Assessment of histogram analysis in distinguishing between low-grade and high-grade brainstem glioma.

Objectives: This retrospective study was designed to evaluate the value of histogram analysis on the apparent diffusion coefficient (ADC) map in distinguishing between low-grade and high-grade brainstem glioma (BSG). In this article, we used the two VOI (volume of interest) placements of the entire tumour method and the entire solid part method, thus aiming to compare the diagnostic value between these two performances. Methods: A total of 28 patients (8 low-grade BSGs and 20 high-grade BSGs) with histological diagnosis of BSG. All victims underwent contrast-enhanced magnetic resonance imaging (MRI). We measured ADC histogram parameters (mean, median, SD, max, min, Kurtosis, Skewness, Entropy, Uniformity, and Variance) and calculated the ratios between tumour and normal brain parenchyma parameters in two methods. Independent samples test, Mann-Whitney U test, and ROC curve were used to determine each value's cut-off point, sensitivity, and specificity. Results: Among the method of VOI placing the entire tumour, the values of ADC_min, rADC_mean, rADC_median, and rADC_min are significantly different between these two neoplasms with cut-off values (sensitivity %, specificity %) of 776 x10-6 m2/s (62.5%, 90%), 2.1765 (62.5%, 95%), 2.1588 (50%, 100%), 1.0535 (100%, 50%), respectively. On the other hand, the method of VOI placing the entire solid part of the tumour showed significantly different in ADC_mean, ADC_median, ADC_min, rADC_mean, rADC_median, rADC_min at the cut-off values (sensitivity%, specificity %) of 1491 x10-6 m2/s (62.5%, 95%), 1438.9 x10-6 m2/s (62.5%, 90%), 862.5 x10-6 m2/s (75%, 100%), 2,112 (62.5%, 95%), 1.9748 (62.5%, 90%), 1.3735 (87.5%, 90%), respectively. Conclusions: The ADC histogram analysis is a promising approach to distinguishing low-grade BSG and high-grade BSG. The entire solid part VOI placement has a superior value compared to the whole tumour VOI placement. The rADC_mean showed the best performance in differentiating between these two entities, followed by ADC_min, rADC_mean, rADC_median, ADC_mean, and ADC_median.

A phase I clinical trial of sonodynamic therapy combined with radiotherapy for brainstem gliomas.

Brainstem gliomas (BSGs) are a class of clinically refractory malignant tumors for which there is no uniform and effective treatment protocol. Ultrasound and radiation can activate hematoporphyrin and produce sonodynamic and radiodynamic effects to kill cancer cells. Therefore, we conducted the first phase I clinical trial of sonodynamic therapy (SDT) combined with radiotherapy (RT) for the treatment of BSGs to verify its safety and efficacy. We conducted a study of SDT combined with RT in 11 patients with BSGs who received SDT and RT after hematoporphyrin administration. Magnetic resonance imaging was performed during this period to assess the tumor, and adverse events were recorded. All adverse events recorded were grade 1-2; no grade 3 or more serious adverse events were observed. Treatment was well tolerated, and no dose-limiting toxicities were observed. There were no treatment-related deaths during the course of treatment. 8 of 11 patients (72.7%) maintained stable disease, 2 (18.2%) achieved partial response, and the tumors were still shrinking as of the last follow-up date. The median progression-free survival (PFS) for patients was 9.2 (95% confidence interval [CI] 6.2-12.2) months, and the median overall survival (OS) was 11.7 (95% CI 9.6-13.8) months. Therefore, SDT combined with RT has a favorable safety and feasibility and shows a preliminary high therapeutic potential.

AB091. Establishment and application of brainstem glioma organoids.

BACKGROUND: Traditional preclinical experiments on brainstem gliomas mainly rely on patient-derived primary cell lines, but there are problems such as low success rate in establishment and inability to preserve tumor heterogeneity, which limit the clinical transformation. As a new type of in vitro tumor model, organoids have similar structure and function to the original tumor, requiring less tissue for cultivation, with short cycle and high success rate, which is particularly suitable for brainstem glioma biopsy. There is currently no precedent for the successful construction of brainstem glioma organoid models. This new established organoid provides us a more robust preclinical tool for comprehending the pathogenesis and conducting drug screening for this kind of disease. METHODS: Cultivate patient-derived brainstem glioma organoids in vitro, verify the genetic fidelity and consistency of the organoids through morphological experiments as well as sequencing technology. Then explore the evolutionary direction of multiple types of brainstem gliomas through pseudo-time series analysis. Complete drug screening, natural killer (NK) cell co-culture, oncolytic virus therapy, and other treatments based on organoids in vitro, and evaluate the efficacy. Complete co-culture of organoids and Institute of Cancer Research (ICR) mouse brain slices in vitro. Establish patient-derived organoid xenograft (PDOX) mouse models derived from organoids in vivo. RESULTS: The establishment of organoids of all types of brainstem gliomas was completed for the first time in the world, with a total of 41/48 organoid models derived from patients, with a success rate of 85.4%, covering all segments and pathological types. The results of morphological experiments and sequencing showed that the genetic characteristics of organoids were highly consistent with those of tumor tissues. Drug screening tests for temozolomide and panobinostat were completed in vitro, and NK cell co-culture and oncolytic virus therapy testing were achieved. Co-culture of brainstem glioma organoids and mouse brain slices was achieved in vitro. Furthermore, a PDOX model of brainstem glioma was established. CONCLUSIONS: Brainstem glioma organoids can be established maturely, stably, and reliably, and can be used for preclinical drug testing for patients. Animal models derived from brainstem glioma organoids have broad preclinical experimental value.

Medulloblastoma in a 13-Year-Old Female: A Comprehensive Case Report.

The majority of children's brain cancers are posterior fossa tumours, which include brainstem gliomas, medulloblastomas (MBs), juvenile pilocytic astrocytomas, and ependymomas. This report details a 13-year-old female presenting with headache, nausea, and ataxia. With typical magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) results, the MRI indicated a solid lesion in the fourth ventricle, producing obstructive hydrocephalus. Pilocytic astrocytoma, ependymoma, MB, and other conditions are examples of differential diagnoses. In addition to underscoring the need for early intervention to enhance prognosis and outcomes for paediatric patients with posterior fossa tumours, the case highlights the vital role that sophisticated imaging plays in early detection and therapy.

A multi-task generative model for simultaneous post-contrast MR image synthesis and brainstem glioma segmentation.

OBJECTIVES: This study aims to generate post-contrast MR images reducing the exposure of gadolinium-based contrast agents (GBCAs) for brainstem glioma (BSG) detection, simultaneously delineating the BSG lesion, and providing high-resolution contrast information. METHODS: A retrospective cohort of 30 patients diagnosed with brainstem glioma was included. Multi-contrast images, including pre-contrast T1 weighted (pre-T1w), T2 weighted (T2w), arterial spin labeling (ASL) and post-contrast T1w images, were collected. A multi-task generative model was developed to synthesize post-contrast T1w images and simultaneously segment BSG masks from the multi-contrast inputs. Performance evaluation was conducted using peak signal-to-noise ratio (PSNR), structural similarity index (SSIM), and mean absolute error (MAE) metrics. A perceptual study was also undertaken to assess diagnostic quality. RESULTS: The proposed model achieved SSIM of 0.86 ± 0.04, PSNR of 26.33 ± 0.05 and MAE of 57.20 ± 20.50 for post-contrast T1w image synthesis. Automated delineation of the BSG lesions achieved Dice similarity coefficient (DSC) score of 0.88 ± 0.27. CONCLUSIONS: The proposed model can synthesize high-quality post-contrast T1w images and accurately segment the BSG region, yielding satisfactory DSC scores. CLINICAL RELEVANCE STATEMENT: The synthesized post-contrast MR image presented in this study has the potential to reduce the usage of gadolinium-based contrast agents, which may pose risks to patients. Moreover, the automated segmentation method proposed in this paper aids radiologists in accurately identifying the brainstem glioma lesion, facilitating the diagnostic process.

The effectiveness of diffusion kurtosis imaging metrics for distinguishing between brainstem glioma and cerebellar medulloblastoma.

Background: In some clinical situations, distinguishing between cerebellar medulloblastoma and brainstem glioma is important. We assessed whether diffusion kurtosis imaging (DKI) metrics could be used to distinguish cerebellar medulloblastomas from brainstem gliomas in children. Patients and methods: This prospective study was approved by the institutional review board. Seventy patients were separated into two groups according to eventual diagnosis: brainstem glioma (n = 30) and cerebellar medulloblastoma (n = 40). Both groups underwent brain magnetic resonance imaging (MRI), including DKI. The Kurtosis value for the tumor region and the ratio between Kurtosis values between the tumor and the normal parenchyma (rKurtosis) were compared between groups using the Mann-Whitney U test. Receiver operating characteristic curve analysis and the Youden's Index were applied to identify a cutoff value for distinguishing between the two tumor types, and the area under the curve (AUC), sensitivity, and specificity for the selected cutoff value were calculated. Results: Compared with brainstem gliomas, cerebellar medulloblastomas had significantly higher Kurtosis and rKurtosis values (p < 0.05). Medulloblastoma could be differentiated from brainstem gliomas using a Kurtosis value of 0.91 or an rKurtosis value of 0.90, both of which achieved 100% sensitivity, 96.7% specificity, and AUC values of 0.990. Conclusions: DKI measurements can contribute to distinguishing between cerebellar medulloblastoma and brainstem glioma in children.

Decoding the puzzle: A multidisciplinary systematic review of adult brainstem glioma.

Adult brainstem gliomas (BSGs) are a group of rare central nervous system tumors with varying prognoses and controversial standard treatment strategies. To provide an overview of current trends, a systematic review using the PRISMA guidelines, Class of evidence (CE) and strength of recommendation (SR), was conducted. The review identified 27 studies. Surgery was found to have a positive impact on survival, particularly for focal lesions with CE II SR C. Stereotactic image-guided biopsy was recommended when resective surgery was not feasible with CE II and SR B. The role of systemic treatments remains unclear. Eight studies provided molecular biology data. This review gathers crucial literature on diagnosis and management of adult BSGs. It provides evidence-based guidance with updated recommendations for diagnosing and treating, taking into account recent molecular and genetic advancements. The importance of brain biopsy is emphasized to optimize treatment using emerging genetic-molecular findings and explore potential targeted therapies.

An uncommon presentation of early brainstem high-grade glioma in a 33-year-old male: A case study and review of literature.

INTRODUCTION AND IMPORTANCE: Unlike children, high-grade brainstem glioma (HG-BSG) in adults is a rare and diverse group of tumors. They can be classified based on their location and physical characteristics, which distinguishes them from pediatric brainstem gliomas. They are rare in adults, constituting only 1 % to 2 % of intracranial gliomas. They are often aggressive and have a poor prognosis, with a median survival time of 24 months. The diagnosis of brainstem gliomas typically involves a combination of clinical evaluation and imaging studies, mainly magnetic resonance imaging (MRI), which provides detailed images and can help identify the characteristics of the tumor. CASE PRESENTATION AND METHODS: We present a case study of an uncommon presentation of an early stage of HG-BSG in a 33-year-old male, who had a contrast-enhancing lesion in the ventrolateral medulla that extended to the lower aspect of the fourth ventricle and caused ventricular compression. CLINICAL DISCUSSION: The findings were consistent with the literature on the current state of HG-BSG MRI findings, which typically show contrast-enhancing, hyperintense, and infiltrative lesions that involve the pons, midbrain, or medulla oblongata. The diagnosis of HG-BSG was based on clinical and radiological criteria, as the patient refused to undergo a surgical biopsy. We also performed a literature review on the current state of brainstem HG-BSG MRI findings, summarizing the main features and patterns of these tumors. CONCLUSION: MRI can offer useful information regarding the tumor's location, size, and features, as well as its impact on surrounding tissues and cerebrospinal fluid circulation.

Surgical Management of Adult Brainstem Gliomas: A Systematic Review and Meta-Analysis.

The present review aims to investigate the survival and functional outcomes in adult high-grade brainstem gliomas (BGSs) by comparing data from resective surgery and biopsy. MEDLINE, EMBASE and Cochrane Library were screened to conduct a systematic review of the literature, according to the PRISMA statement. Analysis was limited to articles including patients older than 18 years of age and those published from 1990 to September 2022. Case reports, review articles, meta-analyses, abstracts, reports of aggregated data, and reports on multimodal therapy where surgery was not the primary treatment were excluded. The ROBINS-I tool was applied to evaluate the risk of bias. Six studies were ultimately considered for the meta-analysis. The resective group was composed of 213 subjects and the bioptic group comprised 125. The analysis demonstrated a survival benefit in those patients in which an extensive resection was possible (STR HR 0.59 (95% CI 0.42, 0.82)) (GTR HR 0.63 (95% CI 0.43, 0.92)). Although surgical resection is associated with increased survival, the significantly higher complication rate makes it difficult to recommend surgery instead of biopsy for BSGs. Future investigations combining volumetric data and molecular profiles could add important data to better define the proper indication between resection and biopsy.

Systematic Review of WHO Grade 4 Astrocytoma in the Cerebellopontine Angle: The Impact of Anatomic Corridor on Treatment Options and Outcomes.

Background Despite advances in multimodal oncologic therapies and molecular genetics, overall survival (OS) in patients with high-grade astrocytomas remains poor. We present an illustrative case and systematic review of rare, predominantly extra-axial World Health Organization (WHO) grade 4 astrocytomas located within the cerebellopontine angle (CPA) and explore the impact of anatomic location on diagnosis, management, and outcomes. Methods A systematic review of adult patients with predominantly extra-axial WHO grade 4 CPA astrocytomas was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines through December 2022. Results Eighteen articles were included comprising 21 astrocytomas: 13 exophytic tumors arising from the cerebellopontine parenchyma and 8 tumors originating from a cranial nerve root entry zone. The median OS was 15 months with one-third of cases demonstrating delayed diagnosis. Gross total resection, molecular genetic profiling, and use of ancillary treatment were low. We report the only patient with an integrated isocitrate dehydrogenase 1 (IDH-1) mutant diagnosis, who, after subtotal resection and chemoradiation, remains alive at 40 months without progression. Conclusion The deep conical-shaped corridor and abundance of eloquent tissue of the CPA significantly limits both surgical resection and utility of device-based therapies in this region. Prompt diagnosis, molecular characterization, and systemic therapeutic advances serve as the predominant means to optimize survival for patients with rare skull base astrocytomas.

Prediction of H3K27M Alteration Status in Brainstem Glioma Using Multi-Shell Diffusion MRI Metrics.

BACKGROUND: Multi-shell diffusion characteristics may help characterize brainstem gliomas (BSGs) and predict H3K27M status. PURPOSE: To identify the diffusion characteristics of BSG patients and investigate the predictive values of various diffusion metrics for H3K27M status in BSG. STUDY TYPE: Prospective. POPULATION: Eighty-four BSG patients (median age 10.5 years [IQR 6.8-30.0 years]) were included, of whom 56 were pediatric and 28 were adult patients. FIELD STRENGTH/SEQUENCE: 3 T, multi-shell diffusion imaging. ASSESSMENT: Diffusion kurtosis imaging and neurite orientation dispersion and density imaging analyses were performed. Age, gender, and diffusion metrics, including fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity, radial diffusivity (RD), mean kurtosis (MK), axial kurtosis (AK), radial kurtosis, intracellular volume fraction (ICVF), orientation dispersion index, and isotropic volume fraction (ISOVF), were compared between H3K27M-altered and wildtype BSG patients. STATISTICAL TESTS: Chi-square test, Mann-Whitney U test, multivariate analysis of variance (MANOVA), step-wise multivariable logistic regression. P-values <0.05 were considered significant. RESULTS: 82.4% pediatric and 57.1% adult patients carried H3K27M alteration. In the whole group, the H3K27M-altered BSGs demonstrated higher FA, AK and lower RD, ISOVF. The combination of age and median ISOVF showed fair performance for H3K27M prediction (AUC = 0.78). In the pediatric group, H3K27M-altered BSGs showed higher FA, AK, MK, ICVF and lower RD, MD, ISOVF. The combinations of median ISOVF, 5th percentile of FA, median MK and median MD showed excellent predictive power (AUC = 0.91). In the adult group, H3K27M-altered BSGs showed higher ICVF and lower RD, MD. The 75th percentile of RD demonstrated fair performance for H3K27M status prediction (AUC = 0.75). DATA CONCLUSION: Different alteration patterns of diffusion measures were identified between H3K27M-altered and wildtype BSGs, which collectively had fair to excellent predictive value for H3K27M alteration status, especially in pediatric patients. EVIDENCE LEVEL: 2 TECHNICAL EFFICACY: Stage 3.

Classification of Brainstem Gliomas Based on Tumor Microenvironment Status.

The inter-tumor heterogeneity of the tumor microenvironment (TME) and how it correlates with clinical profiles and biological characteristics in brainstem gliomas (BSGs) remain unknown, dampening the development of novel therapeutics against BSGs. The TME status was determined with a list of pan-cancer conserved gene expression signatures using a single-sample gene set enrichment analysis (ssGSEA) and was subsequently clustered via consensus clustering. BSGs exhibited a high inter-tumor TME heterogeneity and were classified into four clusters: "immune-enriched, fibrotic", "immune-enriched, non-fibrotic", "fibrotic", and "depleted". The "fibrotic" cluster had a higher proportion of diffuse intrinsic pontine gliomas (p = 0.041), and "PA-like" tumors were more likely to be "immune-enriched, fibrotic" (p = 0.044). The four TME clusters exhibited distinct overall survival (p < 0.001) and independently impacted BSG outcomes. A four-gene panel as well as a radiomics approach were constructed to identify the TME clusters and achieved high accuracy for determining the classification. Together, BSGs exhibited high inter-tumor heterogeneity in the TME and were classified into four clusters with distinct clinical outcomes and tumor biological properties. The TME classification was accurately identified using a four-gene panel that can potentially be examined with the immunohistochemical method and a non-invasive radiomics method, facilitating its clinical application.

Deep learning based clinico-radiological model for paediatric brain tumor detection and subtype prediction.

Aim: Early diagnosis of paediatric brain tumors significantly improves the outcome. The aim is to study magnetic resonance imaging (MRI) features of paediatric brain tumors and to develop an automated segmentation (AS) tool which could segment and classify tumors using deep learning methods and compare with radiologist assessment. Methods: This study included 94 cases, of which 75 were diagnosed cases of ependymoma, medulloblastoma, brainstem glioma, and pilocytic astrocytoma and 19 were normal MRI brain cases. The data was randomized into training data, 64 cases; test data, 21 cases and validation data, 9 cases to devise a deep learning algorithm to segment the paediatric brain tumor. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of the deep learning model were compared with radiologist's findings. Performance evaluation of AS was done based on Dice score and Hausdorff95 distance. Results: Analysis of MRI semantic features was done with necrosis and haemorrhage as predicting features for ependymoma, diffusion restriction and cystic changes were predictors for medulloblastoma. The accuracy of detecting abnormalities was 90%, with a specificity of 100%. Further segmentation of the tumor into enhancing and non-enhancing components was done. The segmentation results for whole tumor (WT), enhancing tumor (ET), and non-enhancing tumor (NET) have been analyzed by Dice score and Hausdorff95 distance. The accuracy of prediction of all MRI features was compared with experienced radiologist's findings. Substantial agreement observed between the classification by model and the radiologist's given classification [K-0.695 (K is Cohen's kappa score for interrater reliability)]. Conclusions: The deep learning model had very high accuracy and specificity for predicting the magnetic resonance (MR) characteristics and close to 80% accuracy in predicting tumor type. This model can serve as a potential tool to make a timely and accurate diagnosis for radiologists not trained in neuroradiology.

Amide proton transfer-weighted imaging of pediatric brainstem glioma and its predicted value for H3 K27 alteration.

BACKGROUND: Non-invasive determination of H3 K27 alteration of pediatric brainstem glioma (pedBSG) remains a clinical challenge. PURPOSE: To predict H3 K27-altered pedBSG using amide proton transfer-weighted (APTw) imaging. MATERIAL AND METHODS: This retrospective study included patients with pedBSG who underwent APTw imaging and had the H3 K27 alteration status determined by immunohistochemical staining. The presence or absence of foci of markedly increased APTw signal in the lesion was visually assessed. Quantitative APTw histogram parameters within the entire solid portion of tumors were extracted and compared between H3 K27-altered and wild-type groups using Student's t-test. The ability of APTw for differential diagnosis was evaluated using logistic regression. RESULTS: Sixty pedBSG patients included 48 patients with H3 K27-altered tumor (aged 2-48 years) and 12 patients with wild-type tumor (aged 3-53 years). Visual assessment showed that the foci of markedly increased APTw signal intensity were more common in the H3 K27-altered group than in wild-type group (60% vs. 16%, P = 0.007). Histogram parameters of APTw signal intensity in the H3 K27-altered group were significantly higher than those in the wild-type group (median, 2.74% vs. 2.22%, P = 0.02). The maximum (area under the receiver operating characteristic curve [AUC] = 0.72, P = 0.01) showed the highest diagnostic performance among histogram analysis. A combination of age, median and maximum APTw signal intensity could predict H3 K27 alteration with a sensitivity of 81%, specificity of 75% and AUC of 0.80. CONCLUSION: APTw imaging may serve as an imaging biomarker for H3 K27 alteration of pedBSGs.

Phase I trial of panobinostat in children with diffuse intrinsic pontine glioma: A report from the Pediatric Brain Tumor Consortium (PBTC-047).

BACKGROUND: Diffuse intrinsic pontine glioma (DIPG) is a lethal childhood cancer with median survival of less than 1 year. Panobinostat is an oral multihistone deacetylase inhibitor with preclinical activity in DIPG models. Study objectives were to determine safety, tolerability, maximum tolerated dose (MTD), toxicity profile, and pharmacokinetics of panobinostat in children with DIPG. PATIENTS AND METHODS: In stratum 1, panobinostat was administered 3 days per week for 3 weeks on, 1 week off to children with progressive DIPG, with dose escalation following a two-stage continual reassessment method. After this MTD was determined, the study was amended to evaluate the MTD in children with nonprogressive DIPG/Diffuse midline glioma (DMG) (stratum 2) on an alternate schedule, 3 days a week every other week in an effort to escalate the dose. RESULTS: For stratum 1, 19 subjects enrolled with 17/19 evaluable for dose-finding. The MTD was 10 mg/m2/dose. Dose-limiting toxicities included thrombocytopenia and neutropenia. Posterior reversible encephalopathy syndrome was reported in 1 patient. For stratum 2, 34 eligible subjects enrolled with 29/34 evaluable for dose finding. The MTD on this schedule was 22 mg/m2/dose. DLTs included thrombocytopenia, neutropenia, neutropenia with grade 4 thrombocytopenia, prolonged intolerable nausea, and increased ALT. CONCLUSIONS: The MTD of panobinostat is 10 mg/m2/dose administered 3 times per week for 3 weeks on/1 week off in children with progressive DIPG/DMG and 22 mg/m2/dose administered 3 times per week for 1 week on/1 week off when administered in a similar population preprogression. The most common toxicity for both schedules was myelosuppression.

The Role of Advanced MRI Sequences in the Diagnosis and Follow-Up of Adult Brainstem Gliomas: A Neuroradiological Review.

The 2021 WHO (World Health Organization) classification of brain tumors incorporated the rapid advances in the molecular, genetic, and pathogenesis understanding of brain tumor pathogenesis, behavior, and treatment response. It revolutionized brain tumor classification by placing great emphasis on molecular types and completely splitting adult-type and pediatric-type diffuse gliomas. Brainstem gliomas (BSGs) are the leading primary tumors of the brainstem, although they are quite uncommon in adults compared with the pediatric population, representing less than 2% of adult gliomas. Surgery is not always the treatment of choice since resection is rarely feasible and does not improve overall survival, and biopsies are not generally performed since the location is treacherous. Therefore, MRI (Magnetic Resonance Imaging) without and with gadolinium administration represents the optimal noninvasive radiological technique to suggest brainstem gliomas diagnosis, plan a multidisciplinary treatment and for follow-up evaluations. The MRI protocol encompasses morphological sequences as well as functional and advanced sequences, such as DWI/ADC (Diffusion-Weighted Imaging/Apparent Diffusion Coefficient), DTI (Diffusion Tensor Imaging), PWI (Perfusion-Weighted Imaging), and MRS (Magnetic Resonance Spectroscopy), which improve the accuracy of the diagnosis of BSGs by adding substantial information regarding the cellularity, the infiltrative behavior toward the v fiber tracts, the vascularity, and the molecular changes. Brainstem gliomas have been divided into four categories on the basis of their MRI radiological appearance, including diffuse intrinsic low-grade gliomas, enhancing malignant gliomas, localized tectal gliomas, and other forms. The aim of our review is to provide insight into the role of advanced MRI sequences in the diagnosis and follow-up of adult brainstem gliomas.

Basilar invagination associated with brainstem tumor opposite the odontoid process.

An 8-year-old girl presented with the symptom of nasal regurgitation and hoarseness of voice. Investigations revealed severe basilar invagination and a large intra-axial pons-medulla brainstem tumor adjoining the tip of the odontoid process. The child underwent lateral mass plate and screw atlantoaxial fixation and attempted craniovertebral junction realignment. Biopsy of the brainstem tumor was done during the same surgical procedure. Histology of the brainstem tumor revealed it to be a pilocytic astrocytoma. Simultaneous presence of brainstem tumor and basilar invagination is a rare clinical association and a complex therapeutic challenge. The possible cause of genesis of brainstem tumor in an unusual location is speculated.

Radiotherapy combined or not with chemotherapy in adult or pediatric patients with brainstem glioma: a population-based study.

Background: The purpose of this study was to assess the treatment outcomes and prognostic factors of brainstem glioma (BCG) patients treated by radiotherapy (RT) or chemoradiation (CHRT) in the last 20 years in a population cohort. Materials and methods: Patients diagnosed with BSG from 2000-2020 treated by RT or CHRT were identified from The Fundação Oncocentro de São Paulo database. Data on age, gender, practice setting, period of treatment, and treatment modality were extracted. The overall survival (OS) was estimated, and the subgroups were compared with the log-rank test. Cox proportional test was used in multivariate analysis. Results: A total of 253 patients with a median follow-up of 12 months were included. There were 197 pediatric and 56 adult patients. For the entire cohort, the 1 and 3-year OS was 46%, and 23%, with a median OS of 11 months. In the subgroup analysis, adults had a median survival of 33 months versus 10 months in pediatric patients (p = 0.002). No significant difference in OS between RT and CHRT was observed in pediatric or adult subgroups (p > 0.05). The use of CHRT has significantly increased over the years. In the multivariate analysis, adult patients were the only independent prognostic factor associated with a better OS (p < 0.001). Conclusions: BSG had poor survival with no significant improvement in the treatment outcomes over the last 20 years, despite the addition of chemotherapy. Adult patients were independently associated with better survival.

Medullary brainstem gliomas in an adult: A rare case report and challenging tumor.

Background: Medullary brainstem lesions are rare tumors that are challenging to treat due to their location in the brainstem, which controls vital functions such as breathing, heart rate, and blood pressure. While the most common subtype is the aggressive diffuse intrinsic pontine glioma, other subtypes exist, including focal brainstem gliomas and cervicomedullary gliomas. The prognosis for patients with brainstem gliomas is generally poor, and treatment options are limited. Early detection and treatment are crucial to improve outcomes for patients with these tumors. Case Description: In this case report, the authors describe a 28-year-old male from Saudi Arabia who presented with headaches and vomiting. Imaging studies and clinical examination revealed a high-grade astrocytoma medullary brainstem lesion. The patient underwent radiation therapy and chemotherapy, effectively controlling tumor growth and improving his quality of life. However, a residual tumor remained, and the patient underwent neurosurgery to resect the remaining tumor was successful in removing the tumor, and the patient showed significant improvement in his symptoms and overall health. Conclusion: This case highlights the importance of early detection and treatment of medullary brainstem lesions. While radiation therapy and chemotherapy are primary treatment options, neurosurgery may be necessary to resect residual tumors. In addition, cultural and social factors may need to be considered in managing these tumors in Saudi Arabia.

Case Report: Brainstem angiocentric glioma presenting in a toddler child-diagnostic and therapeutic challenges.

Introduction: Angiocentric gliomas (AG) in brainstem location are exceedingly rare and might cause differential diagnostic problems and uncertainty regarding the best therapeutic approach. Hereby, we describe the clinicopathological findings in a brainstem AG presenting in a toddler child and review the literature. Case report: A 2-year-old boy presented with 5 weeks history of gait disturbances, frequent falls, left-sided torticollis and swallowing problems. MRI head showed a T2-hyperintense, partly exophytic mass lesion centred in the pontomedullary region, raising the possibility of diffuse midline glioma. The exophytic component was partially resected by suboccipital craniotomy, leaving intact the infiltrative component. Ventriculoperitoneal shunt was implanted due to postoperative hydrocephalus. Histological examination revealed a moderately cellular tumour consisted of bland glial cells infiltrating the brain parenchyma and radially arranged around the blood vessels. By immunohistochemistry, the tumour strongly expressed S100 and GFAP in addition to intense nestin positivity, while OLIG2 was negative in the perivascular tumour cells. DNA methylation array profiled the tumour as "methylation class diffuse astrocytoma, MYB or MYBL1-altered subtype B (infratentorial)" and an in-frame MYB::QKI fusion was identified by RNA sequencing, confirming the diagnosis of angiocentric glioma. The patient has been initially treated with angiogenesis inhibitor and mTOR inhibitor, and now he is receiving palliative vinblastine. He is clinically stable on 9 months follow-up. Conclusion: Brainstem AG may cause a diagnostic problem, and the surgical and oncological management is challenging due to unresectability and lack of response to conventional chemo-radiation. In the future, genetically-tailored therapies might improve the prognosis.