Brainstem glioma | Page 2

Altaf Ali Laghari, Mirza Zain Baig, Ehsan Bari, Aneela Darbar, Naureen Mushtaq, Umm E Hani Abdullah, Daniyal Aziz Khan
Objective: The aim of this study was to analyze the clinical profiles and outcomes of pediatric brainstem gliomas treated at our institute. Methodology: We reviewed the files of 18 pediatric age group patients diagnosed with brainstem glioma at our institution. The following variables were recorded: age, sex, duration of symptoms, date of diagnosis, main clinical symptoms, Karnofsky performance status score, magnetic resonance imaging findings, histopathology findings, details of the treatment given, disease progression, and date of mortality/last follow-up...
October 2019: Asian Journal of Neurosurgery
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 volume of distribution (Vd) in the brainstem, to better inform drug selection and delivery in future DIPG investigations. METHODS: A series of in vivo experiments were conducted using rat models...
January 1, 2020: Neurosurgical Focus
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 (DIPG), the authors measured treatment-related volumetric alterations in the brainstem and ventricles...
January 1, 2020: Neurosurgical Focus
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 H3K27M mutation in nearly 80% of DIPGs, leading to the 2016 WHO classification of diffuse midline glioma H3K27M-mutant, a grade IV brainstem tumor. Developments in epigenetic targeting of transcriptional tendencies have yielded potential molecular targets for clinical trials...
January 1, 2020: Neurosurgical Focus
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 confirmed high-grade brainstem glioma. Covariates including patient demographics, comorbidities, tumor characteristics and treatment parameters were captured...
December 24, 2019: Journal of Neuro-oncology
M Burhan Janjua, Vin Shen Ban, Tarek Y El Ahmadieh, Steven W Hwang, Amer F Samdani, Angela V Price, Bradley E Weprin, Hunt Batjer
Diffuse intrinsic pontine gliomas (DIPG) are high grade gliomas of the brainstem with fatal outcomes. Radiation is known to be partially effective to control the immediate flare but relapse is frequent. There has been ongoing research to study the role of molecular subgroups and identification of specific targets but this is not possible with histopathological diagnosis alone. The authors' objective is to highlight the need for and discuss ongoing molecular research. There is an inherent need for the availability of tumor tissue to be able to conduct research studies...
February 2020: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Victor M Lu, Oluwaseun O Akinduro, David J Daniels
A methionine substitution of lysine at residue 27 of histone H3 (H3K27M) mutation has become synonymous with malignant pediatric diffuse midline glioma (DMG), that occurs commonly in the brainstem. Therefore, recent reports that this same mutation occurs in malignant adult glioblastoma (GBM) located in the cerebellum are both unexpected and intriguing. The biological and clinical considerations of this novel finding are discussed.
January 2020: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Barbara Albuquerque Morais, Davi Jorge Fontoura Solla, Hamilton Matushita, Manoel Jacobsen Teixeira, Bernardo A Monaco
PURPOSE: Brainstem lesions comprise 10-20% of all pediatric brain tumors. Over the past years, the benefits of stereotactic biopsy versus the use alone of MRI features to guide treatment have been controversial. METHODS: Retrospective study with pediatric patients submitted to stereotactic brainstem biopsies between 2008 and 2018. Demographic, clinical, imaging, and surgical characteristics were recorded, as well as the histological diagnosis, complications, and survival...
December 13, 2019: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
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 DIPG. Our findings support this notion that K27M is an early event in development and suggest that the emergence of additional events resulted in rapid progression after a long period of latency...
March 2020: Pediatric Blood & Cancer
Hannah Flower, Pasquele Gallo
Introduction: Neurofibromatosis type 1 is an autosomal dominant tumour syndrome with an increased risk of developing central nervous system neoplasms, mostly benign low-grade gliomas involving the optic pathway and the brainstem. High-grade astrocytomas or glioblastoma multiforme (GBM) are rare. Cerebellar GBMs are rarer still, only seven cases NF1 patients have been reported Case description: We report a case of a cerebellar GBM in a 25-year-old male with NF1 who survived 18 months from the initial diagnosis without surgical debulking but only chemo and radiotherapy...
November 20, 2019: British Journal of Neurosurgery
Tianming Qiu, Apisara Chanchotisatien, Zhiyong Qin, Jinsong Wu, Zunguo Du, Xialing Zhang, Fangyuan Gong, Zhenwei Yao, Shuguang Chu
OBJECTIVE: H3 K27M-mutant gliomas present heterogeneously in terms of pathology, imaging, and prognosis. This study aimed to summarize the imaging characteristics of adult H3 K27M-mutant gliomas. METHODS: The authors retrospectively identified all cases of glioma diagnosed using histopathological studies (n = 3300) that tested positive for histone H3 K27M mutations (n = 75) between January 2016 and December 2018 in a single hospital. Preoperative and follow-up MR images of 66 adult patients (age ≥ 18 years) were reviewed for anatomical location, degree of contrast enhancement, enhancement patterns, hemorrhage, edema, diffusion restriction, tumor dissemination, and tumor spread...
November 15, 2019: Journal of Neurosurgery
Yousra Izzuddeen, Subhash Gupta, K P Haresh, Dayanand Sharma, Prashanth Giridhar, Gour Kishore Rath
INTRODUCTION: Diffuse intrinsic pontine glioma (DIPG) is the most common form of brainstem glioma. The present study was performed to assess if hypofractionated radiotherapy completed in < 3 weeks with temozolomide improves survival in DIPG. MATERIAL AND METHODS: The present study is a phase II open label randomized trial. The study included newly diagnosed patients with DIPG. Patients in arm A received conventional fractionated RT of 60 Gy in 30 fractions over 6 weeks while patients in arm B received hypo-fractionated radiotherapy of 39 Gy in 13 fractions over 2...
November 14, 2019: Journal of Neuro-oncology
José Antonio Valer, Cristina Sánchez-de-Diego, Carolina Pimenta-Lopes, Jose Luis Rosa, Francesc Ventura
Activin A receptor type I (ACVR1) encodes for a bone morphogenetic protein type I receptor of the TGFβ receptor superfamily. It is involved in a wide variety of biological processes, including bone, heart, cartilage, nervous, and reproductive system development and regulation. Moreover, ACVR1 has been extensively studied for its causal role in fibrodysplasia ossificans progressiva (FOP), a rare genetic disorder characterised by progressive heterotopic ossification. ACVR1 is linked to different pathologies, including cardiac malformations and alterations in the reproductive system...
October 31, 2019: Cells
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. The aim of this study was to generate new mouse models of DIPG, and characterize the role of specific oncogenic combinations in DIPG pathogenesis...
October 22, 2019: Neuro-oncology
Joshua Doyle, Adham M Khalafallah, Wuyang Yang, Yi Sun, Chetan Bettegowda, Debraj Mukherjee
BACKGROUND: Brainstem high-grade gliomas (HGG) are rare lesions with aggressive behavior that pose significant treatment challenges. The operative use of brainstem safe entry zones has made such lesions surgically accessible, though the benefits of aggressive resection have been unclear. This study aimed to clarify the survival in adult patients. METHODS: We utilized the SEER database (1973-2015) to analyze the association between survival and demographic data, tumor characteristics, and treatment factors in adult patients with brainstem HGGs...
October 16, 2019: Journal of Neuro-oncology
Johannes Holzapfel, Daniela Kandels, Rene Schmidt, Torsten Pietsch, Monika Warmuth-Metz, Brigitte Bison, Juergen Krauss, Rolf-Dieter Kortmann, Beate Timmermann, Ulrich-Wilhelm Thomale, Michael H Albert, Pablo Hernáiz Driever, Olaf Witt, Astrid K Gnekow
No abstract text is available yet for this article.
October 15, 2019: International Journal of Cancer. Journal International du Cancer
Jong Fu Wong, Elizabeth J Brown, Eleanor Williams, Alex N Bullock
Brain tumours have become the leading cause of child mortality from cancer. Indeed, aggressive brainstem tumours, such as diffuse intrinsic pontine glioma (DIPG), are nearly uniformly fatal. These tumours display a unique set of driver mutations that distinguish them from adult gliomas and define new opportunity for the development of precision medicines. The specific association of ACVR1 mutations with DIPG tumours suggests a direct link to neurodevelopment and highlights the encoded bone morphogenetic protein receptor kinase ALK2 as a promising drug target...
September 24, 2019: Biochemical Society Transactions
S Floro, D Belvedere, C Rosci, M Secchi, C Casellato, G Oggioni, C Gambini, L Campiglio, M Zardoni, R Altavilla, F Bianchi, A D'Arrigo, F Bartesaghi, M Egidi, C Uggetti, G Giaccone, G Marucci, A Mastronuzzi, M Vinci, A Priori
INTRODUCTION: Diffuse midline glioma is a newly WHO defined entity (grade IV) (Louis et al., 2016) which includes diffuse intrinsic pontine glioma (DIPG) reported in pediatric population and, occasionally, in young adults. Here, we present a detailed description of an atypical case of diffuse midline glioma in a 53 years old woman. CASE REPORT: A caucasian woman aged 53 from Ukraine, was referred to another neurological department complaining of 3 months history of progressive postural instability and gait impairment with frequent falling...
January 2020: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Basile Kerleroux, Jean Philippe Cottier, Kévin Janot, Antoine Listrat, Dominique Sirinelli, Baptiste Morel
Imaging plays a major role in the comprehensive assessment of posterior fossa tumor in children (PFTC). The objective is to propose a global method relying on the combined analysis of radiological, clinical and epidemiological criteria, (taking into account the child's age and the topography of the lesion) in order to improve our histological approach in imaging, helping the management and approach for surgeons in providing information to the patients' parents. Infratentorial tumors are the most frequent in children, representing mainly medulloblastoma, pilocytic astrocytoma and brainstem glioma...
February 2020: Journal of Neuroradiology. Journal de Neuroradiologie
Ridzky Firmansyah Hardian, Tetsuya Goto, Haruki Kuwabara, Yoshiki Hanaoka, Shota Kobayashi, Hiroyuki Kanno, Hisashi Shimojo, Tetsuyoshi Horiuchi, Kazuhiro Hongo
Background: Although glioblastoma has been shown to be able to disseminate widely in the intracranially after treatment with bevacizumab without any significant radiological findings, reports on such cases with subsequent autopsy findings are lacking. Case Description: A 36-year-old man presented with a general seizure and a mass of the right frontal lobe, which was diagnosed as diffuse astrocytoma (WHO Grade II). The patient underwent a total of four surgeries from 2005 to 2017...
2019: Surgical Neurology International
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