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gliomatosis cerebri pediatric

Olivia Laugier, Laetitia Padovani, Arnauld Verschuur, Caroline Gaudy-Marqueste, Nicolas André
Glioblastoma cerebri is a rare paediatric malignancy with dismal prognosis [Chappé C, Riffaud L, and Tréguier C et al (2013) Primary gliomatosis cerebri involving gray matter in pediatrics: a distinct entity? A multicenter study of 14 casesChilds Nerv Syst29 565-571 PMID: 23306961] and no established standard of care. Here, we report a case of ulcerated and bleeding striae distensae in a teenage girl following palliative treatment with bevacizumab and steroids.
2017: Ecancermedicalscience
Elena Blanco-Iglesias, Beatriz Cabeza, Elena Garcia Esparza, Inmaculada de Prada, Alvaro Lassaletta, Marcelo Budke, Ana Serrano
Gliomatosis cerebri is a diffuse infiltrating glioma of neuroepithelial origin that affects more than 2 cerebral lobes. This is rarely seen in pediatric patients. The clinical presentation and imaging are very unspecific, and a biopsy is typically needed for the diagnosis. Given the widespread nature of the disease, surgical treatment is not possible and has a poor overall prognosis. A pediatric patient presented with elevated intracranial pressure. All initial studies were negative, and the imaging showed a symmetrical affection involving the supratentorial and infratentorial regions...
May 8, 2017: Pediatric Emergency Care
Koji Yoshimoto, Ryusuke Hatae, Yuhei Sangatsuda, Satoshi O Suzuki, Nobuhiro Hata, Yojiro Akagi, Daisuke Kuga, Murata Hideki, Koji Yamashita, Osamu Togao, Akio Hiwatashi, Toru Iwaki, Masahiro Mizoguchi, Koji Iihara
A recurrent glycine-to-arginine/valine alteration at codon 34 (G34R/V) within H3F3A, a gene that encodes the replication-independent histone variant H3.3, reportedly occurs exclusively in pediatric glioblastomas. However, the clinicopathological and biological significances of this mutation have not been completely elucidated; especially, no such data exist for tumor samples from Japanese patients. We analyzed 411 consecutive glioma cases representing patients of all ages. Our results demonstrated that 14 patients (3...
July 2017: Brain Tumor Pathology
Jeffrey P Greenfield, Alicia Castañeda Heredia, Emilie George, Mark W Kieran, Andres Morales La Madrid
Gliomatosis cerebri (GC) is a universally fatal extensive and diffuse infiltration of brain parenchyma by a glial tumor. Many aspects of this phenomenon remain unknown. The First International Gliomatosis cerebri Group Meeting had the following goals: refine the clinical and radiologic diagnostic criteria for GC, suggest appropriate diagnostic procedures, standardize tissue manipulation for histologic and molecular characterization, and prioritize relevant preclinical projects. Also, general treatment recommendations were outlined for the pediatric population...
December 2016: Pediatric Blood & Cancer
Aurélia Nguyen, Gaelle Bougeard, Meriam Koob, Marie Pierre Chenard, Anne Schneider, Christine Maugard, Natacha Entz-Werle
The constitutional MisMatch Repair deficiency (CMMRD) syndrome is one of the inherited cancer predisposition syndromes. More than two-third patients belonging to a CMMRD family are diagnosed mainly in the first decade with brain cancers and/or hematological malignancies. This syndrome is due to bi-allelic germline mutations in genes of the MMR pathway (MLH1, MSH2, MSH6 or PMS2). Our family report begins with the index case presenting initially with a medulloblastoma, which was even the two relapses in complete remission, when she was diagnosed for an AML...
October 2016: Familial Cancer
Namit Singhal, Vinay Agarwal
Gliomatosis cerebri (GC) is a diffuse infiltrating glial neoplasm of astrocytic origin. GC in children is rare and difficult to diagnose, often presenting with a variety of signs and symptoms that may mimic myriad conditions. We discuss here the presentation and diagnosis of GC in a child who was initially treated on lines of tubercular encephalitis, with transient clinical relief and disappearance of enhancing component of the disease on magnetic resonance imaging. In this report, we highlight the limitations of clinical presentation and neuroimaging as well as the essential role of histological evaluation for the diagnosis of GC in children...
October 2015: Journal of Pediatric Neurosciences
Alberto Broniscer, Omar Chamdine, Scott Hwang, Tong Lin, Stanley Pounds, Arzu Onar-Thomas, Sheila Shurtleff, Sariah Allen, Amar Gajjar, Paul Northcott, Brent A Orr
Gliomatosis cerebri (GC), a rare and deadly CNS neoplasm characterized by involvement of at least three cerebral lobes, predominantly affects adults. While a few small series have reported its occurrence in children, little is known about the molecular characteristics of pediatric GC. We reviewed clinical, radiological, and histological features of pediatric patients with primary GC treated at our institution over 15 years. Targeted sequencing of mutational hotspots in H3F3A, IDH1/2, and BRAF, and genome-wide analysis of DNA methylation and copy number abnormalities was performed in available tumors...
February 2016: Acta Neuropathologica
Aria Fallah, Alexander G Weil, Samir Sur, Ian Miller, Prasanna Jayakar, Glenn Morrison, Sanjiv Bhatia, John Ragheb
OBJECT: Pediatric brain tumors may be associated with medically intractable epilepsy for which surgery is indicated. The authors sought to evaluate the efficacy of epilepsy surgery for seizure control in pediatric patients with brain tumors. METHODS: The authors performed a retrospective review of consecutive patients undergoing resective epilepsy surgery related to pediatric brain tumors at Miami Children's Hospital between June 1986 and June 2014. Time-to-event analysis for seizure recurrence was performed; an "event" was defined as any seizures that occurred following resective epilepsy surgery, not including seizures and auras in the 1st postoperative week...
December 2015: Journal of Neurosurgery. Pediatrics
Emilie George, Allison Settler, Scott Connors, Jeffrey P Greenfield
Gliomatosis cerebri is a rare glial tumor that carries a poor prognosis. Seen in both adults and children, gliomatosis cerebri appears to differ in these populations as with adult versus pediatric glioblastoma. We present 10 children who either presented to the Weill Cornell Medical College or enrolled in the institution's Gliomatosis Cerebri International Registry alongside a cohort of 89 pediatric patients reported in the literature between 2000 and 2014. Age ranged from 4 months to 21 years, with a male to female ratio of 1...
March 2016: Journal of Child Neurology
Ibrahim Qaddoumi, Mehmet Kocak, Atmaram S Pai Panandiker, Gregory T Armstrong, Cynthia Wetmore, John R Crawford, Tong Lin, James M Boyett, Larry E Kun, Fredrick A Boop, Thomas E Merchant, David W Ellison, Amar Gajjar, Alberto Broniscer
BACKGROUND: Epidermal growth factor receptor is overexpressed in most pediatric high-grade gliomas (HGG). Since erlotinib had shown activity in adults with HGG, we conducted a phase II trial of erlotinib and local radiotherapy (RT) in children with newly diagnosed HGG. METHODS: Following maximum surgical resection, patients between 3 and 21 years with non-metastatic HGG received local RT at 59.4 Gy (54 Gy for spinal tumors and those with ≥70% brain involvement)...
2014: Frontiers in Oncology
Giovanni Morana, Arnoldo Piccardo, Claudia Milanaccio, Matteo Puntoni, Paolo Nozza, Armando Cama, Daniele Zefiro, Massimo Cabria, Andrea Rossi, Maria Luisa Garrè
UNLABELLED: Infiltrative astrocytomas (IAs) represent a group of astrocytic gliomas ranging from low-grade to highly malignant, characterized by diffuse invasion of the brain parenchyma. When compared with their adult counterpart, pediatric IAs may be considered biologically distinct entities; nevertheless, similarly to those in adults they represent a complex oncologic challenge. The aim of this study was to investigate the diagnostic role, clinical contribution, and prognostic value of fused (18)F-3,4-dihydroxyphenylalanine ((18)F-DOPA) PET/MR images in pediatric supratentorial IAs...
May 2014: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
Neggy Rismanchi, John R Crawford
Internuclear ophthalmoplegia (INO) is a rare disorder of conjugate lateral gaze that has been described in a number of neurologic conditions including multiple sclerosis, stroke and less commonly brain tumors. We describe a series of 3 boys (11, 12, 15 years) diagnosed with primary central nervous system tumors (pilomyxoid variant astrocytoma, anaplastic oligoastrocytoma, gliomatosis cerebri) who developed bilateral INO as a manifestation of progressive disease. Time from diagnosis to development of bilateral INO ranged from 13-36 months...
December 2013: Journal of Neuro-oncology
Céline Chappé, Laurent Riffaud, Catherine Tréguier, Béatrice Carsin-Nicol, David Veillard, Dan Cristian Chiforeanu, Jacques Grill, Didier Frappaz, Nicolas André, Fréderic Millot, Matthieu Vinchon, Nicolas Sirvent, Christine Edan
BACKGROUND AND PURPOSE: Gliomatosis cerebri (GC) is a rare neoplasm including a variety of tumors, with extremely variable evolution and heterogeneity of prognosis. It may appear either de novo or after a focal glioma, involve predominantly the white or the gray matter, and concern either pediatric or adult patients. We focused on primary GC involving exclusively gray matter in a pediatric population in order better to define the presentation and outcome of this disease. PATIENTS AND METHODS: We reviewed the databases of seven Departments of Pediatric Oncology to identify pediatric cases of GC between 1990 and 2007...
April 2013: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Paul H Phillips
Although PTC most frequently occurs in obese females of childbearing age, the syndrome occurs in children as well.(3),(5) Prepubertal children with PTC have a lower incidence of obesity compared with adults and there is no sex predilection. The onset of puberty is best defined by the onset of secondary sexual characteristics such has menarche, pubic hair, and breast development. Children with PTC have a higher incidence of associated conditions and cranial nerve deficits compared with adults. Similar to adult patients, children are at risk for the development of permanent visual loss...
2012: International Ophthalmology Clinics
Anna Maria Buccoliero, Francesca Castiglione, Duccio Rossi Degl'Innocenti, Chiara Francesca Gheri, Lorenzo Genitori, Gian Luigi Taddei
Mutations in IDH1 gene are observed in gliomas with significant differences according to histotype, grade, prognosis, and age. We analyzed the IDH1 gene mutations frequency in 42 gliomas from 40 children (14 pilocytic astrocytomas; 3 pilomyxoid astrocytomas; 3 diffuse astrocytomas; 1 gliomatosi cerebri; 8 subependymal giant cell astrocytomas; 2 anaplastic astrocytomas; 9 glioblastomas). No IDH1 mutation was detected. Our results indicate that there is no IDH1 gene involvement in the onset and progression of pediatric astrocytomas...
October 2012: Fetal and Pediatric Pathology
Deepti Narasimhaiah, Catherine Miquel, Elisabeth Verhamme, Paul Desclée, Guy Cosnard, Catherine Godfraind
Recently, mutations in IDH1 and IDH2 have been reported as an early and common genetic alteration in diffuse gliomas, being possibly followed by 1p/19q loss in oligodendrogliomas and TP53 mutations in astrocytomas. Lately, IDH1 mutations have also been identified in adult gliomatosis cerebri (GC). The aim of our study was to test the status of IDH1/2, p53 and of chromosomes 1 and 19 in a series of 12 adult and three pediatric GC. For all tumors, clinico-radiologic characteristics, histopathologic features, status of IDH1/2, p53 and of chromosomes 1 and 19 were evaluated...
February 2012: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Jason F Harrison, Hope T Richard, Ty W Abel, Jeffrey S Sosnowski, Anthony M Martino
Gliomatosis cerebri is an uncommon glial neoplasm that is exceedingly rare in children and difficult to diagnose. The authors describe the presentation and diagnosis of GC in 3 children ages 12, 14, and 16 years. These children exhibited signs and symptoms of increased intracranial pressure as well as other vague or site specific neurological signs. Because clinical presentation, CSF analysis, and neuroimaging were nonspecific, a stereotactic biopsy to obtain tissue for pathological review was ultimately necessary to confirm the diagnosis...
September 2010: Journal of Neurosurgery. Pediatrics
Hope T Richard, Jason F Harrison, Ty W Abel, Paul Maertens, Anthony M Martino, Jeffrey S Sosnowski
Gliomatosis cerebri (GC) is a diffuse infiltrating glial neoplasm of astrocytic origin. GC in children is rare and difficult to diagnose, often presenting with a variety of signs and symptoms that may mimic encephalitis. We discuss here the presentation and diagnosis of GC in 2 children who were initially suspected to have acute disseminating encephalomyelitis. In this report we underscore the limitations of relying on clinical presentation and neuroimaging as well as the essential role of pathologic evaluation for the diagnosis of GC in children...
August 2010: Pediatrics
Lily Pal, Sanjay Behari, Shaleen Kumar, Raj Kumar, S K Shankar, R K Gupta
Gliomatosis cerebri(GC) is a highly aggressive, rare form of neuroepithelial tumor. Due to the diffuse nature of the tumor, its pathological grade is not conclusively established unless a biopsy is carried out from multiple sites. The tumor typically infiltrates the cortex in the subpial plane and also insinuates in the perineuronal space and around the Virchow-Robin spaces with relative preservation of the underlying brain parenchyma. Most of the previously reported cases of GC are astrocytic in nature. In this study, we report 2 pediatric patients of GC with oligodendroglial morphology on histopathological examination...
2008: Pediatric Neurosurgery
Gregory T Armstrong, Peter C Phillips, Lucy B Rorke-Adams, Alexander R Judkins, A Russell Localio, Michael J Fisher
BACKGROUND: Gliomatosis cerebri (GC) is a rare and typically fatal glial neoplasm of the central nervous system. In this report, the authors describe the largest cohort of children to date with GC and explore relations between potential prognostic factors, treatment, and survival. METHODS: Imaging, pathologic, and outcome data were reviewed from 13 patients who were diagnosed with GC and were treated at the Children's Hospital of Philadelphia (CHOP) between 1982 and 2005...
October 1, 2006: Cancer
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