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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
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...
October 11, 2016: Neurosurgery
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
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
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
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 histological 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
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
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
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
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
Charles Raybaud
PURPOSE: The aims of this study are to analyze how the nature and the behavior of low-grade glial tumors (LGGT) in children may correlate with the anatomy of the cerebral hemispheres and to evaluate the consequent impact of diffusion tensor imaging (DTI) techniques in the presurgical assessment. METHODS: This is a combined review of a series of 155 cases of LGGT and of the recent literature on the subject. RESULTS: The cases retrieved from our data bank were divided in central hemispheric tumors (basal ganglia and thalami) (36 cases), glioneuronal cortical-based tumors (49 cases), and glial tumors of the cerebral mantle (70 cases)...
October 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Alvaro Lassaletta, Michal Zapotocky, Eric Bouffet, Cynthia Hawkins, Uri Tabori
Hemispheric low-grade gliomas account for the second most common location in pediatric low-grade gliomas (PLGGs) after the cerebellum. The pathological spectrum includes gangliogliomas, dysembryoplastic neuroepithelial tumors (DNETs), diffuse astrocytomas, pilocytic astrocytomas, and pleomorphic xanthoastrocytomas (PXAs), among others. Clinically, hemispheric PLGGs represent a well-recognized cause of intractable epilepsy in children and adolescents. With an excellent long-term outcome, surgery remains the cornerstone and patients with gross total resection typically do not need any further therapies...
October 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Daniel Rusiecki, Boleslaw Lach, Branavan Manoranjan, Adam Fleming, Olufemi Ajani, Sheila K Singh
We report a childhood case of thalamic atypical extraventricular neurocytoma that progressed to highly anaplastic ganglioglioma (GG) after eight years of dormancy following subtotal resection and chemotherapy. The neurocytoma displayed immunoreactivity only for synaptophysin, beta-catenin, S-100 and CD56. The GG acquired strong immunoreactivity for chromogranin, glial fibrillary acidic protein, neuron specific enolase and p53 and showed a very high proliferation rate approaching 50% in some areas. Tumor transformation was associated with overexpression of components of the sonic hedgehog (Shh) and Wnt developmental signaling pathways, which are known to regulate tumor-initiating cells in malignant brain neoplasms...
September 2, 2016: Human Pathology
Louis-Marie Terrier, Luc Bauchet, Valérie Rigau, Aymeric Amelot, Sonia Zouaoui, Isabelle Filipiak, Agnès Caille, Fabien Almairac, Marie-Hélène Aubriot-Lorton, Anne-Marie Bergemer-Fouquet, Eric Bord, Philippe Cornu, Alain Czorny, Phong Dam Hieu, Bertrand Debono, Marie-Bernadette Delisle, Evelyne Emery, Walid Farah, Guillaume Gauchotte, Catherine Godfraind, Jacques Guyotat, Bernard Irthum, Kevin Janot, Pierre-Jean Le Reste, Dominique Liguoro, Hugues Loiseau, Guillaume Lot, Vincent Lubrano, Emmanuel Mandonnet, Philippe Menei, Philippe Metellus, Serge Milin, Bertrand Muckenstrum, Pierre-Hugues Roche, Audrey Rousseau, Emmanuelle Uro-Coste, Anne Vital, Jimmy Voirin, Michel Wager, Marc Zanello, Patrick François, Stéphane Velut, Pascale Varlet, Dominique Figarella-Branger, Johan Pallud, Ilyess Zemmoura
BACKGROUND: Anaplastic gangliogliomas (GGGs) are rare tumors whose natural history is poorly documented. We aimed to define their clinical and imaging features and to identify prognostic factors. METHODS: Consecutive cases of anaplastic GGGs in adults prospectively entered into the French Brain Tumor Database between March 2004 and April 2014 were screened. After diagnosis was confirmed by pathological review, clinical, imaging, therapeutic, and outcome data were collected retrospectively...
August 30, 2016: Neuro-oncology
Marc Zanello, Mélanie Pagès, Alexandre Roux, Sophie Peeters, Edouard Dezamis, Stéphanie Puget, Bertrand Devaux, Christian Sainte-Rose, Michel Zerah, Guillaume Louvel, Sarah N Dumont, Jean-François Meder, Jacques Grill, Gilles Huberfeld, Fabrice Chrétien, Eduardo Parraga, Xavier Sauvageon, Pascale Varlet, Johan Pallud
AIM: Prevalence and predictors of epileptic seizures are unknown in the malignant variant of ganglioglioma. METHODS: In a retrospective exploratory dataset of 18 supratentorial anaplastic World Health Organization grade III gangliogliomas, we studied: (i) the prevalence and predictors of epileptic seizures at diagnosis; (ii) the evolution of seizures during tumor evolution; (iii) seizure control rates and predictors of epilepsy control after oncological treatments...
August 23, 2016: British Journal of Neurosurgery
Ellen Gelpi, Romana Höftberger, Tanja Würger, Johannes Kerschbaumer, Christian F Freyschlag, Tanja Djurdjevic, Johannes A Hainfellner
*Both authors contributed equally.
September 2016: Clinical Neuropathology
Marc Zanello, Mélanie Pages, Arnault Tauziède-Espariat, Raphael Saffroy, Stéphanie Puget, Ludovic Lacroix, Edouard Dezamis, Bertrand Devaux, Fabrice Chrétien, Felipe Andreiuolo, Christian Sainte-Rose, Michel Zerah, Frédéric Dhermain, Sarah Dumont, Guillaume Louvel, Jean-François Meder, Jacques Grill, Christelle Dufour, Johan Pallud, Pascale Varlet
Anaplastic ganglioglioma (AGG) is a rare and malignant variant of ganglioglioma. According to the World Health Organization classification version 2016, their histopathological grading criteria are still ill-defined. The aim of the present study was to assess the clinical, imaging, histopathological, and molecular characteristics and outcomes of AGGs in a large consecutive and retrospective adult and pediatric case series. Eighteen patients with AGGs (13 adults and 5 children) were identified (14 de novo and 4 secondary) from a cohort of 222 gangliogliomas (GG) (8%) treated at our institution between 2000 and 2015...
August 18, 2016: Journal of Neuropathology and Experimental Neurology
Vikas Shashikant Kavishwar, Kirti G Chadha, Shaikhali Moiz Barodawala, Anuradha Krishna Murthy
Ganglioglioma is a slow-growing relatively low-grade mixed glioneuronal tumor with most cases corresponding to the WHO Grade I category. It frequently presents with seizures. The temporal lobe is the most common location followed by frontal, parietal, and occipital lobes. These generally behave in a benign fashion and have a favorable prognosis. We describe a case of a 24-year-old male presenting with convulsions and a calcified parieto-occipital mass. This mass removed from the parietal lobe showed neoplastic glial and dysplastic neuronal tissue amidst extensive areas of calcification and foci of ossification...
July 2016: Indian Journal of Pathology & Microbiology
Seunghyun Lee, Tae Jin Yun, Koung Mi Kang, Jung Hyo Rhim, Chul-Kee Park, Tae Min Kim, Sung-Hye Park, Il Han Kim, Seung Hong Choi
BACKGROUND AND PURPOSE: To evaluate diffusion-weighted imaging (DWI) and dynamic susceptibility contrast perfusion-weighted imaging (DSC-PWI) of ganglioglioma (GG) compared with oligodendroglioma (ODG). MATERIALS AND METHODS: We enrolled 36 patients with histopathologically confirmed GG (12 patients) or ODG (24 patients). The volumetric analyses of normalized ADC (nADC) and normalized CBV (nCBV) maps, tumor volume, and ratio of enhancing portion compared to the tumor volume were performed...
October 2016: Journal of Neuroradiology. Journal de Neuroradiologie
J H Harreld, S N Hwang, I Qaddoumi, R G Tatevossian, X Li, J Dalton, K Haupfear, Y Li, D W Ellison
BACKGROUND AND PURPOSE: Pilocytic astrocytomas, the most common posterior fossa tumors in children, are characterized by KIAA1549-BRAF fusions and shows excellent 5-year survival rates. Pilocytic astrocytoma with gangliocytic differentiation, a recently defined pilocytic astrocytoma variant that includes glial and neuronal elements similar to a ganglioglioma, may be distinguished from a classic ganglioglioma by molecular, radiologic, and histopathologic features. This study investigated whether imaging could distinguish posterior fossa pilocytic astrocytoma with and without gangliocytic differentiation...
July 28, 2016: AJNR. American Journal of Neuroradiology
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