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Brainstem Gliomas

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Sources and lesion-induced changes of VEGF expression in brainstem motoneurons.

Silvia Silva-Hucha, Génova Carrero-Rojas, María Estrella Fernández de Sevilla, Beatriz Benítez-Temiño, María América Davis-López de Carrizosa, Angel M Pastor, Sara Morcuende,

Motoneurons of the oculomotor system show lesser vulnerability to neurodegeneration compared to other cranial motoneurons, as seen in amyotrophic lateral sclerosis (ALS). The overexpression of vascular endothelial growth factor (VEGF) is involved in motoneuronal protection. As previously shown, motoneurons innervating extraocular muscles present a higher amount of VEGF and its ... Read more >>

Brain Struct Funct (Brain structure & function)
[2020, :]

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Generation of diffuse intrinsic pontine glioma mouse models by brainstem-targeted in utero electroporation.

Smruti K Patel, Rachel M Hartley, Xin Wei, Robin Furnish, Fernanda Escobar-Riquelme, Heather Bear, Kwangmin Choi, Christine Fuller, Timothy N Phoenix,

BACKGROUND:Diffuse intrinsic pontine gliomas (DIPGs) are highly lethal childhood brain tumors. Their unique genetic makeup, pathological heterogeneity, and brainstem location all present challenges to treatment. Developing mouse models that accurately reflect each of these distinct features will be critical to advance our understanding of DIPG development, progression, and therapeutic resistance. ... Read more >>

Neuro-oncology (Neuro-oncology)
[2020, 22(3):381-392]

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Diffuse intrinsic pontine glioma-like tumor with EZHIP expression and molecular features of PFA ependymoma.

Drew Pratt, Martha Quezado, Zied Abdullaev, Debra Hawes, Fusheng Yang, Hugh J L Garton, Alexander R Judkins, Rajen Mody, Arul Chinnaiyan, Kenneth Aldape, Carl Koschmann, Sriram Venneti,

Acta Neuropathol Commun (Acta neuropathologica communications)
[2020, 8(1):37]

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Complications of stereotactic biopsy of lesions in the sellar region, pineal gland, and brainstem: A retrospective, single-center study.

Gang Cheng, Xin Yu, Hulin Zhao, Weidong Cao, Hailong Li, Qinggang Li, Zhicaho Li, Feng Yin, Rui Liu, Jianning Zhang,

Stereotactic biopsy (STB) is commonly used in the pathological diagnosis of intracranial lesions. The associated complication and mortality rates are low, but few reports with large sample sizes have assessed the complications of STB for lesions in the brain midline.To evaluate the complications of STB of lesions in the sellar ... Read more >>

Medicine (Baltimore) (Medicine)
[2020, 99(8):e18572]

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Impact of Intravenous Vitamin C and Endolaser Therapies on a Pediatric Brainstem Glioma Case.

Ivette M Solís-Nolasco, Gilberto Caraballo, Michael J González, José Olalde, Raul H Morales-Borges,

Pediatric cases of a brainstem glioma are one of the most difficult tumors to treat. In children, this type of cancer is perhaps the most dreaded of cancers due to its poor prognosis, yet it remains an area of intense research. In the case presented herein, conventional chemotherapy and radiotherapy ... Read more >>

Glob Adv Health Med (Global advances in health and medicine)
[2020, 9:2164956120901489]

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Progress in diffuse intrinsic pontine glioma: advocating for stereotactic biopsy in the standard of care.

John R Williams, Christopher C Young, Nicholas A Vitanza, Margaret McGrath, Abdullah H Feroze, Samuel R Browd, Jason S Hauptman,

Diffuse intrinsic pontine glioma (DIPG) is a universally fatal pediatric brainstem tumor affecting approximately 300 children in the US annually. Median survival is less than 1 year, and radiation therapy has been the mainstay of treatment for decades. Recent advances in the biological understanding of the disease have identified the ... Read more >>

Neurosurg Focus (Neurosurgical focus)
[2020, 48(1):E4]

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Epigenetic reprogramming and chromatin accessibility in pediatric diffuse intrinsic pontine gliomas: a neural developmental disease.

Flor M Mendez, Felipe J Núñez, Maria B Garcia-Fabiani, Santiago Haase, Stephen Carney, Jessica C Gauss, Oren J Becher, Pedro R Lowenstein, Maria G Castro,

Diffuse intrinsic pontine glioma (DIPG) is a rare but deadly pediatric brainstem tumor. To date, there is no effective therapy for DIPG. Transcriptomic analyses have revealed DIPGs have a distinct profile from other pediatric high-grade gliomas occurring in the cerebral hemispheres. These unique genomic characteristics coupled with the younger median ... Read more >>

Neuro-oncology (Neuro-oncology)
[2020, 22(2):195-206]

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Piles of profiles help identify vulnerabilities.

Sarah Seton-Rogers,

Nat. Rev. Cancer (Nature reviews. Cancer)
[2020, 20(2):73]

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Beyond the Blood: CSF-Derived cfDNA for Diagnosis and Characterization of CNS Tumors.

Abbye E McEwen, Sarah E S Leary, Christina M Lockwood,

Genetic data are rapidly becoming part of tumor classification and are integral to prognosis and predicting response to therapy. Current molecular tumor profiling relies heavily on tissue resection or biopsy. Tissue profiling has several disadvantages in tumors of the central nervous system, including the challenge associated with invasive biopsy, the ... Read more >>

Front Cell Dev Biol (Frontiers in cell and developmental biology)
[2020, 8:45]

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En mann i 60-årene med gangvansker, dysartri og lett kognitiv svikt.

Ludmila Nordaa, Elin Bjelland Forsaa, Audun Odland, Soffien Ajmi,

BACKGROUND:Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome is an immune-mediated, treatable and inflammatory CNS disease first reported by Pittock et al. (2010). CASE PRESENTATION:We describe a 66-year-old man with previous history of diabetes, atrial fibrillation and hypertension, who was admitted to hospital with reduced general ... Read more >>

Tidsskr. Nor. Laegeforen. (Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke)
[2020, 140(4):]

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Letter: High Prevalence of Developmental Venous Anomaly in Diffuse Intrinsic Pontine Gliomas: A Pediatric Control Study.

Waleed Brinjikji, Thomas J Sorenson, Giuseppe Lanzino,

Neurosurgery (Neurosurgery)
[2020, 86(2):E254]

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In Reply: High Prevalence of Developmental Venous Anomaly in Diffuse Intrinsic Pontine Gliomas: A Pediatric Control Study.

Alexandre Roux, Pascale Varlet, Johan Pallud,

Neurosurgery (Neurosurgery)
[2020, 86(2):E255]

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Keyhole Retrosigmoid Craniotomy for Resection of Pontine Juvenile Pilocytic Astrocytoma: 2-Dimensional Operative Video.

Kaan Yağmurlu, John Pomeraniec, Pedro Norat, M Yashar S Kalani, Hasan Syed,

Resection of intrinsic brainstem tumors is fraught with a high risk of morbidity. In select cases, however, complete tumor resection is possible and can result in long-term benefit to the patient. Informed consent for the operation was obtained. In this video, we illustrate the use of a keyhole retrosigmoid craniotomy ... Read more >>

Oper Neurosurg (Hagerstown) (Operative neurosurgery (Hagerstown, Md.))
[2020, 18(1):E16]

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Longitudinal Monitoring of Gd-DTPA Following Convection Enhanced Delivery in the Brainstem.

Umberto Tosi, Mark M Souweidane,

BACKGROUND:Convection-enhanced delivery (CED) has been introduced into contemporary therapeutic strategies for incurable brain neoplasms as diffuse intrinsic pontine glioma. Therapeutic benefit in part is predictably dependent on drug distribution within tumors. However, therapeutics can rarely be detected through conventional imaging techniques. Coinfusion of the tracer gadolinium-diethylenetriaminepentacetate (Gd-DTPA) has been advocated ... Read more >>

World Neurosurg (World neurosurgery)
[2020, 137:38-42]

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The role of radiation and chemotherapy in adult patients with high-grade brainstem gliomas: results from the National Cancer Database.

Panagiotis Kerezoudis, Anshit Goyal, Victor M Lu, Mohammed Ali Alvi, Mohamad Bydon, Sani H Kizilbash, Terry C Burns,

INTRODUCTION:Surgical resection of high-grade brainstem gliomas is challenging and treatment mostly involves radiation and chemotherapy. In this study, we utilized registry data to determine prognostic features and impact of chemotherapy and radiation on overall survival. METHODS:The National Cancer Database was queried from 2006 to 2015 for adult cases with histologically ... Read more >>

J. Neurooncol. (Journal of neuro-oncology)
[2020, 146(2):303-310]

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Deformational changes after convection-enhanced delivery in the pediatric brainstem.

Evan D Bander, Karima Tizi, Eva Wembacher-Schroeder, Rowena Thomson, Maria Donzelli, Elizabeth Vasconcellos, Mark M Souweidane,

OBJECTIVE:In the brainstem, there are concerns regarding volumetric alterations following convection-enhanced delivery (CED). The relationship between distribution volume and infusion volume is predictably greater than one. Whether this translates into deformational changes and influences clinical management is unknown. As part of a trial using CED for diffuse intrinsic pontine glioma ... Read more >>

Neurosurg Focus (Neurosurgical focus)
[2020, 48(1):E3]

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Cervicomedullary Ependymoma with Hemorrhage: A Case Report and Review of Literature.

Raja K Kutty, Kazumi Ohmori, Yasuhiro Yamada, Yoko Kato,

Ependymoma is a rare tumor central nervous system that arises from the ependymal lining of the ventricles or the central canal of the spinal cord. They are of neuroectodermal in origin and constitute about 30%-86% of tumors arising in the spinal cord. The occurrence of these tumors in the cervicomedullary ... Read more >>

Asian J Neurosurg (Asian journal of neurosurgery)
[2020, 15(1):190-193]

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Evaluating infusate parameters for direct drug delivery to the brainstem: a comparative study of convection-enhanced delivery versus osmotic pump delivery.

Julian S Rechberger, Erica A Power, Victor M Lu, Liang Zhang, Jann N Sarkaria, David J Daniels,

OBJECTIVE:Convection-enhanced delivery (CED) and osmotic pump delivery both have been promoted as promising techniques to deliver drugs to pediatric diffuse intrinsic pontine gliomas (DIPGs). Correspondingly, the aim of this study was to understand how infusate molecular weight (MW), duration of delivery, and mechanism of delivery (CED or osmotic pump) affect ... Read more >>

Neurosurg Focus (Neurosurgical focus)
[2020, 48(1):E2]

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Adult H3K27M-mutant diffuse midline glioma with gliomatosis cerebri growth pattern: Case report and review of the literature.

Anudeep Yekula, Mihir Gupta, Nicholas Coley, Hoi Sang U,

INTRODUCTION:H3K27M-mutant diffuse midline glioma is a recently classified unique entity predominantly affecting pediatric patients and rarely adults. The clinicopathologic features in adults remain poorly characterized. PRESENTATION OF CASE:A 36-year-old man presented with subacute progressive cognitive and visual deterioration, and hydrocephalus requiring ventricular shunting. MRI revealed a diffusely infiltrating lesion with ... Read more >>

Int J Surg Case Rep (International journal of surgery case reports)
[2020, 68:124-128]

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The role of large volume re-irradiation with Bevacizumab in chemorefractory high grade glioma.

Joseph Chan, Dasantha Jayamanne, Helen Wheeler, Mustafa Khasraw, Matthew Wong, Marina Kastelan, Lesley Guo, Michael Back,

Background and purpose:Current practice in re-irradiation (reRT) of previously treated high-grade gliomas (HGG) has generally been limited to small volume reRT with stereotactic procedures. Less evidence exists for large volume reRT involving treatment volumes equivalent to that used at initial diagnosis. The primary aim of this study was to investigate ... Read more >>

Clin Transl Radiat Oncol (Clinical and Translational Radiation Oncology)
[2020, 22:33-39]

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Combined Targeting of Mutant p53 and Jumonji Family Histone Demethylase Augments Therapeutic Efficacy of Radiation in H3K27M DIPG

Anatoly Nikolaev, John Fiveash, Eddy Yang,

Diffuse intrinsic pontine glioma (DIPG) is an aggressive pediatric brainstem tumor with a 5-year survival of <1%. Up to 80% of DIPG tumors contain a specific K27M mutation in one of two genes encoding histone H3 (H3K27M). Furthermore, p53 mutations found in >70-80% of H3K27M DIPG, and mutant p53 status ... Read more >>

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Therapeutic efficacy of an immune stimulatory gene therapy strategy in a mouse model of high grade brainstem glioma

Flor Mendez, Padma Kadiyala, Felipe Nunez, Stephen Carney, Fernando Nunez, Jessica Gauss, Ramya Ravindran, Sheeba Pawar, Marta Edwards, Pedro Lowenstein, Maria Castro,

Abstract Purpose Diffuse intrinsic pontine glioma (DIPG) bears a dismal prognosis. A genetically engineered brainstem glioma model harboring the recurrent DIPG mutation, ACVR1-G328V (mACVR1), was developed for testing an immune-stimulatory gene therapy. Experimental Design We utilized the Sleeping Beauty transposase system to generate an endogenous mouse model of mACVR1 brainstem ... Read more >>

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Radiation dose response of neurologic symptoms during conformal radiotherapy for diffuse intrinsic pontine glioma.

Christopher L Tinkle, Kristen Campbell, Yuanyuan Han, Yimei Li, Brandon Bianski, Alberto Broniscer, Raja B Khan, Thomas E Merchant,

PURPOSE:To estimate the rate and magnitude of neurologic symptom change during radiation therapy (RT) and impact of symptom change on survival outcomes in patients with diffuse intrinsic pontine glioma (DIPG). METHODS:From 2006 to 2014, 108 patients with newly diagnosed DIPG were treated with conventionally fractionated radiation therapy (RT) to 54 ... Read more >>

J. Neurooncol. (Journal of neuro-oncology)
[2020, 147(1):195-203]

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Indolent course of brainstem tumors with K27M-H3.3 mutation.

Lorena V Baroni, Palma Solano-Paez, Liana Nobre, Orli Michaeli, Cynthia Hawkins, Suzanne Laughlin, Ute Bartels, Vijay Ramaswamy, Eric Bouffet,

Diffuse intrinsic pontine glioma (DIPG) is characterized by a short history of brainstem symptoms and well-known magnetic resonance imaging features with a fatal outcome. However, we report three unusual cases of brainstem tumors with an initial indolent and protracted course, which subsequently developed the classical imaging and clinical features of ... Read more >>

Pediatr Blood Cancer (Pediatric blood & cancer)
[2020, 67(3):e28102]

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Stereotactic Radiotherapy with Fractionation for the Lesions in and Around the Brainstem and Optic Nerve.

Yoshihisa Kida, Yoshimasa Mori,

Purpose Among the components of the central nervous system, the optic nerve and the brainstem are considered to be the eloquent structures that are sensitive to stereotactic radiosurgery (SRS) and stereotactic radiotherapy (SRT). SRS or SRT with fractionation in areas adjacent to these tissues is both promising and challenging. Materials ... Read more >>

Cureus (Cureus)
[2019, 11(11):e6087]

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