) or near total resection (1, 11%) was achieved in all cases. No complications were observed postoperatively in all cases. Eight patients subsequently underwent additional chemo-radiation therapy according to the initial diagnosis. All patients are alive with no evidence of recurrence with a mean follow-up of 33 months. Conclusions: Neoadjuvant chemotherapy and second-look surgery for pediatric pineal region tumors was considered to be effective in reducing the tumor volume and vascularity, which facilitates the safe maximal tumor resection. [ABSTRACT FROM AUTHOR]

Background: Sporadic multiple meningioma are uncommon. Population-based data suggests that these patients have a reduced overall survival when compared to patients with solitary meningioma. The aim of this study was to investigate the clinical outcomes in multiple and solitary meningioma. Methods: A single-center matched cohort study (2008–2018) was performed. Patients with synchronous multiple meningioma at presentation, with no history of prior intracranial radiation, concurrent hormone replacement therapy or features of NF2-schwannomatosis were included. Eligible patients were matched 1:1 to patients with solitary meningioma. Outcomes of interest were occurrence of an intervention, recurrence, new meningioma development and mortality. Results: Thirty-four patients harboring 76 meningioma at presentation were included. Mean age was 59.3 years (SD = 13.5). Thirty-one (91.2%) were female. The median number of meningioma per patient was 2 (range 2–6). Eighteen patients (52.9%) were symptomatic at presentation. Median overall follow-up was 80.6 months (IQR 44.1–99.6). Compared to patients with a sporadic meningioma, there was no difference in intervention rates (67.6% vs 70.6%, P = 0.792). Eight patients (34.8%) with a multiple meningioma had a WHO grade 2 meningioma compared to 7 (29.2%) with a solitary meningioma (P = 0.679). Median recurrence-free survival was 89 months (95% CI 76–104) with no difference between the two groups (P = 0.209). Mean overall survival was 132 months (95% CI 127–138) with no difference between the two groups (P = 0.860). One patient with multiple meningioma developed two further new meningioma 36 months following diagnosis. Conclusion: Sporadic multiple meningioma may not have worse clinical outcomes. Management of patients with sporadic multiple meningioma should be tailored towards the symptomatic meningioma or high-risk asymptomatic meningioma. [ABSTRACT FROM AUTHOR]

Background: Various complications of endoscopic third ventriculostomy (ETV) have been described. One has to recognize these complications and learn how to avoid them. Methods: We performed a literature review regarding the reported complications of ETV procedures discussed in a correlated manner with the surgical steps. Furthermore, we reviewed the technical notes described by experienced neuroendoscopists, including surgical indications, choice of the endoscopic entry point and trajectory, anatomic orientation, proper bleeding control and tight closure, to prevent and deal with such complications. Results and conclusion: A lesson learned that comprehensive knowledge of ventricular anatomy with proper orientation by studying the preoperative images is mandatory and one should be aware of all complication types and rates. [ABSTRACT FROM AUTHOR]

Craniopharyngioma (CP) is an intracranial benign tumor that behaves aggressively due to its location, infiltration of the surrounding nervous tissue and high capacity for recurrence. Treatment of choice is surgery followed or not by radiotherapy. Recent advances in molecular biology techniques and the better understanding of the genetic alterations of the two histological types of CP have open new therapeutic perspectives with targeted drugs. Adamantinomatous CP (ACP) is associated with activating mutations of the CTNNB1 gene. Such mutations are accompanied by intracellular accumulation of β-catenin, an oncogenic protein that activates the intracellular Wnt/ β-catenin signaling pathway, which regulates the transcription of genes involved in cell proliferation. Therefore, the use of molecular therapies directed against the activation of the Wnt/ β-catenin pathway could be an attractive and promising therapeutic option in the management of ACPs. On the other hand, papillary CP (PCP) is associated with activating mutations in the BRAF gene. This gene encodes a BRAF protein that plays an important role in the intracellular mitogen-activated protein kinase (MAPK) signaling pathway, which also regulates cell proliferation. The use of BRAF inhibitors either in monotherapy or in combination with mitogen-activated protein kinase (MEK) inhibitors has demonstrated therapeutic efficacy in isolated clinical cases of relapsed PCPs. A preliminary report of a recent phase II clinical trial has shown a therapeutic response in 93.7% of patients with BRAFV600E -mutated PCP, with an 85% reduction in tumor size. In the present review we comment on the efficacy and safety of the different drugs being used in patients with PCP. [ABSTRACT FROM AUTHOR]

Purpose: Diffuse Brainstem Glioma (DBG) is a catastrophic brain tumor with a survival rate of less than 10% two years after diagnosis despite the existence of different treatment protocols. Among the devices that use magnetic fields generated by Magnetic Resonance Imaging is Quantum Magnetic Resonance Therapy (QMRT). Methods: Five children diagnosed with DBG in our institution in Mexico City underwent treatment of compassionate use with QMRT between December 2018 and July 2019. A survival analysis was performed with previously reported historical data (n = 15). Results: Two patients (40%) survived after three years of follow-up; the log-rank test showed a statistically significant difference in overall survival between both groups (p = 0.032). All patients tolerated the treatment adequately without reporting any severe clinical or neuroradiological adverse effects. Of the patients included, all showed a decrease in the tumor one month after the end of the treatment, although there was great variability in the response and the difference was not statistically significant (p = 0.06). Conclusions: Although future investigations are needed to confirm the findings reported in the present study, the improvement in survival is promising for a group of patients whose prognosis has been catastrophic over the years. Trial registration NCT03577600. [ABSTRACT FROM AUTHOR]

Introduction: Standards for chemotherapy against choroid plexus tumors (CPT) have not yet been established. Methods: CPT-SIOP-2000 (NCT00500890) was an international registry for all CPT nesting a chemotherapy randomization for high-risk CPT with Carboplatin/Etoposide/Vincristine (CarbEV) versus Cyclophosphamide/Etoposide/Vincristine (CycEV). Patients older than three years were recommended to receive irradiation: focal fields for non-metastatic CPC, incompletely resected atypical choroid plexus papilloma (APP) or metastatic choroid plexus papilloma (CPP); craniospinal fields for metastatic CPC/APP and non-responsive CPC. High risk was defined as choroid plexus carcinoma (CPC), incompletely resected APP, and all metastatic CPT. From 2000 until 2010, 158 CPT patients from 23 countries were enrolled. Results: For randomized CPC, the 5/10 year progression free survival (PFS) of patients on CarbEV (n = 20) were 62%/47%, respectively, compared to 27%/18%, on CycEV (n = 15), (intention-to-treat, HR 2.6, p = 0.032). Within the registry, histological grading was the most influential prognostic factor: for CPP (n = 55) the 5/10 year overall survival (OS) and the event free survival (EFS) probabilities were 100%/97% and 92%/92%, respectively; for APP (n = 49) 96%/96% and 76%/76%, respectively; and for CPC (n = 54) 65%/51% and 41%/39%, respectively. Without irradiation, 12 out of 33 patients with CPC younger than three years were alive for a median of 8.52 years. Extent of surgery and metastases were not independent prognosticators. Conclusions: Chemotherapy for Choroid Plexus Carcinoma is feasible and effective. CarbEV is superior to CycEV. A subset of CPC can be cured without irradiation. [ABSTRACT FROM AUTHOR]

The rhabdoid tumor (RT) predisposition syndromes 1 and 2 (RTPS1 and 2) are rare genetic conditions rendering young children vulnerable to an increased risk of RT, malignant neoplasms affecting the kidney, miscellaneous soft-part tissues, the liver and the central nervous system (Atypical Teratoid Rhabdoid Tumors, ATRT). Both, RTPS1&2 are due to pathogenic variants (PV) in genes encoding constituents of the BAF chromatin remodeling complex, i.e. SMARCB1 (RTPS1) and SMARCA4 (RTPS2). In contrast to other genetic disorders related to PVs in SMARCB1 and SMARCA4 such as Coffin-Siris Syndrome, RTPS1&2 are characterized by a predominance of truncating PVs, terminating transcription thus explaining a specific cancer risk. The penetrance of RTPS1 early in life is high and associated with a poor survival. However, few unaffected carriers may be encountered. Beyond RT, the tumor spectrum may be larger than initially suspected, and cancer surveillance offered to unaffected carriers (siblings or parents) and long-term survivors of RT is still a matter of discussion. RTPS2 exposes female carriers to an ill-defined risk of small cell carcinoma of the ovaries, hypercalcemic type (SCCOHT), which may appear in prepubertal females. RT surveillance protocols for these rare families have not been established. To address unresolved issues in the care of individuals with RTPS and to propose appropriate surveillance guidelines in childhood, the SIOPe Host Genome working group invited pediatric oncologists and geneticists to contribute to an expert meeting. The current manuscript summarizes conclusions of the panel discussion, including consented statements as well as non-evidence-based proposals for validation in the future. [ABSTRACT FROM AUTHOR]

Introduction: Standardisation of imaging acquisition is essential in facilitating multicentre studies related to childhood CNS tumours. It is important to ensure that the imaging protocol can be adopted by centres with varying imaging capabilities without compromising image quality. Materials and method: An imaging protocol has been developed by the Brain Tumour Imaging Working Group of the European Society for Paediatric Oncology (SIOPE) based on consensus among its members, which consists of neuroradiologists, imaging scientists and paediatric neuro-oncologists. This protocol has been developed to facilitate SIOPE led studies and regularly reviewed by the imaging working group. Results: The protocol consists of essential MRI sequences with imaging parameters for 1.5 and 3 Tesla MRI scanners and a set of optional sequences that can be used in appropriate clinical settings. The protocol also provides guidelines for early post-operative imaging and surveillance imaging. The complementary use of multimodal advanced MRI including diffusion tensor imaging (DTI), MR spectroscopy and perfusion imaging is encouraged, and optional guidance is provided in this publication. Conclusion: The SIOPE brain tumour imaging protocol will enable consistent imaging across multiple centres involved in paediatric CNS tumour studies. [ABSTRACT FROM AUTHOR]

Positional plagiocephaly is the most common cause of cranial asymmetry. Deformational brachycephaly denotes a head shape characterized by occipital flattening and increased bilateral width, which can also be caused by external deformation of the moldable infant cranium in positional bilateral posterior plagiocephaly. There are reports of craniosynostosis associated with Chiari I malformation (CIM), possibly caused by decreased posterior fossa volume and related to increased intracranial pressure. To the best of our knowledge, this is only the second case report demonstrating acquired CIM in a child with positional brachycephaly. Of note, the fact that the CIM resolved after helmet therapy could support the hypothesis that CIM is associated with decreased volume of the posterior fossa. However, these two conditions may be independent of one another. More research is needed to identify an association between the two conditions. [ABSTRACT FROM AUTHOR]

Background: The Miethke Sensor Reservoir sits within a ventriculoperitoneal shunt system to give a reading of the pressure within the shunt. This information can guide the management of hydrocephalus patients who present frequently with headaches. Methods: We reviewed a cohort of 12 patients who underwent implantation of a Sensor Reservoir to assess how the management of their symptoms changed over a 4-year period. Results: When comparing the group before the Sensor Reservoir and after the Sensor Reservoir insertion, there was a 75% reduction in number of CT head scans (P<0.05), 100% reduction in episodes of ICP monitoring (P<0.05), 55% reduction in number of X-ray shunt series, and a 50% reduction in acute presentation to hospital with shunt-related symptoms. The number of clinic attendances increased by 44%. In addition, cost analysis showed a saving of £6952 per patients over the 2-year period following Sensor Reservoir insertion as a result of reduced admissions and investigations. Complications were seen in 3 patients—two patients developed shunt-related infections, and 1 patient underwent shunt revision due to a proximal shunt obstruction. Seventy-five percent of patients showed an improvement in their symptoms at the end of the 4-year period. Conclusion: Implantation of a Sensor Reservoir in shunt patients with chronic headaches can reduce the number of investigations and hospital admissions and guide management resulting in a clinical improvement. [ABSTRACT FROM AUTHOR]

The understanding of raised intracranial pressure (ICP) is increasing with the directed use of intracranial telemetric ICP monitors. This case uniquely observed ICP changes by telemetric monitoring in a patient with idiopathic intracranial hypertension (IIH), who developed rapid sight-threatening disease. A lumbar drain was inserted, as a temporising measure, and was clamped prior to surgery. This resulted in a rapid rise in ICP, which normalised after insertion of a ventriculoperitoneal shunt. This case highlighted the utility of the ICP monitor and the lumbar drain as a temporising measure to control ICP prior to a definitive procedure as recommended by the IIH consensus guidelines. [ABSTRACT FROM AUTHOR]

Purpose of Review: Over the last years, our understanding of the molecular biology of pediatric brain tumors has vastly improved. This has led to more narrowly defined subgroups of these tumors and has created new potential targets for molecularly driven therapies. This review presents an overview of the latest advances and challenges of implementing targeted therapies into the clinical management of pediatric brain tumors, with a focus on gliomas, craniopharyngiomas, and medulloblastomas. Recent Findings: Pediatric low-grade gliomas (pLGG) show generally a low mutational burden with the mitogen-activated protein kinase (MAPK) signaling presenting a key driver for these tumors. Direct inhibition of this pathway through BRAF and/or MEK inhibitors has proven to be a clinically relevant strategy. More recently, MEK and IL-6 receptor inhibitors have started to be evaluated in the treatment for craniopharyngiomas. Aside these low-grade tumors, pediatric high-grade gliomas (pHGG) and medulloblastomas exhibit substantially greater molecular heterogeneity with various and sometimes unknown tumor driver alterations. The clinical benefit of different targeted therapy approaches to interfere with altered signaling pathways and restore epigenetic dysregulation is undergoing active clinical testing. For these multiple pathway-driven tumors, combination strategies will most likely be required to achieve clinical benefit. Summary: The field of pediatric neuro-oncology made tremendous progress with regard to improved diagnosis setting the stage for precision medicine approaches over the last decades. The potential of targeted therapies has been clearly demonstrated for a subset of pediatric brain tumors. However, despite clear response rates, questions of sufficient blood-brain barrier penetration, optimal dosing, treatment duration as well as mechanisms of resistance and how these can be overcome with potential combination strategies need to be addressed in future investigations. Along this line, it is critical for future trials to define appropriate endpoints to assess therapy responses as well as short and long-term toxicities in the growing and developing child. [ABSTRACT FROM AUTHOR]

Background: Hydrocephalus may present with heterogeneous signs and symptoms. The indication for its treatment and the optimal drainage in complex cases may be challenging. Telemetric intracranial pressure measurements (TICPM) may open new perspectives for those circumstances. We report our experiences using the Neurovent-P-tel and the Sensor Reservoir in a retrospective study. Methods: A series of 21 patients (age range 10–39.5 years) treated in our Pediatric Neurosurgical Unit receiving a TICPM was analyzed. In 8 patients, a Neurovent-P-Tel was implanted; 13 patients received a Sensor Reservoir, 6 of which as a stand-alone implant, while 7 were already shunted. TICPM were performed on an outpatient basis. Possible complications, follow-up surgeries, and TICPM were analyzed. Results: Concerning the complications, one infection was seen in each group and one postoperative seizure was observed in the P-tel group. TICPM-assisted shunt adjustments lead to clinical improvements in six patients in the P-tel group and six patients in the Sensor Reservoir group. In four out of six non-shunted patients, TICPM contributed to the indication toward shunt implantation. Conclusions: TICPM seems to be a promising tool to improve clinical management of shunted patients with complex hydrocephalus. The two available systems will need further technical improvements, concerning implantation time, measurements, and data analysis in order to optimize handling and interpretation of the data. [ABSTRACT FROM AUTHOR]

1 a Unsupervised hierarchical clustering of DNA methylation profiles in nine YAP1-fused meningiomas (MNG YAP1) alongside 128 well-characterized CNS neoplasms and control tissue shown in a two-dimensional representation of pairwise sample correlations using the 15,000 most variant probes by t-distributed stochastic neighbor embedding (t-SNE) dimensionality reduction. Histological meningioma subtypes meningiomas and WHO grade are provided in Supplementary Table 2 I MNG i meningioma, I PXA i pleomorphic xanthoastrocytoma, I pHGG i pediatric high grade glioma, I F i female, I M i male Seven tumors harbored a rearrangement of I YAP1-MAML2 i involving exons 1-5 ( I n i = 5) or only in exon 1 ( I n i = 2) of I YAP1 i (NM 001130145) and exon 2-5 of I MAML2 i (NM 032427) I . In line, I YAP1 i fusion positive meningiomas clustered closer to I NF2 i mutant cases than other pediatric meningiomas (Online Resource Supplementary Fig. [Extracted from the article]

Purpose: The purpose of this study is to investigate the robustness of pharmacokinetic modelling of DCE-MRI brain tumour data and to ascertain reliable perfusion parameters through a model selection process and a stability test. Methods: DCE-MRI data of 14 patients with primary brain tumours were analysed using the Tofts model (TM), the extended Tofts model (ETM), the shutter speed model (SSM) and the extended shutter speed model (ESSM). A no-effect model (NEM) was implemented to assess overfitting of data by the other models. For each lesion, the Akaike Information Criteria (AIC) was used to build a 3D model selection map. The variability of each pharmacokinetic parameter extracted from this map was assessed with a noise propagation procedure, resulting in voxel-wise distributions of the coefficient of variation (CV). Results: The model selection map over all patients showed NEM had the best fit in 35.5% of voxels, followed by ETM (32%), TM (28.2%), SSM (4.3%) and ESSM (< 0.1%). In analysing the reliability of Ktrans, when considering regions with a CV < 20%, ≈ 25% of voxels were found to be stable across all patients. The remaining 75% of voxels were considered unreliable. Conclusions: The majority of studies quantifying DCE-MRI data in brain tumours only consider a single model and whole tumour statistics for the output parameters. Appropriate model selection, considering tissue biology and its effects on blood brain barrier permeability and exchange conditions, together with an analysis on the reliability and stability of the calculated parameters, is critical in processing robust brain tumour DCE-MRI data. [ABSTRACT FROM AUTHOR]

Background: As technical progress advances, telemonitoring has become an important part of patient care in many areas of medical treatment. However, distanced surveillance of intracranial pressure (ICP) could not be established so far. With the recent introduction of a telemetric ICP measurement probe, new possibilities arise. Here, we report on a new home setup enabling home telemonitoring of intracranial pressure. Methods: Twenty patients suffering from disturbances of cerebrospinal fluid circulation, who underwent insertion of a telemetric ICP measurement probe, were provided with medical equipment to read ICP at home and save the data on an internet-enabled computer. Training in handling the equipment was performed during in-patient stay; recorded and uploaded ICP data was then analyzed online. Therefore, the treating medical team was able to access the ICP data via a secure internet connection while telephone conferencing with the patient. Results: Almost 7400 h of ICP data were recorded at home and evaluated via an internet connection according to the telemonitoring setup. This corresponds to an average record time of about 370 h per patient. ICP profiles were observed following endoscopic treatment, shunting procedures, or valve adjustments. The mean distance between the patients' residence and the consulting hospital was 172 km (range, 16–649 km). Conclusions: ICP measurements have become accessible for telemonitoring purposes. This new management of hydrocephalus reflects an alternative method in patient care, especially for those who live far away from specialized centers. [ABSTRACT FROM AUTHOR]

The aim of the study was to evaluate the B‑flow sonography (BFS) to image the basal cerebral arteries in newborn infants. For this purpose 34 newborns, who underwent standardized ultrasound of the brain including BFS, color Doppler (CDS) and power Doppler (PDS) techniques were retrospectively assessed. Delineation of the anterior communicating (Acom), the posterior communicating (Pcom), the middle cerebral (M1 and M2 segments), the anterior cerebral (A1 and A2 segments), and the posterior cerebral artery (P1 and P2 segments) were visually scored. Vessel delineation was better with BFS compared with CDS and PDS for the M2 segment (p = 0.0006 and p = 0.0136) and P2 segment (p = 0.0021 and p = 0.0014). Superior detectability was also noted for the Pcom with BFS compared with PDS (p = 0.0062). For all other vessel segments no significant differences were found. In conclusion BFS is feasible to image the basal cerebral arteries in newborns with an equal or better vessel delineation compared with standard vascular ultrasound methods. [ABSTRACT FROM AUTHOR]

Long-term ICP monitoring with the Neurovent-P-tel® after ETV for 8 to 12 weeks is especially useful for treatment response prediction since the average time of ETV failure occurs within this time period [[4], [10], [23]]. The Sensor Reservoir® is to be integrated into the shunt system, and there is no limitation about the approved implantation time in opposition to the P-tel probe whose approved implantation time is limited to 90 days. This article is part of the Topical Collection on CSF Circulation The commercial use of telemetric intracranial pressure (ICP) monitoring has been in use for over a decade now. Additionally, it allows the monitoring of the shunt effect and the verification of a proper shunt function over a longer time period in complex shunt patients [[1], [19]]. [Extracted from the article]

Background: Telemetric intracranial pressure (ICP) monitoring enable long-term ICP monitoring on patients during normal day activities and may accordingly be of use during evaluation and treatment of complicated ICP disorders. However, the benefits of such equipment depend strongly on the validity of the recordings and how often the telemetric sensor needs to be re-implanted. This study investigates the clinical and technical sensor survival time and drift of the telemetric ICP sensor: Raumedic Neurovent-P-tel.Methods: Implanted telemetric ICP sensors in the period from January 2011 to December 2017 were identified, and medical records reviewed for complications, explantation reasons, and parameters relevant for determining clinical and technical sensor survival time. Explanted sensors were tested in an experimental setup to study baseline drift.Results: In total, implantation of 119 sensors were identified. Five sensors (4.2%) were explanted due to skin damage, three (2.5%) due to wound infection, and two (1.7%) due to ethylene oxide allergy. No other complications were observed. The median clinical sensor survival time was 208 days (95% CI 150-382). The median technical sensor survival time was 556 days (95% CI 382-605). Explanted sensors had a median baseline drift of 2.5 mmHg (IQR 2.0-5.5).Conclusion: In most cases, the ICP sensor provides reliable measurements beyond the approved implantation time of 90 days. Thus, the sensor should not be routinely removed after this period, if ICP monitoring is still indicated. However, some sensors showed technical malfunction prior to the CE-approval, underlining that caution should always be taken when analyzing telemetric ICP curves. [ABSTRACT FROM AUTHOR]

To assess the value of resection in glioblastoma based on pre-surgical tumor characteristics and a subsequent staging system. The lack of a staging system for glioblastoma hinders the analysis of treatment outcome. We classified 292 uniformly treated glioblastoma patients as stage I, II, or III based on tumor size, location, and eloquence and then analyzed the impact of the extent of resection. We classified 62% of patients as stage I, 25.3% as stage II, and 12.7% as stage III. Gross total resection (GTR) was performed mainly in stage I rather than stage II or III patients (79.2% vs. 14.6% vs. 6.3%; P < 0.001). Overall survival (OS) was 17.7, 14.6, and 10.8 months for stage I, II, and III patients, respectively (P = 0.005). Longer OS was significantly associated with greater extent of resection, younger age, KPS ≥ 70%, MGMT methylation, lower stage, and tumor ≤ 5 cm. In the subgroups of stage I (P = 0.04) and stage II (P < 0.001)—but not stage III—patients, GTR and partial resection (PR) were associated with longer OS. We constructed several multivariable models including different variables, and greater extent of resection, smaller tumor size, and MGMT methylation consistently emerged as independent markers of longer OS. This staging system provides a feasible tool for comparison of results. We confirmed the value of partial resection in stage I and II patients, in contrast to some reports suggesting that biopsy only is sufficient when gross total resection cannot be safely achieved. [ABSTRACT FROM AUTHOR]

Purpose: Blood-brain barrier breakdown (BBBB) occurs in relapsing remitting multiple sclerosis (RRMS). Relative recirculation (rR), a BBBB surrogate, may show inflammation undetectable by gadolinium. We compared normal appearing white matter (NAWM) rR in patients with and without disability measured with Symbol Digit Modalities Test and the Expanded Disability Status Scale (EDSS). Methods: Thirty-nine RRMS patients were prospectively recruited and classified as impaired or non-impaired based on the SDMT and EDSS threshold ≥3. Significant demographic, MRI structural and regional rR characteristics were advanced into multivariate analysis to assess the association with impairment of cognition and EDSS. Bonferroni corrected p < 0.025 was applied to demographic and rR group comparisons; p < 0.05 was used in the final multivariate logistic regression. Results: rR was higher in NAWM ( p = 0.012), NAGM ( p = 0.004), and basal ganglia ( p = 0.007) in cognitively impaired versus non-impaired patients. The difference between NAWM and T2HL rR was significant in cognitively non-impaired patients and approximated that of T2HL in impairment (0.084 vs. 0.075, p = 0.008; 0.118 vs. 0.101, p = 0.091, respectively). After adjusting for confounders, rR elevation for NAWM (OR 1.777; 95% CI 1.068-2.956; p = 0.026), NAGM (OR 2.138; 1.100-4.157; p = 0.025), and basal ganglia (OR 2.192; 1.120-4.289; p = 0.022) remained significantly predictive of cognitive impairment. NAWM area under the curve (AUC) for cognitive impairment was 0.783. No significant group differences or associations were seen for rR and EDSS impairment. No NAGM and cortical lesion rR difference was present within any of the impaired or non-impaired groups. Conclusion: rR elevation in NAWM, NAGM, and basal ganglia appears sensitive to cognitive impairment but not EDSS. [ABSTRACT FROM AUTHOR]

Choroid plexus tumors (CPT) are rare neoplasms accounting for 1-4% of all pediatric brain tumors. They are divided into choroid plexus papilloma (CPP), atypical choroid plexus papilloma (APP) and choroid plexus carcinoma (CPC). CPTs are known to primarily affect children less than 2 years of age. Gross total resection is the most important predictor of survival especially in CPC. Although small case series have been published, limited clinical data are available to describe treatment and outcome of CPTs. More clinical data would be necessary to complete the picture, particularly in populations that are not age limited. Here we share data from the two major hospitals in Cleveland to describe treatment and outcome of adult and pediatric patients. We performed a retrospective analysis of patients with CPT seen in Cleveland Clinic from 1990 to 2015 and at University Hospitals from 1994 to 2015. Results were compared to previously published historical controls. We identified 30 cases with CPT, including 22 pediatric and eight adult cases; 11 females and 19 males. The mean age at presentation was 12.4 years with a median age of 4.5 years (range 2 months-51 years). Gross total surgical resection was achieved in 22, subtotal resection in four, partial resection in two and unknown in two. The histology was CPP in 23 patients, two of whom developed recurrence requiring repeat resection and adjuvant therapy. Median event free survival (EFS) for CPP patients was 7.6 years. The histology was CPC in seven patients. All CPC patients were treated with adjuvant therapy. Median EFS of CPC patients was 4.4 years. Overall survival of all CPT patients was 100% with a median follow up of 7 years. A systematic literature review identified 1012 CPT patients treated from 1989 to 2013. The mean and median age of CPT patients was 13 and 3 years respectively. The median survival of 541 CPP patients was undefined vs. 2.7 years for the 452 CPC patients. The difference between the two populations was highly significant (p < 0.001). Kaplan-Meier survival curves comparing CPTs at Cleveland Clinic and University Hospitals versus a systematic literature review showed a statistically significant advancement in overall survival among the patients treated at Cleveland Clinic and University Hospitals. Our data are consistent with the literature review regarding epidemiology, clinical presentation, and treatment modalities but differed in regards to survival. Differences in survival may be related to different methods of data collection or details in patient care. [ABSTRACT FROM AUTHOR]

Object: Endoscopic third ventriculostomy (ETV) uses anatomical spaces of the ventricular system to reach the third ventricle floor and create an alternative pathway for cerebrospinal fluid flow. Optimal ETV trajectories have been previously proposed in the literature, designed to grant access to the third ventricle floor without a displacement of eloquent periventricular structures. However, in hydrocephalus, there is a significant variability to the configuration of the ventricular system, implying that the optimal ETV trajectory and cranial entry point needs to be planned on a case-by-case basis. In the current study, we created a mathematical model, which tailors the optimal ETV entry point to the individual case by incorporating the ventricle dimensions. Methods: We retrospectively reviewed the imaging of 30 consecutive pediatric patients with varying degrees of ventriculomegaly. Three dimensional radioanatomical models were created using preoperative MRI scans to simulate the optimal ETV trajectory and entry point for each case. The surface location of cranial entry points for individual ETV trajectories was recorded as Cartesian coordinates centered at Bregma. The distance from the Bregma in the coronal plane represented as 'x', and the distance from the coronal suture in the sagittal plane represented as 'y'. The correlation between the ventricle dimensions and the x, y coordinates were tested using linear regression models. Results: The distance of the optimal ETV entry point from the Bregma in the coronal plane ('x') and from the coronal suture in the sagittal plane ('y') correlated well with the frontal horn ratio (FHR). The coordinates for x and y were fitted along the following linear equations: x = 85.8 FHR−13.3 ( r = 0.84, p < 0.001) and y = −69.6 FHR + 16.7 ( r = 0.83, p < 0.001). Conclusion: The surface location of the optimal cranial ETV entry point correlates well with the ventricle size. We provide the first model that can be used as a surgical planning aid for a case specific ETV entry site with the incorporation of the ventricle size. [ABSTRACT FROM AUTHOR]

Background: Choroid plexus papilloma is a rare intracranial neoplasm derived from choroid plexus epithelium accounting for less than 1 % of all brain tumors. These tumors are highly vascularized, and tumor resection is difficult, especially in small children, due to severe intraoperative bleeding. Preoperative embolization is helpful to reduce intraoperative bleeding, but it may not be possible in small children or if the tumor has no suitable feeding vessels for embolization. Methods: We present the case of a 2-year-old girl with a giant choroid plexus papilloma. An attempt of tumor resection was previously performed in another clinic, but the surgery was aborted due to massive intraoperative bleeding and only a biopsy was done. Angiography showed no suitable vessels for embolization. A new attempt of tumor removal was carried out, but again, severe intraoperative bleeding occurred and only a partial resection was possible. Intratumoral embolization with onyx through direct percutaneous puncture was performed. Results: Radical tumor removal was possible after two additional surgeries. Intraoperative blood loss was 345 ml (first surgery = only partial removal), 250 ml (second procedure = 1/3 of tumor volume resected), and 250 ml (third surgery = total removal). The patient presented no additional deficits. Conclusions: Intratumoral percutaneous embolization with onyx was very helpful in reducing intraoperative bleeding in this case. Safe radical tumor resection was possible. This technique may be useful for those surgeons dealing with highly vascularized tumors, especially in small children. No report of intratumoral embolization with onyx in cases of intracerebral tumors could be found in the literature. [ABSTRACT FROM AUTHOR]

Aim: The purpose of this case is to highlight the benefits of preoperative embolization as well as to review the vascular anatomy that needs to be recognized in order to perform pre-operative embolization of choroid plexus tumors. Method: We achieve this by presenting the case of a 12-month-old female who had symptoms of raised intracranial pressure. MRI demonstrated a large vividly enhancing mass centered within the atria of the right lateral ventricle associated with hydrocephalus in keeping with a choroid plexus tumor. Result: Enlarged anterior and posterior choroidal arteries supplying the tumor were noted on the MRI scan. Pre-operative embolization was performed with NBCA glue via the anterior and posterior choroidal arteries. Subsequently, total surgical resection was achieved with only 200 cc of blood loss. Pathology confirmed a choroid plexus carcinoma. Conclusion: Pre-operative embolization can be useful in minimizing blood loss during excision of choroid plexus tumors. It is important to understand the anatomy of the anterior and posterior choroidal arteries to perform embolization of these tumors safely. [ABSTRACT FROM AUTHOR]

Introduction: Management of posterior fossa tumors in infants and neonates is challenging. The characteristics of the young babies make surgery very difficult, sometimes precluding a safe complete removal. Methods: A review of the literature was undertaken to examine the incidence, histology, surgical aspects, and prognosis of posterior fossa tumors in the first year of life. Therapeutical strategies of the most frequent tumor types are also discussed in detail. Results: Histology is dominated by tumors with aggressive behavior, such as medulloblastomas, atypical teratoid/rhabdoid tumors, and anaplastic ependymomas. The most important surgical considerations in small children are the small circulating blood volume; the poor thermoregulation; and incomplete maturation of the brain, of the skull, and of the soft tissue. Treatment toxicity is inversely related to the age of the patients. Radiation therapy is usually considered as contraindicated in young children, with few exceptions. Proton therapy is a promising tool, but access to this kind of treatment is still limited. The therapeutic limitations of irradiation render resection of this tumor and adjuvant chemotherapy often the only therapeutic strategy in many cases. Conclusions: The overall prognosis remains dismal because of the prevalent aggressive histologies, the surgical challenges, and the limitations of adjuvant treatment. Nevertheless, the impressive improvements in anesthesiology and surgical techniques allow, in the vast majority of the cases, complete removal of the lesions with minor sequelae in high-volume referral pediatric centers. [ABSTRACT FROM AUTHOR]

Introduction: We performed a retrospective study on clinical assessment, tumor location, radiological imaging, histopathological characteristics, and therapeutic management of 7 patients affected by choroid plexus carcinoma (CPC) or atypical choroid plexus papilloma (ACPP) who have been observed in the last 12 years. Methods: Four patients fulfilled the criteria for classification as ACPP and three cases as CPC. The median age of the patients at the diagnosis was 42 months (range 3-190 months). Except one older patient (15 years old), all patients were younger than 3 years of age. In all patients affected by ACPP, a total surgical resection was achieved. Two children relapsed 12 and 8 months following radical removal. Both of them underwent adjuvant chemotherapy (carboplatin, cyclophosphamide, etoposide, doxorubicin, and methotrexate); a complete remission was maintained in all cases. In all three patients with CPC, it was impossible to achieve complete resection at first surgery. The response to chemotherapy was variable: in one case, it was complete with complete remission following 6 months; in one case, it was partial with reduction on volume (the patient underwent second-look surgery with complete resection); in the third case, there was no response and the patient progressed and finally died with metastatic disease, 8 months after chemotherapy was started. For children with CPC, the OS was 75 % at 6 years. Results: In our series, surgery associated with chemotherapy led to long-term survival in 4/4 patients affected by ACPP and 2/3 patients affected by CPC. Clinical results achieved in our series confirm that our therapeutic regimen is feasible and efficient as a possible adjuvant treatment for both CPC and ACPP. It also suggests that surgery has a pivotal role in the management of most children affected by CPTs. [ABSTRACT FROM AUTHOR]

The current optimal surgery for glioblastomas (GBMs) near the pyramidal tract (PT) is to remove as much tumor as possible and to preserve motor function. The purpose of this study is to investigate the usefulness of tractography-integrated navigation-guided fence-post catheter techniques and motor-evoked potentials (MEPs) for preserving postoperative motor function after GBM surgery. We retrospectively examined 49 patients who underwent resection for GBM near the PT. Diffusion tensor (DT) imaging-based tractography of the PT was performed preoperatively and integrated into the navigation system. When possible, silicon catheters were used as 'fence-posts' and were inserted along the tumor boundaries, avoiding the PT, before tumor removal using the navigation system (fence-post catheter techniques). Cortical and subcortical MEPs were also monitored during resection of the tumor. Fence-post catheter techniques using a tractography-integrated navigation system were used in 45 of 49 patients. This technique enabled placement of the catheters, avoided the motor pathways, and allowed easier resection of the tumors. Tumors near the PT were resected using subcortical and cortical MEPs. The amplitudes of cortical MEPs after tumor removal were maintained at over 33 % of those obtained before resection. Thirty-six patients showed obvious responses of subcortical MEPs at ≤20 mA. The degree of resection was gross total in 21 patients, subtotal in 21, and partial in seven. One month after surgery, only one patient showed worsened motor function. Therefore, fence-post catheter techniques using a tractography-integrated navigation system and MEPs may contribute to preserving motor function after surgery for GBMs that are near the PT. [ABSTRACT FROM AUTHOR]

Introduction: Although meningiomas are frequently diagnosed in adults, it is a rare (intracranial) tumor in the pediatric population, with an incidence of 0.06/100,000. The pathology and treatment of meningiomas in adulthood has been a topic of increasing investigation. So far, the treatment of pediatric meningiomas has been extrapolated from these results. The question remains, however, whether translation of adult meningioma data into the childhood population is legitimate. Methods: We present the case of a 3-year-old girl diagnosed with an intraventricular malignant meningioma and type 2 neurofibromatosis. She was operated on multiple times to achieve complete resection and received adjuvant chemotherapy. Since, she has been stable with no neurological sequelae and/or recurrence of the meningioma. Conclusion: Pediatric meningiomas are rare tumors and differ from their adult counterparts in various aspects. We believe that gross total resection of meningioma in the pediatric population, when possible, is the treatment of choice. In the event of a subtotal resection, repeat resection is recommended. Any adjuvant treatment with chemotherapy or radiation therapy should be carefully considered during multidisciplinary meetings. [ABSTRACT FROM AUTHOR]

Choroid plexus tumors (CPTs) are rare neoplasms of the central nervous system whose optimal management is not well defined. The Surveillance Epidemiology and End Results (SEER) Database from 1978 to 2009 was queried to define population-based outcomes for all patients with CPTs. Patient demographic data, histological classification (choroid plexus papilloma [CPP], atypical CPP [aCPP], and choroid plexus carcinoma [CPC]), extent of surgery, and use of radiation therapy (RT) as part of an initial course of therapy were analyzed for impact on overall survival (OS) and cause-specific survival (CSS). Chemotherapy data were not available within the SEER database. A total of 349 patients with CPTs were identified (120 CPCs, 26 aCPPs, and 203 CPPs). Patients with CPC presented at a younger age (median 3, mean 14.8 years) relative to CPP (median 25, mean 28.4 years; p < 0.0001). Histology was a significant predictor of OS, with 5-year OS rates of 90, 77, and 58 % for CPP, aCPP, and CPC, respectively. Older age and male sex were prognostic for worse OS and CSS for CPP. Only extent of surgery had a significant impact on survival for CPC. The use of adjuvant RT in patients with CPC undergoing surgery was not associated with a significantly improved OS ( p = 0.17). For patients undergoing GTR without RT as part of an initial course of therapy, estimated 5- and 10-year OS were 70 % (±7 %) and 67 % (±8 %), respectively. Prospective data are required to define the optimal combination of surgery with adjuvant therapies, including chemotherapy. [ABSTRACT FROM AUTHOR]

Introduction: In diffuse intrinsic pontine gliomas (DIPG), subtracting pre-contrast from post-contrast T1-weighted images (T1WI) occasionally reveals subtle, 'occult' enhancement. We hypothesized that this represents intravascular enhancement related to angiogenesis and hence that these tumors should have greater blood volume fractions than do non-enhancing tumors. Methods: We retrospectively screened MR images of 66 patients initially diagnosed with DIPG and analyzed pretreatment conventional and dynamic susceptibility contrast (DSC) perfusion MRI studies of 61 patients. To determine the incidence of occult enhancement, cerebral blood volume (CBV) values were compared in areas of occult enhancement (OcE), no enhancement (NE), and normal-appearing deep cerebellar white matter (DCWM). Results: Tumors of 10 patients (16.4 %) had occult enhancement; those of 6 patients (9.8 %) had no enhancement at all. The average CBV in areas of occult enhancement was significantly higher than that in non-enhancing areas of the same tumor ( P = .03), within DCWM in the same patient ( P = .03), and when compared to anatomically paired/similar regions of interest (ROI) in patients with non-enhancing tumors ( P = .005). Conclusion: Areas of OcE correspond to areas of higher CBV in DIPG, which may be an MRI marker for angiogenesis, but larger scale studies may be needed to determine its potential relevance to grading by imaging, treatment stratification, biopsy guidance, and evaluation of response to targeted therapy. [ABSTRACT FROM AUTHOR]

Choroid plexus carcinoma (CPC) is a World Health Organization (WHO) grade III brain tumor with a poor prognosis that occurs mainly in children. Gross total resection of CPC is highly recommended and is associated with improved overall survival, although it is often associated with increased morbidity. The use of adjuvant therapies has yet to be standardized, although evidence suggests that for patients with incompletely resected CPCs, a combination of chemotherapy and radiation therapy may be beneficial. The use of radiation therapy for younger children (<3 years old) with CPC, however, is not recommended, due to the potential negative neurological sequelae associated with radiation to the developing brain. Given that the majority of CPC patients are young children, questions regarding optimal radiation dose, chemotherapy agents, and how to combine these two adjuvant treatment modalities to achieve the best outcomes remain unanswered. In this paper we summarize the current management of CPC in the literature. Further studies are needed to standardize the treatment paradigm for this malignant brain tumor. [ABSTRACT FROM AUTHOR]

Background: Choroid plexus tumor is a rare brain tumor with variable clinical features according to the histological grade. We reviewed the treatment outcome of 23 children, focusing on the biological behavior of the atypical choroid plexus papilloma (ACPP) and the current therapeutic strategy in choroid plexus carcinoma (CPC). Methods: The demographics, clinical features, surgical treatments, adjuvant therapies, and survival were reviewed. Results: The median age at diagnosis was 18 months-55 months for choroid plexus papilloma (CPP), 8 months for ACPP, and 15 months for CPC. Gross total resections were achieved in seven of eight patients with CPP, seven of seven with ACPP, and three of eight with CPC. Seven patients with CPC received chemotherapy. Four patients received high-dose chemotherapy (HDCT) and autologous peripheral blood stem cell transplantation (aPBSCT), and three among them have survived. Four patients with CPC received radiotherapy. One CPP patient and one CPC patient underwent radiosurgery. All CPP and ACPP patients have survived. The overall survival rate of the CPC patients was 62.5 % in the first year and 42.9 % in the second year. The progression-free survival rate of the CPC patients was 50 % in the first year and 0 % in the second year. Seven patients underwent permanent cerebrospinal fluid diversion surgery because of hydrocephalus or subdural effusion. Conclusion: CPP and ACPP were surgically curable. Multi-modal treatments are necessary in the management of CPC with poor prognosis. HDCT and aPBSCT may be important to treat infants for whom radiotherapy is limited. Hydrocephalus and subdural effusion should be resolved with appropriate management. [ABSTRACT FROM AUTHOR]

Choroid plexus carcinomas are malignant brain tumors predominantly arising in young children. Because a prognostic role of p53 alterations has been demonstrated, further research into potential underlying mechanisms is essential. Our objective was, therefore, to investigate the role of p53 in the growth-inhibitory potential of a variety of anticancer agents in the rodent choroid plexus epithelial cell line Z310. Furthermore, association of p53 alterations with proliferative activity (Ki67/MIB1) in choroid plexus carcinoma samples ( N = 20) was examined by use of immunohistochemistry. Silencing of TP53 expression did not significantly alter metabolic activity in Z310 cells and p53 protein expression status was not associated with increased proliferative activity in choroid plexus carcinomas. However, the growth-inhibitory activity of vincristine, doxorubicin, carboplatin, etoposide, and temozolomide was significantly impaired by silencing of TP53. In conclusion, these results indicate a potential predictive role of p53 in choroid plexus carcinomas. Alterations of p53 should be taken into account when evaluating the effect of anticancer agents in future clinical trials. [ABSTRACT FROM AUTHOR]

Recurrent diffuse intrinsic pontine gliomas (DIPG) are traditionally treated with palliative care since no effective treatments have been described for these tumors. Recently, clinical studies have been emerging, and individualized treatment is attempted more frequently. However, an informative way to compare the treatment outcomes has not been established, and historical control data are missing for recurrent disease. We conducted a retrospective chart review of patients with recurrent DIPG treated between 1998 and 2010. Response progression-free survival and possible influencing factors were evaluated. Thirty-one patients were identified who were treated in 61 treatment attempts using 26 treatment elements in 31 different regimens. The most frequently used drugs were etoposide (14), bevacizumab (13), irinotecan (13), nimotuzumab (13), and valproic acid (13). Seven patients had repeat radiation therapy to the primary tumor. Response was recorded after 58 treatment attempts and was comprised of 0 treatment attempts with complete responses, 7 with partial responses, 20 with stable diseases, and 31 with progressive diseases The median progression-free survival after treatment start was 0.16 years (2 months) and was found to be correlated to the prior time to progression but not to the number of previous treatment attempts. Repeat radiation resulted in the highest response rates (4/7), and the longest progression-free survival. These data provide a basis to plan future clinical trials for recurrent DIPG. Repeat radiation therapy should be tested in a prospective clinical study. [ABSTRACT FROM AUTHOR]

Objectives: T2' values reflect the presence of deoxyhaemoglobin related to high local oxygen extraction. We assessed the feasibility of T2' imaging to display regions with high metabolic activity in brain gliomas.Methods: MRI was performed in 25 patients (12 female; median age 46 years; range 2-69) with brain gliomas with additional T2 and T2* sequences. T2' maps were derived from T2 and T2*. Dynamic susceptibility weighted contrast (DSC) perfusion was performed in 12/25 patients. Images were visually assessed by two readers and five ROIs were evaluated for each patient. Pearson correlation, Mann-Whitney and Kruskal-Wallis tests were applied for statistical analysis.Results: Three patients were not further evaluated because of artefacts. Mean values of high-grade (III-IV) gliomas showed significantly lower T2' values than low-grade (II) gliomas (p < 0.001). An inverse relationship was observed between rCBV and sqr (T2') (r = -0.463, p < 0.001). No correlation was observed between T2' and rCBV for grade II tumours (r = 0.038; p = 0.875).Conclusions: High-grade tumours revealed lower T2' values, presumably because of higher oxygen consumption in proliferating tissue. Our results indicate that T2' imaging can be used as an alternative to DSC perfusion in the detection of subtle deviations in tumour metabolism. [ABSTRACT FROM AUTHOR]

Background: Choroid plexus tumors (CPT) are rare pediatric tumors. A population-based study on choroid plexus carcinoma (CPC) and choroid plexus papilloma (CPP) was carried out to describe the incidence, demographic, and outcome data and to identify potential prognostic factors. Methods: The CPT population from the Canadian databank of CNS tumor in children ≤36 months diagnosed between 1990 and 2005 was reviewed Results: Out of the 579 reported cases of CNS tumors, 37 were CPT. The annual age-adjusted incidence rate was 0.22 + 0.12 (95% CI 0.16-0.28)/100,000 children <3 years. There were 21 (56.7%) CPP and 16 (43.3.5%) CPC. Twenty patients (54%) were males. Median age at diagnosis was 7 months(range 0-30). Ten patients(62.5%) with CPC and one with CPP were metastatic at diagnosis. Twenty patients with CPP (95%) had a complete resection, whereas 6/16 CPC (37.5%) achieved a resection >90%. Fourteen CPC patients received adjuvant chemotherapy. None of the 37 patients received adjuvant radiation. At completion of survey, all CPP and five CPC were alive. Median survival time for CPC patients was 15 months (0-120). One death was related to intraoperative hemorrhage, another to chemotherapy-induced toxicity, and one to secondary AML. Age at diagnosis, degree of resection and metastatic status were not significant prognostic factors for CPC. Conclusion: By contrast to CPC, CPP have an excellent prognosis following surgery alone. Survival of CPC remains poor. However, these data may suggest adjuvant chemotherapy can alter the aggressive natural history of CPC. As with other rare CNS tumors, international collaboration is required to identify optimal therapy. [ABSTRACT FROM AUTHOR]

The concept of organ donation for research purposes has evolved to overcome limited availability of tissue and foster research, in particular in diseases for which surgery is not routinely performed. Diffuse intrinsic pontine glioma (DIPG) is an almost invariably fatal childhood tumour, not amenable to surgery because of its location and infiltrative nature. We report our institutional experience of post mortem brain or tumour donation and its impact on the families. Since July 2002, we have requested permission for post mortem brain examination for research purposes, from parents whose children succumbed to DIPG. Brain donation was discussed with 21 of 27 families of patients diagnosed with DIPG between July 2002 and June 2008 at the Hospital for Sick Children. Ten (47.6%) agreed, and autopsy was performed in 9 cases. Eight of these children died at home, one in the hospital. Autopsy was performed within 48 h from death in all cases except one. Pathological diagnosis was PNET in two and glioblastoma multiforme in seven patients. A disclosure of autopsy results was provided to all parents by the neuro-oncology team. None of the families expressed distress from the autopsy, or regretted the choice made. Autopsy limited to the brain did not interfere with the choices related to palliation. The families derived comfort from the hope that scientific breakthroughs could be made and felt that they were helping to make a difference in the future management of DIPG. [ABSTRACT FROM AUTHOR]

Objectives: To examine the feasibility of deriving quantitative microcirculatory parameters and to investigate the relationship between vascular and metabolic characteristics of head and neck tumours in vivo, using dynamic contrast-enhanced (DCE) MRI and fluorodeoxyglucose (FDG) PET imaging.Methods: Twenty-seven patients with primary squamous cell carcinoma (SCCA) underwent DCE-MRI and combined PET/CT imaging. DCE-MRI data were post-processed by using commercially available software. Transfer constant (K (trans)), extravascular extracellular blood volume (v (e)), transfer constant from the extracellular extravascular space to plasma (k (ep)) and iAUC (initial area under the signal intensity-time curve) were calculated. 3D static PET data were acquired and standardised uptake values (SUV) calculated.Results: All microcirculatory parameters in tumours were higher than in normal muscle tissue (P ≤ 0.0019). Significant correlations were shown between k (ep) and K (trans) (ρ = 0.77), v (e) and k (ep) (ρ = -0.7), and iAUC and v (e) (ρ = 0.53). Significant correlations were observed for SUV(mean) and v (e) as well as iAUC (ρ = 0.42 and ρ = 0.66, respectively). SUV(max) was significantly correlated with iAUC (ρ = 0.69).Conclusions: The demonstrated relationships between vascular and metabolic characteristics of primary SCCA imply a complex interaction between vascular delivery characteristics and tumour metabolism. The lack of correlation between SUV and K (trans)/k (ep) suggests that both diagnostic techniques may provide complementary information. [ABSTRACT FROM AUTHOR]

Background: The development of a quantifiable and noninvasive method of monitoring disease activity and response to therapy is vital for arthritis management.Objective: The purpose of this study was to investigate the utility of quantitative dynamic contrast-enhanced MRI (DCE-MRI) based on pharmacokinetic (PK) modeling to evaluate disease activity in the knee and correlate the results with the clinical assessment in children with juvenile idiopathic arthritis (JIA).Materials and Methods: A group of 17 children with JIA underwent longitudinal clinical and laboratory assessment and DCE-MRI of the knee at enrollment, 3 months, and 12 months. A PK model was employed using MRI signal enhancement data to give three parameters, K(trans') (min(-1)), k(ep) (min(-1)), and V(p) (') and to calculate synovial volume.Results: The PK parameters, synovial volumes, and clinical and laboratory assessments in most children were significantly decreased (P < 0.05) at 12 months when compared to the enrollment values. There was excellent correlation between the PK and synovial volume and the clinical and laboratory assessments. Differences in MR and clinical parameter values in individual subjects illustrate persistent synovitis when in clinical remission.Conclusion: A decrease in PK parameter values obtained from DCE-MRI in children with JIA likely reflects diminution of disease activity. This technique may be used as an objective follow-up measure of therapeutic efficacy in patients with JIA. MR imaging can detect persistent synovitis in patients considered to be in clinical remission. [ABSTRACT FROM AUTHOR]

Summary  Cerebrospinal metastases of benign choroid plexus papillomas (CPPs) are extremely rare. We report a case of 32-year-old woman who presented with a 6-month history of vertigo and intermittent headache. Plain CT scan of the cranium revealed a partly calcified tumor filling the fourth ventricle and its right recess. Cranial MRI showed an inhomogeneously contrast-enhancing tumor and leptomeningeal enhancement encasing the brain stem. Complete resection of the tumor was carried out, and seedings to the floor of the fourth ventricle and cervico-medullary junction were found during the operation. While intraoperative frozen section suggested pathology of papillary ependymoma or CPP, to our surprise, final histological examination revealed a benign choroid plexus papilloma. One month after the first operation, a metastatic nodule was found in the spinal subarachnoidal space at the level of T8-9. Two months after the first operation, on follow-up MRI of the cranium, the leptomeningeal enhancement encasing the brain stem had resolved spontaneously. This special case helps increase our understanding of benign CPPs and expands our differential diagnostic consideration of lesions with similar manifestations. [ABSTRACT FROM AUTHOR]

Recent advances in molecular biology have enhanced our understanding of the pathogenesis of brain tumors, particularly in children. The use of molecular diagnostic tools is quickly becoming a standard component in the diagnosis and classification of brain tumors in children, in addition to providing insight leading to treatment stratification and improved outcome prediction. All new protocols involving treatments for brain tumors in children include studies of biomarkers and biologic correlates as a means to identify new targets for therapeutics and possible intervention strategies. [ABSTRACT FROM AUTHOR]

Presents the scientific sessions of the European Society of Neuroradiology during its 30th Congresses and 14th Advanced Course in Barcelona, Spain. Schedule of the event; Topics discussed; Lists of lecturers.

Vascular and angiogenic processes provide an important target for novel cancer therapeutics. Dynamic contrast-enhanced magnetic resonance imaging is being used increasingly to noninvasively monitor the action of these therapeutics in early-stage clinical trials. This publication reports the outcome of a workshop that considered the methodology and design of magnetic resonance studies, recommending how this new tool might best be used.British Journal of Cancer (2005) 92, 1599-1610. doi:10.1038/sj.bjc.6602550 www.bjcancer.com [ABSTRACT FROM AUTHOR]

Magnetic resonance imaging (MRI) provides a range of non-invasive measures for visualization of tumor angiogenesis in the clinic as well as in experimental tumor models. MRI methods were developed for assessment of spatial and temporal changes in perfusion, blood volume fraction, vascular permeability, vascular function, vascular maturation, vessel diameter and tortuosity. Molecular targeted contrast agents were used for mapping specific markers of neovasculature. These approaches were applied for analysis of a number of regulatory mechanisms controlling tumor angiogenesis and for preclinical evaluation of tumor response to antiangiogenic agents. [ABSTRACT FROM AUTHOR]