Brainstem glioma

OBJECTIVE: Diffuse intrinsic pontine glioma (DIPG) is a rare and devastating brainstem glioma that occurs predominately in children. To date, the prognostic impact of radiotherapy (RT) in conjunction with temozolomide (TMZ) in DIPG has not been thoroughly analyzed. The aim of this meta-analysis was to quantitatively and precisely analyze the effectiveness of RT in conjunction with TMZ in improving the prognosis of DIPG. METHODS: A systematic search of eight electronic databases was conducted...

Part 1 of this series of two articles describes conventional and advanced MRI techniques that are useful for evaluating brainstem pathologies. In addition, it provides a review of the embryology, normal progression of myelination, and clinically and radiologically salient imaging anatomy of the normal brainstem. Finally, it discusses congenital diseases of the brainstem with a focus on distinctive imaging features that allow for differentiating pathologies. Part 2 of this series of two articles includes discussion of neoplasms; infections; and vascular, demyelinating, toxic, metabolic and miscellaneous disease processes affecting the brainstem...

Angiocentric glioma is a rare brain tumor commonly found in frontal or temporal lobes. It has a benign course, and surgical resection can be curative. Brainstem location is extremely rare, with only six cases reported so far in the literature. In the present study, the seventh case of brainstem angiocentric glioma has been reported, and its course in comparison with supratentorial location and the role of molecular diagnosis has been discussed.

Anaplastic ganglioglioma (AGG) is a rare and aggressive counterpart of the more benign and frequently encountered glioma. Herein, we present a 21-year-old female who presented with episodes of total amnesia and complex partial seizures, which led to the diagnosis of AGG localized to the medial temporal lobe. She subsequently underwent surgical cytoreduction of the tumor three times with adjuvant chemoradiotherapy. The extent of resection throughout the surgeries was hindered by the extension of the tumor to critical neurovascular structures; during the last surgery, invasion into the pons was noted, which posed a significant clinical challenge...

Diffuse intrinsic pontine glioma (DIPG) is a rare brainstem tumor which carries a dismal prognosis. To date. there are no effective treatments for DIPG. Transcriptomic studies have shown that DIPGs have a distinct profile compared to hemispheric high-grade pediatric gliomas. These specific genomic features coupled with the younger median age group suggest that DIPG is of developmental origin. There is a major unmet need for novel effective therapeutic approaches for DIPG. Clinical and preclinical studies have expanded our understanding of the molecular pathways in this deadly disease...

PURPOSE: CNS malignancies are currently the most common cause of disease related deaths in children. Although brainstem gliomas are invariably fatal cancers in children, clinical studies against this disease are limited. This review is to lead to a succinct collection of knowledge of known biological mechanisms of this disease and discuss available therapeutics. METHODS: A hallmark of brainstem gliomas are mutations in the histone H3.3 with the majority of cases expressing the mutation K27M on histone 3...

OBJECTIVE: Diffusion tensor imaging (DTI) and diffusion tensor tractography (DTT) have the ability to noninvasively visualize changes in white matter tracts, as well as their relationships with lesions and other structures. DTI/DTT has been increasingly used to improve the safety and results of surgical treatment for lesions in eloquent areas, such as brainstem cavernous malformations. This study aimed to investigate the application value of DTI/DTT in brainstem glioma surgery and to validate the spatial accuracy of reconstructed corticospinal tracts (CSTs)...

Tectal gliomas are a rare subset of intrinsic brainstem lesions. The microsurgical resection of these lesions remains a major challenge.1,2 Transcollicular approaches on one side, via the superior or inferior colliculi or both, are neurologically well tolerated without obvious or major auditory or oculomotor consequences. However, any postoperative acute visually triggered saccadic abnormalities caused by iatrogenic superior colliculus damage generally resolve during the postoperative period, as other oculomotor structures compensate for these functions in unilateral lesions...

Brainstem gliomas are aggressive tumors that are more prevalent in pediatric patients. The location of these tumors makes them inoperable, and currently there is no effective treatment. Recent genomic data revealed the unique biology of these tumors. The following protocol provides a method to incorporate these specific genetic lesions in a mouse glioma model. Using this model, the effects of these mutations in tumor progression and response to treatments can be studied within a relevant in vivo context. For complete details on the use and execution of this protocol, please refer to Mendez et al...

OBJECTIVE: Biopsies of tumors located in deep midline structures require highly accurate stereotaxy to safely obtain lesional tissue suitable for molecular and histological analysis. Versatile platforms are needed to meet a broad range of technical requirements and surgeon preferences. The authors present their institutional experience with the robotic stereotactic assistance (ROSA) system in a series of robot-assisted biopsies of pediatric brainstem and thalamic tumors. METHODS: A retrospective analysis was performed of 22 consecutive patients who underwent 23 stereotactic biopsies of brainstem or thalamic lesions using the ROSA platform at Rady Children's Hospital in San Diego between December 2015 and January 2020...

Background: "Diffuse midline glioma (DMG), H3 K27M-mutant" is a new tumor entity established in the 2016 WHO classification of Tumors of the Central Nervous System that comprises a set of diffuse gliomas arising in midline structures and is molecularly defined by a K27M mutation in genes encoding the histone 3 variants H3.3 or H3.1. While this tumor entity is associated with poor prognosis in children, clinical experience in adults remains limited. Methods: Patient demographics, radiologic and pathologic characteristics, treatment course, progression, and patient survival were collected for 60 adult patients with DMG, H3 K27M-mutant...

BACKGROUND: Limited population-based data exists for the brainstem gliomas for children ages ≤19 years, which includes high-grade aggressively-growing tumors such as diffuse intrinsic pontine glioma (DIPG). We examined the overall incidence and survival patterns in children with brainstem High-Grade glioma (HGG) by age, sex, and race and ethnicity. METHODS: We used data from Central Brain Tumor Registry of the United States (CBTRUS), obtained through data use agreements with the Centers for Disease Control (CDC) and the National Cancer Institute (NCI) from 2000 - 2017, and survival data from the CDC's National Program of Cancer Registries (NPCR), from 2001 - 2016 for malignant brainstem HGG for ages ≤19 years (per WHO ICD-O-3 codes)...

OBJECTIVE: The endoscopic endonasal approach (EEA) has been proposed as an alternative in the surgical removal of ventral brainstem lesions. However, the feasibility and limitations of this approach to treat such pathologies are still poorly understood. This study aimed to report our experience in five consecutive cases of intrinsic brainstem lesions that were managed via an EEA, as well as the specific anatomy of each case. METHODS: All patients were treated in a single center by a multidisciplinary surgical team between 2015 and 2019...

OBJECTIVE: The extreme lateral supracerebellar infratentorial (ELSI) approach has the potential to access several distinct anatomical regions, otherwise difficult to reach. The surgical anatomy is illustrated through cadaveric dissections. An extensive review of the literature is provided in order to highlight the versatility of this approach along with its limits and comparisons with alternative approaches. METHODS: The surgical anatomy of the ELSI was described through one adult injected cadaveric head...

Diffuse midline gliomas (DMGs) are a diffuse glioma subtype arising from midline brain structures. It is predominantly a disease of childhood; however, it can also occur in adults. Adult DMG has not been previously well described. The aim of this study was to define the characteristics of adult DMG. We described and analyzed the clinical, radiological, and genetic alterations of 9 adult DMGs and compared them with those of 257 non-midline adult high-grade IDH-WT gliomas. The median age of all patients was 38-years old (23-68-years)...

Brainstem gliomas comprise 10% to 20% of pediatric intracranial tumors.1 Gross total resection is associated with the greatest long-term survival.1 However, due to the eloquence of surrounding brain tissue, an aggressive resection is not always achievable and can be associated with significant risk.2 Sodium fluorescein can be used to help differentiate between tumor and normal tissue.3,4 In this operative video, we demonstrate the step-by-step technique to utilize fluorescence in surgically resecting an exophytic glioma arising from the midbrain in a 16-yr-old male...

BACKGROUND: F18-FET PET has an established diagnostic role in adult brain gliomas, here we analyzed image derived static & dynamic parameters with available conventional MRI, histological, clinical & follow up data in assessment of pediatric brain tumor patient at different stages of the disease. METHODS: Forty-four pediatric patients with median age 7 years, diagnosed with brain tumors and underwent forty-seven 18F-FET PET scans either initially (20 scans) or post-therapy (27 scans) were enrolled...

Background: Tissue diagnosis is essential in the usual management of high-grade glioma. In rare circumstances, due to patient preference, performance status, comorbidities, or tumor location, biopsy is not feasible. Sometimes a biopsy is nondiagnostic. Many neuro-oncology clinics have patients like this, but these patients' outcomes and responses to treatment are not known. Methods: We retrospectively reviewed records from adult patients diagnosed with presumed high-grade glioma of the brain without definitive pathology, diagnosed between 2004 and 2016...

BACKGROUND: Childhood diffuse brainstem glioma (dBSG) is a rare tumor with a poor prognosis. Any tumor-directed surgical intervention is difficult. Magnetic resonance imaging forms the mainstay of diagnosis and radiation therapy has remained the backbone of therapy. In this study, we compare the outcomes of conformal radiotherapy with conventional therapy in the context of resource-constrained settings. METHODS: In this retrospective analysis, conducted between 2010 and 2019, all pediatric patients with a diagnosis of dBSG were analyzed...

PURPOSE: Diffuse intrinsic pontine glioma (DIPG) is among the deadliest of pediatric brain tumors. Radiation therapy is the standard of care treatment for DIPG, but offers only transient relief of symptoms for DIPG patients without providing significant survival benefit. Oncolytic virotherapy (OV) is an anti-cancer treatment that has been investigated for treating various types of brain tumors. EXPERIMENTAL DESIGN: Here, we have explored the use of mesenchymal stem cells (MSC) for OV delivery and evaluated treatment efficacy using preclinical models of DIPG...