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low grade pontine glioma

Yuehui Liu, Haiping Liu, Dongwei Zhang
Diffuse intrinsic pontine glioma (DIPG) is one of the most devastating types of pediatric cancer. Accumulating evidence suggests that the dysregulated expression of long non-coding (lnc)-RNAs is associated with various pathologies of the CNS. However, the expression patterns and prognostic roles of lncRNAs in DIPG have not yet been systematically determined. In the present study, lncRNA expression profiles were obtained from the Gene Expression Omnibus (GEO) database using the lncRNA-mining approach and a differential expression analysis for lncRNAs was performed between DIPG and low-grade brainstem glioma and DIPG and normal pediatric brainstem tissue...
November 2018: Oncology Letters
D S Tsang, N J Laperriere
Despite best available therapy, many children with cancer develop recurrence after multimodal treatment, including initial radiation therapy. Re-irradiation is defined as the use of a second course of radiation therapy with a retreatment volume that overlaps substantially with that of a previously delivered course of radiation therapy. Re-irradiation is an important part of salvage treatment for patients with recurrent ependymoma, diffuse intrinsic pontine glioma, medulloblastoma and germinoma. In patients with ependymoma, conventionally fractionated re-irradiation (1...
October 29, 2018: Clinical Oncology: a Journal of the Royal College of Radiologists
Nicole A P Lieberman, Kole DeGolier, Heather M Kovar, Amira Davis, Virginia Hoglund, Jeffrey Stevens, Conrad Winter, Gail Deutsch, Scott N Furlan, Nicholas A Vitanza, Sarah E S Leary, Courtney A Crane
Background: Diffuse Intrinsic Pontine Glioma (DIPG) is a uniformly fatal CNS tumor diagnosed in 300 American children per year. Radiation is the only effective treatment and extends overall survival to a median of 11 months. Due to its location in the brainstem, DIPG tumors cannot be surgically resected. Immunotherapy has the ability to target tumor cells specifically, however, little is known about the tumor microenvironment in DIPGs. We sought to characterize infiltrating immune cells and immunosuppressive factor expression in pediatric low- and high-grade gliomas and DIPG...
August 28, 2018: Neuro-oncology
Grant L Lin, Surya Nagaraja, Mariella G Filbin, Mario L Suvà, Hannes Vogel, Michelle Monje
Diffuse intrinsic pontine glioma (DIPG) is a universally fatal malignancy of the childhood central nervous system, with a median overall survival of 9-11 months. We have previously shown that primary DIPG tissue contains numerous tumor-associated macrophages, and substantial work has demonstrated a significant pathological role for adult glioma-associated macrophages. However, work over the past decade has highlighted many molecular and genomic differences between pediatric and adult high-grade gliomas. Thus, we directly compared inflammatory characteristics of DIPG and adult glioblastoma (GBM)...
June 28, 2018: Acta Neuropathologica Communications
Keita Terashima
Under specific indications, chemotherapy may play an important role in the treatment of pediatric patients with intracranial gliomas. It can be effectively administered in inoperable low-grade tumors, particularly with the use of combination regimens based on carboplatin and vincristine. In very young children with high-grade gliomas (HGG), chemotherapy may result in control of tumor growth, which allows to postpone fractionated radiation therapy (FRT). At the same time, in difference with adults, there is no current evidence that addition of chemotherapy to aggressive surgical resection followed by FRT has any positive impact on survival of pediatric patients with non-pontine HGG...
2018: Progress in Neurological Surgery
James A Balogun, James T Rutka
Gliomas are the most common type of brain cancer in the pediatric patients, constituting about 50% of all childhood intracranial tumors. This is a highly heterogeneous group, varying from the benign WHO histopathological grades I and II to malignant WHO grades III and IV. The histology and location are significant prognostic factors, which influence the decision for surgical intervention, as well as the extent of possible tumor removal. In low-grade gliomas, surgery remains the initial option and should be directed at gross total resection in favorable locations, such as the cerebral hemispheres and the cerebellum...
2018: Progress in Neurological Surgery
Fanfan Chen, Zongyang Li, Chengyin Weng, Peng Li, Lanbo Tu, Lei Chen, Wei Xie, Ling Li
Multifocal pontine glioblastoma exhibiting an exophytic growth pattern in the cerebello-pontine angle (CPA) is rare. We present a case of a 5-year-old girl with consecutive neurological imaging and other clinical findings indicating progressive multifocal exophytic pontine glioblastoma. Three lesions were reported, of which two were initially presented, and one was developed 2 months later. One lesion demonstrated a progressing exophytic extension in the cistern of the left side of the CPA. The other two lesions were located and confined within the pons...
March 27, 2017: Chinese Journal of Cancer
Kenichi Ishibashi, Takeshi Inoue, Hiroko Fukushima, Yusuke Watanabe, Yoshiyasu Iwai, Hiroaki Sakamoto, Kai Yamasaki, Jyunichi Hara, Tomoko Shofuda, Daiksuke Kanematsu, Ema Yoshioka, Yonehiro Kanemura
PURPOSE: Histone H3.3 (H3F3A) mutation in the codon for lysine 27 (K27M) has been found as driver mutations in pediatric glioblastoma and has been suggested to play critical roles in the pathogenesis of thalamic gliomas and diffuse intrinsic pontine gliomas. We report a case of thalamic glioma with H3F3A K27M mutation, which was detected in both the primary tumor diagnosed as diffuse astrocytoma obtained during the first surgery and also in the tumor diagnosed as anaplastic astrocytoma obtained at the second surgery...
December 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Matthias W Wagner, W Robert Bell, Jason Kern, Thangamadhan Bosemani, Joyce Mhlanga, Kathryn A Carson, Kenneth J Cohen, Eric H Raabe, Fausto Rodriguez, Thierry A G M Huisman, Andrea Poretti
PURPOSE: To apply DTI to detect early extrapontine extension of pediatric diffuse intrinsic pontine glioma along the corticospinal tracts. METHODS: In children with diffuse intrinsic pontine glioma, low-grade brainstem glioma, and age-matched controls, DTI metrics were measured in the posterior limb of the internal capsule and posterior centrum semiovale. Histological examination was available in one patient. RESULTS: 6 diffuse intrinsic pontine glioma, 8 low-grade brainstem glioma, and two groups of 25 controls were included...
April 2016: European Journal of Radiology
Katherine L Misuraca, Guo Hu, Kelly L Barton, Alexander Chung, Oren J Becher
Diffuse intrinsic pontine glioma (DIPG) is a rare and incurable brain tumor that arises predominately in children and involves the pons, a structure that along with the midbrain and medulla makes up the brainstem. We have previously developed genetically engineered mouse models of brainstem glioma using the RCAS/Tv-a system by targeting PDGF-B overexpression, p53 loss, and H3.3K27M mutation to Nestin-expressing brainstem progenitor cells of the neonatal mouse. Here we describe a novel mouse model targeting these same genetic alterations to Pax3-expressing cells, which in the neonatal mouse pons consist of a Pax3+/Nestin+/Sox2+ population lining the fourth ventricle and a Pax3+/NeuN+ parenchymal population...
January 2016: Neoplasia: An International Journal for Oncology Research
Paul Klimo, Cody L Nesvick, Alberto Broniscer, Brent A Orr, Asim F Choudhri
OBJECT Malignant tumors of the brainstem, excluding classic diffuse intrinsic pontine gliomas (DIPGs), are a very rare, heterogeneous group of neoplasms that have been infrequently described in the literature. In this paper, the authors present their experiences with treating these unique cancers. METHODS A retrospective chart review was conducted to identify eligible cases over a 15-year period. All tumors involving the pons were, by consensus, felt not to be DIPGs based on their neuroimaging features. Demographic information, pathological specimens, neuroimaging characteristics, surgical and nonsurgical management plans, and survival data were gathered for analysis...
January 2016: Journal of Neurosurgery. Pediatrics
Amar Gajjar, Daniel C Bowers, Matthias A Karajannis, Sarah Leary, Hendrik Witt, Nicholas G Gottardo
Pediatric neuro-oncology has undergone an exciting and dramatic transformation during the past 5 years. This article summarizes data from collaborative group and institutional trials that have advanced the science of pediatric brain tumors and survival of patients with these tumors. Advanced genomic analysis of the entire spectrum of pediatric brain tumors has heralded an era in which stakeholders in the pediatric neuro-oncology community are being challenged to reconsider their current research and diagnostic and treatment strategies...
September 20, 2015: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
Vishwajith Sridharan, Laura M Urbanski, Wenya Linda Bi, Katrina Thistle, Michael B Miller, Shakti Ramkissoon, David A Reardon, Ian F Dunn
BACKGROUND: Multicentric low-grade gliomas are rare entities that occur in disparate regions of the brain. They can present with distinct pathologic and imaging findings and may harbor a worse prognosis. We present a case of multicentric low-grade gliomas and highlight their pathogenesis, imaging characteristics, and molecular signatures, with implications for clinical management. CASE: A 49-year-old man presented with left-sided headaches for 3 months. Magnetic resonance imaging revealed concurrent non-enhancing lesions in the left medial temporal lobe and superior cerebellum...
October 2015: World Neurosurgery
Branavan Manoranjan, Neha Garg, David Bakhshinyan, Sheila K Singh
Representing the leading cause of childhood cancer mortality, pediatric brain tumors are comprised of diverse histological features, genetic perturbations, cellular populations, treatment protocols, and clinical outcomes. In this chapter we discuss recent and emerging data that implicate cancer stem cells (also known as brain tumor-initiating cells) in initiating and maintaining the growth of a number of pediatric brain tumors including: medulloblastoma, supratentorial primitive neuroectodermal tumor, atypical teratoid/rhabdoid tumor, ependymoma, low-grade glioma, glioblastoma, diffuse intrinsic pontine glioma, germ cell tumor, and craniopharyngioma...
2015: Advances in Experimental Medicine and Biology
Satish Kallappagoudar, Rajesh K Yadav, Brandon R Lowe, Janet F Partridge
Brain tumors are the most common solid tumors in children. Pediatric high-grade glioma (HGG) accounts for ∼8-12 % of these brain tumors and is a devastating disease as 70-90 % of patients die within 2 years of diagnosis. The failure to advance therapy for these children over the last 30 years is largely due to limited knowledge of the molecular basis for these tumors and a lack of disease models. Recently, sequencing of tumor cells revealed that histone H3 is frequently mutated in pediatric HGG, with up to 78 % of diffuse intrinsic pontine gliomas (DIPGs) carrying K27M and 36 % of non-brainstem gliomas carrying either K27M or G34R/V mutations...
June 2015: Chromosoma
Adam L Green, Mark W Kieran
Pediatric brainstem gliomas include low-grade focal brainstem gliomas (FBSG) and high-grade diffuse intrinsic pontine gliomas (DIPG). These tumors share a crucial and eloquent area of the brain as their location, which carries common challenges for treatment. Otherwise, though, these two diseases are very different in terms of presentation, biology, treatment, and prognosis. FBSG usually present with greater than 3 months of symptoms, while DIPG are usually diagnosed within 3 months of symptom onset. Surgery remains the preferred initial treatment for FBSG, with chemotherapy used for persistent, recurrent, or inoperable disease; conversely, radiation is the only known effective treatment for DIPG...
March 2015: Current Oncology Reports
Amar Gajjar, Stefan M Pfister, Michael D Taylor, Richard J Gilbertson
High-throughput genomic technologies have shed light on the biologic heterogeneity of several pediatric brain tumors. The biology of the four common pediatric brain tumors-namely medulloblastoma; ependymoma; high-grade glioma (HGG), including diffuse intrinsic pontine glioma; and low-grade glioma-is highlighted in this CCR Focus article. The discovery that medulloblastoma consists of four different subgroups, namely WNT, SHH, Group 3, and Group 4, each with distinct clinical and molecular features, has affected the treatment of children with medulloblastoma...
November 15, 2014: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
Jayant S Goda, Debnarayan Dutta, Nirmal Raut, Shashikant L Juvekar, Nilendu Purandare, Venkatesh Rangarajan, Brijesh Arora, Tejpal Gupta, Purna Kurkure, Rakesh Jalali
PURPOSE: To study the impact of multiparametric MRI and (18)F-FDG-PET on the outcome of children with diffuse intrinsic pontine gliomas (DIPG). MATERIALS AND METHODS: Imaging data from a phase-II prospective therapeutic study in children with newly diagnosed DIPG were considered for evaluation. They included baseline MRI with contrast enhancement before treatment. Functional imaging included MR spectroscopy, MR perfusion and FDG-PET studies. All patients (n = 20) had baseline MRI and 11 patients had FDG-PET...
2013: Pediatric Neurosurgery
Sumihito Nobusawa, Junko Hirato, Hideaki Yokoo
Here, we review the recent literature on molecular discoveries in ependymomas and pediatric diffuse gliomas. Ependymomas can now be categorized into three location-related subgroups according to their biological profile: posterior fossa ependymomas, group A (PFA) and B (PFB), and supratentorial ependymomas. Although no recurrently mutated genes were found throughout these groups of ependymomas, PFA exhibited a CpG island methylator phenotype, PFB was associated with extensive chromosomal aberrations, and the C11orf95-RELA fusion gene was frequently observed in supratentorial ependymomas...
October 2014: Brain Tumor Pathology
Tadashi Watanabe, Taku Sato, Yugo Kishida, Eiji Ito, Masahiro Ichikawa, Jun Sakuma, Tetsuya Nagatani, Kiyoshi Saito
OBJECTIVE AND IMPORTANCE: We describe a new endoscopic technique, the dual-endoscopic method, in which two independent endoscopic systems are simultaneously used in the subtemporal space and intraparenchymal field for patients with pontine cystic tumours. Tumours were successfully removed through a sheath placed in the brainstem via the subtemporal keyhole approach, with safety ensured by the second subtemporal endoscope. CLINICAL PRESENTATION: A purely endoscopic subtemporal approach was performed in three cases of pontine cystic tumour, including two low-grade gliomas and a recurrent chordoma...
June 2014: Acta Neurochirurgica
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