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gliomatosis cerebri pediatric

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...
July 28, 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
Patrick B Senatus, Shearwood McClelland, Kurenai Tanji, Alexander Khandji, Judy Huang, Neil Feldstein
Cerebellar glioblastoma multiforme (GBM) is a rare entity in adults and an extremely rare entity in children. Approximately 30 cases have been reported in the literature. The authors report the case of a histologically confirmed cerebellar GBM presenting initially as supra- and infratentorial gliomatosis cerebri. Acute disseminated encephalomyelitis had been diagnosed in the patient and that diagnosis remained until near the end of his treatment. This case underscores the need for recognizing the clinical presentation of gliomatosis cerebri and multifocal GBM in the pediatric subpopulation thought to harbor demyelinating disease...
January 2005: Journal of Neurosurgery
Rosario Maria S Riel-Romero, Robert J Baumann, Charles D Smith
Childhood cancer is a leading cause of mortality in children less than 15 years of age, accounting for about 10.4 of total childhood deaths [Robinson LL: In: Pizzo PA, Polack DA (eds) Principles and Practice of Pediatric Oncology, 3rd edn. Lippincott--Raven, Philadelphia--NewYork, 1997, pp. 1-10.]. As more aggressive therapeutic regimens have been adopted and ostensibly cured patients are being followed for longer periods of time, it has become increasingly clear that the treatment of cancer can have significant late effects on the growing child, one of the more troublesome of which is the induction of secondary malignancy...
July 2005: Journal of Neuro-oncology
Martin Benesch, Sabine Wagner, Frank Berthold, Johannes E A Wolff
PURPOSE: Clinical data on central nervous system (CNS) dissemination of high-grade gliomas (HGG) at initial presentation in children are rare. PATIENTS AND METHODS: We conducted a retrospective data analysis of all patients enrolled into four consecutive HGG protocols of the Pediatric Oncology and Hematology Society of the German Language Group (GPOH) to determine the incidence of primary CNS dissemination of HGG and to describe clinical characteristics and outcome of children with HGG who were diagnosed with CNS dissemination at initial presentation...
April 2005: Journal of Neuro-oncology
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