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ganglioglioma

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https://www.readbyqxmd.com/read/29770223/profound-hearing-loss-following-surgery-in-pediatric-patients-with-posterior-fossa-low-grade-glioma
#1
Yahya Ghazwani, Ibrahim Qaddoumi, Johnnie K Bass, Shengjie Wu, Jason Chiang, Frederick Boop, Amar Gajjar, Zsila Sadighi
Background: Hearing loss may occur in patients with posterior fossa low-grade glioma who undergo surgery. Methods: We retrospectively reviewed 217 patients with posterior fossa low-grade glioma, including 115 for whom results of hearing tests performed after surgery and before chemotherapy or radiation therapy were available. We explored the association of UHL with age at diagnosis, sex, race, tumor location, extent of resection, posterior fossa syndrome, ventriculoperitoneal shunt placement, and histology...
May 2018: Neuro-oncology Practice
https://www.readbyqxmd.com/read/29753075/pigmented-ganglioglioma-of-the-cerebellum-case-report-and-review
#2
Mohit Agrawal, Sachin A Borkar, Gaurav Khanna, Mehar C Sharma, Shashank S Kale
BACKGROUND: Gangliogliomas are rare intra-axial tumors. Cerebellar seizures caused by gangliogliomas have been rarely described. Pigmented neural cell tumors are well described in the literature but infrequent, especially when presenting as primary neuroepithelial tumors. Only five cases of pigmented ganglioglioma have been described in literature, out of which 4 were in the pediatric population. CASE DESCRIPTION: A seventeen year old female presented to us with cerebellar seizures, which resolved after tumor excision...
May 9, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29733410/perioperative-multimodal-evaluation-and-surgical-tactics-of-tumor-related-epilepsy-2-dimensional-operative-video
#3
Chengxin Ma, Rui Feng, N U Farrukh Hameed, Jie Zhang, Dongxiao Zhuang, Jinsong Wu
Treatment of tumor-related epilepsy (TRE), especially for tumors near critical areas, requires surgeons to strike a balance between the epileptic benefit and functional outcome after surgery. Here, we present a case in which multimodal evaluation facilitated the achievement of such surgical balance. Informed patient consent was obtained. A 17-yr-old female presented with seizure attacks for 2 yr. Magnetic resonance imaging (MRI) revealed a right parietal mass lesion with hypointense signal on T1W imaging, hyperintense signal on T2W imaging, and homogeneous enhancement...
May 4, 2018: Operative Neurosurgery (Hagerstown, Md.)
https://www.readbyqxmd.com/read/29687738/ganglioglioma-epilepsy-and-intellectual-impairment-due-to-familial-tsc1-deletion
#4
Tal Gilboa, Reeval Segel, Sharon Zeligson, Gheona Alterescu, Hilla Ben-Pazi
BACKGROUND: Tuberous sclerosis complex (TSC) is a multisystem disorder diagnosed by clinical criteria and/or genetic testing. Genetic testing reveals atypical phenotypes that have not met clinical criteria, with practical implications. METHODS: We describe 4 family members with pathogenic partial deletion in TSC1 who individually did not meet tuberous sclerosis complex clinical criteria. RESULTS: Family members had different and atypical findings of tuberous sclerosis complex...
January 1, 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29680921/neurosurgical-treatment-of-gangliogliomas-in-children-and-adolescents-long-term-follow-up-of-a-single-institution-series-of-32-patients
#5
Tryggve Lundar, Bernt Johan Due-Tønnessen, Radek Fric, Arild Egge, Bård Krossnes, Paulina Due-Tønnessen, Einar Stensvold, Petter Brandal
OBJECT: The object of this study was to delineate long-term results of the surgical treatment of pediatric tumors classified as ganglioglioma or gangliocytoma. METHODS: A cohort of consecutive patients 19 years or younger who had undergone primary resection of CNS tumors during the years 1980-2016 at a single institution were reviewed in this retrospective study of surgical morbidity, mortality, and academic achievement and/or work participation. Gross motor function and activities of daily living were scored using the Barthel Index (BI)...
April 22, 2018: Acta Neurochirurgica
https://www.readbyqxmd.com/read/29679497/ewsr1-patz1-gene-fusion-may-define-a-new-glioneuronal-tumor-entity
#6
Aurore Siegfried, Audrey Rousseau, Claude-Alain Maurage, Sarah Pericart, Yvan Nicaise, Frederic Escudie, David Grand, Alix Delrieu, Anne Gomez-Brouchet, Sophie Le Guellec, Camille Franchet, Sergio Boetto, Matthieu Vinchon, Jean-Christophe Sol, Franck-Emmanuel Roux, Valerie Rigau, Anne-Isabelle Bertozzi, David Tw Jones, Dominique Figarella-Branger, Emmanuelle Uro-Coste
We investigated the challenging diagnostic case of a ventricular cystic glioneuronal tumor with papillary features, by RNA sequencing using the Illumina TruSight RNA Fusion panel. We did not retrieve the SLC44A1-PRKCA fusion gene specific for papillary glioneuronal tumor, but an EWSR1-PATZ1 fusion transcript. RT-PCR followed by Sanger sequencing confirmed the EWSR1-PATZ1 fusion. It matched with canonic EWSR1 fusion oncogene, juxtaposing the entire N terminal transcriptional activation domain of EWSR1 gene and the C terminal DNA binding domain of a transcription factor gene, PATZ1...
April 21, 2018: Brain Pathology
https://www.readbyqxmd.com/read/29677585/age-at-epilepsy-onset-in-patients-with-focal-cortical-dysplasias-gangliogliomas-and-dysembryoplastic-neuroepithelial-tumours
#7
Attila Rácz, Andreas-Markus Müller, Johannes Schwerdt, Albert Becker, Hartmut Vatter, Christian E Elger
PURPOSE: The age at epilepsy onset in patients with inborn or very early acquired brain lesions depends on the epileptogenic potential of the lesion and the patients' individual "susceptibility" to epileptic seizures. To gain insight into these determinants, we analysed the case history of patients with focal cortical dysplasias (FCDs) and neuroglial tumours. METHODS: In a systematic, retrospective analysis comprised of 233 patients who underwent surgery (116 with FCDs and 117 with neuroglial tumours), we evaluated the age at epilepsy onset according to histopathologic subgroups, lesion location and family history...
April 4, 2018: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/29675936/pediatric-ganglioglioma-with-an-h3-k27m-mutation-arising-from-the-cervical-spinal-cord
#8
Tomohiro Okuda, Nobuhiro Hata, Satoshi O Suzuki, Koji Yoshimoto, Koichi Arimura, Takeo Amemiya, Yojiro Akagi, Daisuke Kuga, Utako Oba, Yuhki Koga, Shouichi Ohga, Toru Iwaki, Koji Iihara
The 2016 edition of the World Health Organization Classification of Tumors of the Central Nervous System introduced "diffuse midline glioma H3 K27M mutant" as a new diagnostic entity. These tumors predominately affect pediatric patients and arise from midline structures such as the brainstem, thalamus and spinal cord. Here, we report a rare patient with spinal ganglioglioma carrying an H3 K27M mutation. A 10-year-old boy presented with an intramedullary tumor in the cervical spinal cord. The lesion was partially removed and histologically diagnosed as ganglioglioma...
April 19, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29594461/impact-of-surgery-adjuvant-treatment-and-other-prognostic-factors-in-the-management-of-anaplastic-ganglioglioma
#9
Supriya Mallick, Rony Benson, Wineeta Melgandi, Prashanth Giridhar, G K Rath
BACKGROUND/PURPOSE: Anaplastic ganglioglioma (AGG) is a rare tumor with both glial and neuronal component accounting for less than 1% of all CNS tumors with limited information about the optimum treatment and outcome of these tumors. METHOD AND MATERIALS: We did a thorough search of the PubMed with the following MesH terms: "Ganglioglioma; Anaplastic ganglioglioma; Ganglioglioma AND treatment; and Anaplastic ganglioglioma AND survival" to find all possible publications related to AGG to perform an individual patient data analysis and derive the survival outcome and optimum treatment of these tumors...
March 29, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29527389/a-case-of-adult-anaplastic-cerebellar-ganglioglioma
#10
Sofiene Bouali, Nidhal Maatar, Alia Zehani, Maha Mahmoud, Jalel Kallel, Hafedh Jemel
Background: Anaplastic posterior fossa ganglioglioma in adults is exceedingly rare. To date, only one case of adult anaplastic posterior fossa ganglioglioma has been reported in the English literature and none has been described at the cerebellum. To our knowledge, this report is the third case of malignant posterior fossa ganglioglioma in adults and the first at the cerebellum. In general, this entity can be misdiagnosed preoperatively as a primary posterior fossa neoplasm, and by reporting our clinical and radiographic observations we want to add to the existing literature on this rare entity...
2018: Surgical Neurology International
https://www.readbyqxmd.com/read/29524710/technical-modification-of-amygdalo-hippocampectomy-in-temporal-lobe-epilepsy-surgery-to-further-reduce-severe-neurological-complications-a-clinical-anatomical-study
#11
Karl Roessler, Burkhard S Kasper, Roland Coras, Soheil Arinrad, Michael Scholz, Hajo H Hamer, Ingmar Blümcke, Michael Buchfelder
BACKGROUND: Temporal lobe resection (TLR) including amygdalohippocampectomy (AHE) is the most frequent performed procedure in epilepsy surgery. Owing to the close anatomic relationship of the mesial temporal structures and the midbrain and choroidal fissure, the incidence of severe complications, such as postoperative stroke, is as high as 2.5%. METHODS: We developed a modification of the classical technique for AHE that involves entering the choroidal fissure early via the anterior part of the frontomesial temporal horn cleft to identify the crus cerebri, posterior cerebri artery, and oculomotor nerve...
March 7, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29502353/novel-tle4-ntrk2-fusion-in-a-ganglioglioma-identified-by-array-cgh-and-confirmed-by-ngs-potential-for-a-gene-targeted-therapy
#12
Nitya Prabhakaran, Miguel A Guzman, Pournima Navalkele, Edna Chow-Maneval, Jacqueline R Batanian
Gangliogliomas are rare neoplasms of the central nervous system that mostly originate in the temporal lobe and are associated with seizures. Literature mentions that BRAF mutations are most commonly associated with gangliogliomas. We discuss a unique case of ganglioglioma originating in the posterior fossa that showed multiple losses and a unique interstitial deletion at 9q21 by an array-comparative genome hybridization (array-CGH). The deletion led to a novel molecular fusion (TLE4-NTRK2) which was confirmed by next generation sequencing and provides a potential for a gene-targeted therapy...
March 4, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29502115/anaplastic-ganglioglioma-involving-the-entire-length-of-the-spinal-cord
#13
Bruno Niemeyer, Edson Marchiori
No abstract text is available yet for this article.
March 2, 2018: European Neurology
https://www.readbyqxmd.com/read/29498601/edema-of-the-optic-tract-in-patients-with-tumors-of-the-sellar-region-clinical-and-visual-implications-in-the-pediatric-population
#14
Aurore Bussat, Maia Proisy, Bertrand Bruneau, Guillaume Bouzillé, Céline Chappé, Laurent Riffaud
OBJECTIVE Tumor-related edema of the optic tract (EOT) corresponds to a preferential posterior distribution of peritumoral edema along the white matter tract of the visual system. To date, the consequences of EOT have never been evaluated specifically in the pediatric population. In this study, the authors attempted to identify clinical and radiological features associated with the development of EOT and the specific influence of this edema on visual function. METHODS A retrospective review was performed of data collected from patients younger than 18 years who underwent surgery for a tumor in the sellar region at the authors' institution between January 2005 and January 2016...
May 2018: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29492135/intractable-yawning-as-a-predominant-symptom-of-temporal-lobe-ganglioglioma-case-report-and-review-of-literature
#15
Raja K Kutty, Jacob Paul Alapatt, Aparna Govindan
Yawning, a physiologic reflex exhibited by vertebrates, is seldom noticed as a symptom of a disease. Not too often is a patient aware of it as a symptom, unless it is of such a distressing nature to seek attention. In this situation, to distinguish between normal and abnormal behavior would pose a diagnostic dilemma for the attending physician. Intractable yawning has been a presenting symptom of many pathologic states such as stroke, epilepsy, and migraine. Literature is sparse regarding intractable yawning caused by tumors of the brain...
January 2018: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/29487755/suprasellar-ganglioglioma-expanding-the-differential-diagnosis
#16
Isabella Tondi Resta, Arminder Singh, Bruce C Gilbert, Mumtaz V Rojiani, Cargill Alleyne, Amyn M Rojiani
This case study describes a young man with symptoms suggestive of the presence of a space-occupying lesion within the cranial cavity. Imaging studies confirmed a lesion in the suprasellar region and surgical intervention to remove the tumor yielded an unexpected diagnosis. Neuroimaging characteristics and histopathology including immunohistochemistry are described. Gangliogliomas are uncommon CNS neoplasms and are most commonly found in the temporal and frontal lobes of young, male adults. They are rarely seen in the suprasellar region and only a handful of cases have been reported to date...
2018: Case Reports in Pathology
https://www.readbyqxmd.com/read/29434497/cerebellar-ganglioglioma-in-childhood-histopathologic-implications-for-management-during-long-term-survival-a-case-report
#17
Koyo Ohno, Yoshiaki Saito, Akiko Tamasaki-Kondo, Atsushi Kambe, Yasushi Horie, Shinsuke Kato, Yoshihiro Maegaki
We report the case of a 19-year-old female with cerebellar ganglioglioma that was diagnosed at 4 years of age. Despite treatment with partial resection, radiation, and chemotherapy, residual tumor slowly expanded into the brainstem and upper cervical cord, resulting in nocturnal hypopnea, progressive tetraparesis, and feeding difficulty during 8-10 years of age. Initiation of temozolomide and bevacizumab was effective in preventing further expansion of the tumor, and the patient has been treated at home and in school with noninvasive positive pressure ventilation and gastrostomy...
December 2017: Yonago Acta Medica
https://www.readbyqxmd.com/read/29434027/genome-sequencing-identifies-somatic-braf-duplication-c-1794_1796duptac-p-thr599dup-in-pediatric-patient-with-low-grade-ganglioglioma
#18
Katherine E Miller, Benjamin Kelly, James Fitch, Nicole Ross, Matthew R Avenarius, Elizabeth Varga, Daniel C Koboldt, Daniel R Boué, Vincent Magrini, Scott L Coven, Jonathan L Finlay, Catherine E Cottrell, Peter White, Julie M Gastier-Foster, Richard K Wilson, Jeffrey Leonard, Elaine R Mardis
Gangliogliomas (WHO grade I) are rare tumors affecting the central nervous system and are most frequently observed in children. Next-generation sequencing of tumors is being utilized at an increasing rate in both research and clinical settings to characterize the genetic factors that drive tumorigenesis. Here, we report a rare BRAF somatic mutation (NM_004333.4:c.1794_1796dupTAC; p.Thr599dup) in the tumor genome from a pediatric patient in her late teens, who was initially diagnosed with low-grade ganglioglioma at age 13...
April 2018: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/29393845/h3-k27m-mutant-gliomas-in-adults-vs-children-share-similar-histological-features-and-adverse-prognosis
#19
Bette K Kleinschmidt-DeMasters, Jean M Mulcahy Levy
BACKGROUND: H3 K27M mutation was originally described in pediatric diffuse intrinsic pontine gliomas (DIPGs), but has been recently recognized to occur also in adult midline diffuse gliomas, as well as midline tumors with other morphologies, including gangliogliomas (GGs), anaplastic GGs, pilocytic astrocytomas (PAs), and posterior fossa ependymomas. In a few patients with H3 K27M;mutant tumors with these alternate morphologies, longer survival has been reported, making grading difficult for the neuropathologist...
March 2018: Clinical Neuropathology
https://www.readbyqxmd.com/read/29380516/response-to-the-braf-mek-inhibitors-dabrafenib-trametinib-in-an-adolescent-with-a-braf-v600e-mutated-anaplastic-ganglioglioma-intolerant-to-vemurafenib
#20
Asher M Marks, Ranjit S Bindra, Michael L DiLuna, Anita Huttner, Vikram Jairam, Kristopher T Kahle, Mark W Kieran
Efficacy of BRAF V600E targeted therapies in brain tumors harboring the mutation has been shown in several case reports and is currently being studied in larger clinical trials. Monotherapy with vemurafenib has been associated with significant side effects, including rashes, papillomas, and squamous cell carcinomas. Here we describe an adolescent female with anaplastic ganglioglioma and significant skin reaction to vemurafenib with subsequent tumor response and tolerance to the BRAF/MEK inhibitor combination of dabrafenib and trametinib without recurrence of previous reaction...
May 2018: Pediatric Blood & Cancer
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