Brainstem glioma

INTRODUCTION: Acute acquired comitant esotropia (AACE) is an acquired strabismus with uncrossed sudden-onset diplopia due to esodeviation, comitant esotropia without accommodation factor, or paretic eye movement. The diagnosis of AACE entails differentiation from incomitant esotropia caused by abnormalities in the central nervous system. We present 2 pediatric patients with AACE as the first symptom of brainstem tumor. CASE PRESENTATION: The 2 patients were aware of their diplopia and had no other neurological abnormalities...

INTRODUCTION: Brainstem tumors represent a challenge. Their management and prognosis vary according to anatomopathological findings and genetic and bio-molecular fingerprints. We present our experience with pediatric brainstem tumors. MATERIAL AND METHODS: All patients admitted for a brainstem tumor at the Pediatric Neurosurgical Unit at Hôpital Femme Mère Enfant hospital between January 1997 and December 2019 were considered. Patients data were obtained through a retrospective review of the medical records; follow-up was from the last outpatient consultation...

OBJECTIVES: To investigate the value of diffusion MRI (dMRI) in H3K27M genotyping of brainstem glioma (BSG). METHODS: A primary cohort of BSG patients with dMRI data (b = 0, 1000 and 2000 s/mm2 ) and H3K27M mutation information were included. A total of 13 diffusion tensor and kurtosis imaging (DTI; DKI) metrics were calculated, then 17 whole-tumor histogram features and 29 along-tract white matter (WM) microstructural measurements were extracted from each metric and assessed within genotypes...

A man in his 30s presented with a 6-month history of progressive left face, arm and leg weakness. Medical history included epilepsy and vitamin B12 deficiency. Three maternal second degree relatives died before the age of 7 from various neurological disorders. Examination revealed a mild left facial droop and weakness of the left shoulder, hip and ankle. Reflexes were symmetrical and tone was normal. Differential diagnosis included glioma, subacute infarction, lymphoma and demyelination. MRI brain showed an extensive right sided subcortical white matter lesion, with extension into the brainstem...

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INTRODUCTION: H3 K27-altered diffuse midline glioma (DMG) is the most common malignant brainstem tumor in the pediatric population. Despite enormous preclinical and clinical efforts, the prognosis remains dismal, with fewer than 10% of patients surviving for two years after diagnosis. Fractionated radiation remains the only standard treatment options for DMG. Developing novel treatments and therapeutic delivery methods is critical to improving outcomes in this devastating disease. AREAS COVERED: This review addresses recent advances in molecularly targeted pharmacotherapy and immunotherapy in DMG...

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...

An 85-year-old woman was admitted to our hospital with unsteady gait, dizziness, nausea, and vomiting. MRI revealed characteristic abnormal signals in the bilateral cerebellar hemispheres. A brain biopsy was performed which confirmed a definitive histological diagnosis of diffuse glioma. Follow-up MRI showed diffuse abnormal signals that extended from the cerebellum to the brainstem through the cerebellar peduncle without mass formation. Her general condition gradually deteriorated even with the best supportive care, and she died 195 days after admission...

BACKGROUND: Herein, the authors describe the successful utilization of 5-aminolevulinic acid (5-ALA) and the first case of GammaTile cesium-131 therapy in a pediatric patient with recurrent high-grade glioma. 5-ALA was utilized to optimize gross-total resection prior to GammaTile implantation. After conversion to an equivalent dose in 2-Gy fractions (EQD2), a composite was made of the GammaTile dose with the initial external beam radiotherapy. Two hypothetical plans consisting of a standard hypofractionated strategy for glioma reirradiation and a CyberKnife plan using GammaTile's planning target volume were developed and likewise underwent EQD2 conversion and composite plan generation with the initial radiotherapy...

BACKGROUND: Germinomatous germ cell tumor is highly sensitive to chemoradiotherapy; patients are expected to survive for decades. Many radiation-induced malignant gliomas (RIMGs) occur >10 years after radiotherapy. Standard therapy for RIMGs has not been established because of the lesion's rarity, the patient's shorter survival period, and the risk of radiation necrosis by repeat radiation. OBSERVATIONS: Two patients, a 32-year-old man and a 50-year-old man, developed glioblastomas more than 20 years after radiation monotherapy for germinoma with or without mature teratoma...

Meningioangiomatosis (MA) is a rare proliferative meningovascular disorder that affects mainly the cerebral cortex, brainstem and spinal cord of humans and animals and can coexist with other proliferative disorders. A 7.5-year-old male Brazilian Campeiro Bulldog died after a convulsive crisis and cardiorespiratory arrest. At necropsy, a firm, white mass involving the piriform and right occipital lobes was seen. Histologically, the mass consisted of two morphologically distinct entities that collided: a congenital malformation characterized by a proliferation of meningothelial cells around blood vessels, within the perivascular spaces; and a neoplasm composed of two cell populations with astrocytic and oligodendrocytic differentiation...

Diffuse midline gliomas (DMGs) pose treatment challenges due to their location within the brainstem and invasive nature. Although classical immune checkpoint inhibitors have demonstrated limited success in clinical trials, Ausejo-Mauleon et al. demonstrate TIM-3 is an effective DMG strategy, targeting both immune and tumor cells for dual therapeutic benefit.

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...

Diffuse midline gliomas (DMGs) are a group of aggressive CNS tumors, primarily affecting children and young adults, which have historically been associated with dismal outcomes. As the name implies, they arise in midline structures in the CNS, primarily in the thalamus, brainstem, and spinal cord. In more recent years, significant advances have been made in our understanding of DMGs, including molecular features, with the identification of potential therapeutic targets. We aim to provide an overview of the most recent updates in the field of DMGs, including classification, molecular subtypes, diagnostic techniques, and emerging therapeutic strategies including a review of the ongoing clinical trials, thus providing the treating multidisciplinary team with a comprehensive understanding of the current landscape and potential therapeutic strategies for this devastating group of tumors...

BACKGROUND: Diffuse intrinsic pontine glioma (DIPG) is the most common and deadliest pediatric brainstem tumor and is difficult to treat with chemotherapy in part due to the blood-brain barrier (BBB). Focused ultrasound (FUS) and microbubbles (MBs) have been shown to cause BBB opening, allowing larger chemotherapeutics to enter the parenchyma. Panobinostat is an example of a promising in vitro agent in DIPG with poor clinical efficacy due to low BBB penetrance. In this study, we hypothesized that using FUS to disrupt the BBB allows higher concentrations of panobinostat to accumulate in the tumor, providing a therapeutic effect...

PURPOSE/OBJECTIVE(S): Despite being the second most frequent tumors in children, pediatric central nervous system (CNS) tumors are rare, and there are limited epidemiological data. The current study aimed to determine the survival rates of patients diagnosed with pediatric gliomas in Brazil, accounting for the influence of age, treatment modalities, and tumor site using population-based national database. MATERIALS/METHODS: Patients diagnosed with pediatric gliomas of CNS from 1999-2020 were identified from The Fundação Oncocentro de São Paulo database...

PURPOSE/OBJECTIVE(S): Radiotherapy (RT) improves survival in pediatric diffuse intrinsic pontine glioma (DIPG). After recurrence, re-irradiation (re-RT) offers palliation of symptoms and extends life in patients after exhaustion of investigational options and clinical decline. Practice patterns vary in dose and fractionation for re-RT, with majority of reported cases treated with conventional fractionation. Given radiobiologic rationale for hypofractionation in glioblastoma, we hypothesized that treatment with 3 Gy per fraction is safe and effective for re-RT to the brainstem for DIPG...

PURPOSE/OBJECTIVE(S): Diffuse midline glioma with H3K27 mutation is a fatal pediatric brain tumor, most commonly arising in the brainstem. This tumor remains universally fatal, despite a multitude of clinical trials, with a median overall survival of only 9-12 months. While immune-checkpoint inhibitors (ICIs) have transformed the treatment landscape of multiple solid tumors, delivery past the blood brain barrier (BBB) remains challenging. Programmed cell death protein 1 (PD1) is an immune checkpoint protein expressed on the surface of activated T cells; interaction with its ligand, PDL1, is tumor-protective, dampening T cell response...

Intraoperative neurophysiology (ION) in brainstem surgery evolved as brainstem surgery advanced.The original idea of brainstem mapping (BSM) is a neurophysiological procedure to locate cranial nerve motor nuclei (CNMN) on the floor of the fourth ventricle. With the introduction of various skull base approaches to the brainstem, BSM is carried out on any surface of the brainstem to expose the safe entry zone to the intrinsic brainstem lesion. It is the modern concept of BSM, a broader definition of BSM. BSM enables to avoid direct damage to the CNMN when approaching the brainstem through the negative mapping region...

Midline gliomas are tumors that occur in midline structures and can be circumscribed or diffuse. Classical midline structures include the thalamus, brainstem, and spinal cord. Other midline structures include the corpus callosum, basal ganglia, ventricles, paraventricular structures, and cerebellum. Diffuse midline glioma (DMG) is a diffuse glioma that occurs in the classical midline structures, characterized by a specific genetic alteration, and associated with grim outcome. This study was conducted at King Hussein Cancer Center and reviewed the medical records of 104 patients with circumscribed and diffuse gliomas involving midline structures that underwent biopsy between 2005 and 2022...