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https://www.readbyqxmd.com/read/27921213/pediatric-intracranial-primary-anaplastic-ganglioglioma
#1
Wolf Lüdemann, Rouzbeh Banan, Christian Hartmann, Helmut Bertalanffy, Concezio Di Rocco
BACKGROUND: Primary intracranial anaplastic gangliogliomas are rare tumors in the pediatric patient group. Most of them present with symptoms of elevated pressure or symptomatic epilepsy. Extraaxial location is far more common than axial location. On MRI examination, they mimic pilocytic astrocytomas. The outcome after surgery depends mainly on the possible amount of surgical resection, and oncological therapy is necessary to prevent recurrence of the disease. CASE REPORT: An 11-year-old boy presented with headache and double vision due to obstructive hydrocephalus...
December 5, 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27910945/thyroid-transcription-factor-1-distinguishes-subependymal-giant-cell-astrocytoma-from-its-mimics-and-supports-its-cell-origin-from-the-progenitor-cells-in-the-medial-ganglionic-eminence
#2
Jen-Fan Hang, Chih-Yi Hsu, Shih-Chieh Lin, Chih-Chun Wu, Han-Jui Lee, Donald Ming-Tak Ho
Subependymal giant cell astrocytoma is a benign brain tumor mostly associated with tuberous sclerosis complex. However, it may be misinterpreted as other high-grade brain tumors due to the presence of large tumor cells with conspicuous pleomorphism and occasional atypical features, such as tumor necrosis and endothelial proliferation. In this study, we first investigated thyroid transcription factor-1 (TTF-1) expression in a large series of subependymal giant cell astrocytomas and other histologic and locational mimics to validate the diagnostic utility of this marker...
December 2, 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/27860162/desmoplastic-infantile-astrocytoma-ganglioglioma-with-rare-braf-v600d-mutation
#3
Ashley Greer, Nicholas K Foreman, Andrew Donson, Kurtis D Davies, B K Kleinschmidt-DeMasters
BACKGROUND: Desmoplastic infantile astrocytoma (DIA) and desmoplastic infantile gangliogliomas (DIGs) are rare, massive, cystic and solid tumors of infants usually found in superficial cerebral hemispheres. They manifest prominent desmoplastic stroma, admixed neoplastic astrocytes, primitive-appearing small cells, and additional neoplastic ganglion cells in the case of DIGs. While v-Raf murine sarcoma viral oncogene homolog B (BRAF) mutation is found in up to 50% of pediatric gangliogliomas, two recent studies found that it was rare in DIA/DIGs; we sought to assess BRAF status in DIA/DIGs from our institution...
November 10, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27853959/epidemiology-for-primary-brain-tumors-a-nationwide-population-based-study
#4
Amélie Darlix, Sonia Zouaoui, Valérie Rigau, Faiza Bessaoud, Dominique Figarella-Branger, Hélène Mathieu-Daudé, Brigitte Trétarre, Fabienne Bauchet, Hugues Duffau, Luc Taillandier, Luc Bauchet
Primary central nervous system tumors (PCNST) are rare tumors responsible for high mortality and morbidity. Their epidemiology is poorly known, and clinical data are scarcely analyzed at a national level. In this study, we aimed at providing descriptive epidemiological data and incidence rates for all histological subtypes of PCNST according to the WHO classification. We conducted a nationwide population-based study of all newly diagnosed and histologically confirmed PCNST in France, between 2006 and 2011. A total of 57,816 patients were included: male 46...
November 16, 2016: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/27846400/spinal-cordectomy-a-new-hope-for-morbid-spinal-conditions
#5
REVIEW
Subhas K Konar, Tanmoy K Maiti, Shyamal C Bir, Anil Nanda
A spinal cordectomy is a treatment option for several disorders of the spinal cord like post-traumatic syringomyelia, spinal cord tumor and myelomeningocele. We have done a systematic analysis of all reported cases of spinal cordectomy to investigate the possible outcomes and complications. A PubMed search was performed for literature published from 1949 to 2015 with search words "spinal cordectomy", "spinal cord transection" and "cordectomy for malignant spinal cord tumors" to select articles containing information about the indication, outcome and complication of spinal cordectomy performed for diverse etiologies...
November 10, 2016: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/27823711/central-sleep-apnea-in-children-experience-at-a-single-center
#6
Orlane Felix, Alessandro Amaddeo, Jorge Olmo Arroyo, Michel Zerah, Stephanie Puget, Valerie Cormier-Daire, Genevieve Baujat, Graziella Pinto, Marta Fernandez-Bolanos, Brigitte Fauroux
OBJECTIVE: Central sleep apnea (CSA) syndromes are rare in children and data in children over one year of age are scarce. The aim of the study was to describe the sleep characteristics, underlying disorders, management, and outcome of children with CSA. PATIENTS/METHODS: A retrospective chart review of all children >1 year of age, diagnosed with CSA on a laboratory sleep study during a 20-month period, was performed. CSA was defined by a central apnea index (CAI) >5 events/h...
September 2016: Sleep Medicine
https://www.readbyqxmd.com/read/27799506/personalized-treatment-for-a-patient-with-a-braf-v600e-mutation-using-dabrafenib-and-a-tumor-treatment-fields-device-in-a-high-grade-glioma-arising-from-ganglioglioma
#7
Silviya K Meletath, Dean Pavlick, Tim Brennan, Roy Hamilton, Juliann Chmielecki, Julia A Elvin, Norma Palma, Jeffrey S Ross, Vincent A Miller, Philip J Stephens, George Snipes, Veena Rajaram, Siraj M Ali, Isaac Melguizo-Gavilanes
BACKGROUND: Gangliogliomas are slow-growing, low-grade central nervous system tumors affecting children and young adults. However, some patients will experience tumor recurrence and/or malignant progression. This article reports on the clinical history, molecular findings, and treatment response in a patient with BRAF V600-mutated high-grade glioma arising from ganglioglioma. METHODS: Hematoxylin-eosin staining and comprehensive genomic profiling via Foundation One were performed on the tumor sample from a male patient undergoing treatment at the Department of Neuro-Oncology at Baylor University Medical Center...
November 2016: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/27792249/a-comprehensive-analysis-identifies-braf-hotspot-mutations-associated-with-gliomas-with-peculiar-epithelial-morphology
#8
Ryusuke Hatae, Nobuhiro Hata, Satoshi O Suzuki, Koji Yoshimoto, Daisuke Kuga, Hideki Murata, Yojiro Akagi, Yuhei Sangatsuda, Toru Iwaki, Masahiro Mizoguchi, Koji Iihara
Brain tumors harbor various BRAF alterations, the vast majority of which are the BRAF kinase-activating V600E mutation. BRAF mutations are most frequently detected in certain subtypes of low-grade glioma, such as pilocytic astrocytoma (PA), pleomorphic xanthoastrocytoma (PXA), ganglioglioma (GG) and dysembryoplastic neuroepithelial tumor (DNT). However, it is unclear whether gliomas harboring BRAF mutations can be invariably regarded as these glioma subtypes or their derivatives. To address this question, we analyzed 274 gliomas in our institutional case series...
October 28, 2016: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/27791984/droplet-digital-pcr-is-a-powerful-technique-to-demonstrate-frequent-fgfr1-duplication-in-dysembryoplastic-neuroepithelial-tumors
#9
Frédéric Fina, Doriane Barets, Carole Colin, Corinne Bouvier, Laëtitia Padovani, Isabelle Nanni-Metellus, L'Houcine Ouafik, Didier Scavarda, Andrey Korshunov, David T W Jones, Dominique Figarella-Branger
Dysembryoplastic neuroepithelial tumors (DNT) share V600E mutation in the BRAF gene with other low grade neuroepithelial tumors (LGNTs). FGFR1 internal tandem duplication of the tyrosine-kinase domain (FGFR1-ITD), another genetic alteration that also leads to MAP kinase pathway alteration, has been previously reported in LGNTs by whole-genome sequencing. In the present study we searched for FGFR1-ITD by droplet digital PCR (DDPCR™) and for FGFR1 point mutations by HRM-sequencing in a series of formalin-fixed paraffin-embedded (FFPE) LGNTs including 12 DNT, 2 oligodendrogliomas lacking IDH mutation and 1p/19q co- deletion (pediatric-type oligodendrogliomas; PTOs), 3 pediatric diffuse astrocytomas (PDAs), 14 gangliogliomas (GGs) and 5 pilocytic astrocytomas (PAs)...
October 25, 2016: Oncotarget
https://www.readbyqxmd.com/read/27771109/cerebellopontine-angle-gangliogliomas-report-of-two-cases
#10
S Boissonneau, L-M Terrier, S De La Rosa Morilla, L Troude, J P Lavieille, P-H Roche
BACKGROUND: Gangliogliomas are rare tumors of the central nervous system. We report two unusual cases of gangliogliomas located in the cerebellopontine angle (CPA). POPULATION AND METHODS: The first patient was a 57-year-old woman, who presented with dizziness and harbored a non-enhanced heterogeneous mass located in the cisternal space of the CPA. A partial microsurgical removal was performed, and the pathological examination concluded a grade I ganglioglioma according to the WHO Classification...
October 19, 2016: Neuro-Chirurgie
https://www.readbyqxmd.com/read/27761184/diffusion-perfusion-and-histopathologic-characteristics-of-desmoplastic-infantile-ganglioglioma
#11
Chang Y Ho, Melissa Gener, Jose Bonnin, Stephen F Kralik
We present a case series of a rare tumor, the desmoplastic infantile ganglioglioma (DIG) with MRI diffusion and perfusion imaging quantification as well as histopathologic characterization. Four cases with pathologically-proven DIG had diffusion weighted imaging (DWI) and two of the four had dynamic susceptibility contrast imaging. All four tumors demonstrate DWI findings compatible with low-grade pediatric tumors. For the two cases with perfusion imaging, a higher relative cerebral blood volume was associated with higher proliferation index on histopathology for one of the cases...
July 2016: Journal of Radiology Case Reports
https://www.readbyqxmd.com/read/27741220/intraoperative-near-infrared-optical-imaging-can-localize-gadolinium-enhancing-gliomas-during-surgery
#12
John Y K Lee, Jayesh P Thawani, John Pierce, Ryan Zeh, Maria Martinez-Lage, Michelle Chanin, Ollin Venegas, Sarah Nims, Kim Learned, Jane Keating, Sunil Singhal
BACKGROUND: Although real-time localization of gliomas has improved with intraoperative image guidance systems, these tools are limited by brain shift, surgical cavity deformation, and expense. OBJECTIVE: To propose a novel method to perform near-infrared (NIR) imaging during glioma resections based on preclinical and clinical investigations, in order to localize tumors and to potentially identify residual disease. METHODS: Fifteen patients were identified and administered a Food and Drug Administration-approved, NIR contrast agent (Second Window indocyanine green [ICG], 5 mg/kg) before surgical resection...
December 2016: Neurosurgery
https://www.readbyqxmd.com/read/27718322/longitudinal-mutational-analysis-of-a-cerebellar-pilocytic-astrocytoma-recurring-as-a-ganglioglioma
#13
Pierre O Fiset, Adam M Fontebasso, Nicolas De Jay, Tenzin Gayden, Hamid Nikbakht, Jacek Majewski, Nada Jabado, Steffen Albrecht
A cerebellar pilocytic astrocytoma (PA) in a child recurred first with a PA histology and then with features of a ganglioglioma (GG). Molecular genetic analyses of the tumors confirmed a BRAF V600E mutation in all. They also all harbored a T202M mutation in ERK1, a kinase downstream of BRAF that is implicated in glial versus neuronal differentiation. The GG sample contained several variants that were not present in the PA samples; in particular, it had a truncating mutation in MAP2. These findings not only underscore the role of BRAF as oncogenic driver but also suggest that other genes may influence tumor morphology...
October 8, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27695570/ganglioglioma-of-conus-medullaris-in-a-patient-of-neurofibromatosis-type-1-a-novel-association
#14
Ilangovan Vijay Sundar, Manish Jaiswal, Devendra Purohit, R S Mittal
Ganglioglioma of the conus region is quite rare with only 12 reported cases. Ganglioglioma shares biologic features with neurofibromatosis leading to suggestions that the co-existence of the two diseases may be more than coincidental. We report a case of ganglioglioma of the conus medullaris in a patient of neurofibromatosis and explore the possible association of the two diseases.
October 2016: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/27695546/ganglioglioma-single-institutional-experience-of-24-cases-with-review-of-literature
#15
Vivek Tandon, Sumit Bansal, P Sarat Chandra, Ashish Suri, Manjari Tripathi, Mehar C Sharma, Avijit Sarkari, Ashok K Mahapatra
BACKGROUND: Ganglioglioma is a common seizure associated tumor. The goal of this study was to observe the postoperative outcome in patients with gangliogliomas. MATERIAL AND METHODS: A total 24 patients with gangliogliomas who underwent surgery at our institute from 2008 to 2011 were included. There were 13 males (54%) in our study. A retrospective analysis for the demographic profile, surgery and outcome was performed using STATA software. Literature on this subject was also reviewed, MEDLINE and PUBMED databases were searched...
October 2016: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/27671879/genetic-characterization-of-a-multifocal-ganglioglioma-originating-within-the-spinal-cord
#16
Joshua L Wang, Christopher S Hong, Jose Otero, Vinay K Puduvalli, J Bradley Elder
BACKGROUND: Gangliogliomas are rare, low-grade intra-axial tumors that exhibit both neuronal and glial components. Although the vast majority present as an intracranial lesion, a rare subset exist as isolated lesions of the spinal cord. Gangliogliomas have also been shown to harbor mutations in the p53 tumor suppressor gene and BRAF oncogene. Previous studies in ganglioglioma have correlated p53 mutations with histologic transformation and BRAF mutations with worse prognosis. CASE DESCRIPTION: In this report, we describe a 35-year-old female who presented with multifocal ganglioglioma, involving both the conus medullaris and filum terminale...
September 23, 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27668845/desmoplastic-infantile-astrocytoma-and-ganglioglioma-case-report-and-review-of-the-literature
#17
Alaa Samkari, Faris Alzahrani, Abeer Almehdar, Hussein Algahtani
Desmoplastic infantile astrocytoma (DIA) is a rare, supratentorial, dural-based, large cystic tumor that usually arises in the first 24 months of life. However, non-infantile cases were also reported in the literature. Desmoplastic infantile astrocytoma and desmoplastic infantile ganglioglioma (DIG) are both classified as grade I astrocytoma by the World Health Organization (WHO). Grossly, DIA/DIG are large tumors composed of solid and cystic portions. Although large in nature, they are slow-growing tumors, with good prognosis after complete surgical removal, and rarely require a chemotherapy or radiotherapy...
September 26, 2016: Clinical Neuropathology
https://www.readbyqxmd.com/read/27659837/mri-guided-laser-interstitial-thermal-therapy-for-the-treatment-of-low-grade-gliomas-in-children-a-case-series-review-description-of-the-current-technologies-and-perspectives
#18
Zulma Tovar-Spinoza, Hoon Choi
BACKGROUND: Pediatric low-grade gliomas (LGGs) account for approximately half of all pediatric central nervous system tumors. The low-grade gliomas' first line of treatment is gross total resection. However, when gross total resection is not possible, options for adjuvant therapy are limited. MRI-guided laser ablation (magnetic resonance-guided laser interstitial thermal therapy (MRgLITT)) offers a new option for treatment in selected cases. We present a description of the current MRgLITT technology and an exemplary case-series review of our experience in its use in LGGs...
October 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27659827/glioneuronal-tumors-of-cerebral-hemisphere-in-children-correlation-of-surgical-resection-with-seizure-outcomes-and-tumor-recurrences
#19
Tadanori Tomita, Jerome M Volk, Wenjun Shen, Tatiana Pundy
OBJECT: Glioneuronal tumors are common neoplasms among the cerebral hemisphere during childhood. They consist of several histological types, of which gangliogliomas (GGs) and dysembryoplastic neuroepithelial tumors (DNTs) are most common and often present with seizures. A great majority of glioneuronal tumors are benign. However, there are conflict reports regarding postoperative tumor recurrence rates and seizure control. The authors analyzed and compared these tumors for their locations and histology and the tumor and seizure control following resection...
October 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27659826/supratentorial-tumors-typical-of-the-infantile-age-desmoplastic-infantile-ganglioglioma-dig-and-astrocytoma-dia-a-review
#20
F Bianchi, G Tamburrini, L Massimi, M Caldarelli
OBJECT: Desmoplastic infantile gangliogliomas (DIGs) and desmoplastic infantile astrocytomas (DIAs) are tumors typical of the infantile age. A large size, with a mixed solid and cystic component, clinical presentation with progressing signs of increased intracranial pressure, a prominent benign desmoplastic structure at histological examination, and a favorable clinical course in the majority of cases are the prominent features of these tumors. The objective of the present paper was to review the pertinent literature on the topic together with our personal experience, with the aim of an updated review of the subject...
October 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
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