scholarly journals EPCT-20. TECHNICAL FEASIBILITY SODIUM (23NA) MRI OF PEDIATRIC GLIOMAS

2021 ◽  
Vol 23 (Supplement_1) ◽  
pp. i51-i51
Author(s):  
Aashim Bhatia
Keyword(s):  
Pediatric Population ◽  
Pediatric Brain Tumor ◽  
Sodium Concentration ◽  
Adult Brain ◽  
Low Grade ◽  
Technical Feasibility ◽  
Pediatric Glioma ◽  
Tumor Studies ◽  
Immunotherapy Trial ◽  
23Na Mri

Abstract Pediatric glioma response to novel targeted therapy can be heterogeneous on conventional proton (1H) MRI. Sodium concentration, as measured with 23Na MRI in adult brain tumors can provide complementary assessment of tumor proliferation to conventional MRI. However, 23Na MRI pediatric brain tumor studies are lacking. Determine the technical feasibility of performing sodium23Na MRI on pediatric glioma patients. Prospective study of an immunotherapy trial for newly diagnosed and recurrent gliomas (high-grade gliomas, low-grade gliomas, brainstem gliomas) in which participants were imaged with 23Na MRI at 3.0 Tesla. The participants (n=26, 14 males) with median age of 11 years (range = 4–23 years of age) were prospectively evaluated with sodium. 23Na MRI is technically feasible in the pediatric population and can distinguish different types of pediatric gliomas at baseline.

scholarly journals P14.77 Reduced dose craniospinal irradiation (CSI) is feasible for standard risk adult medulloblastoma (MBL) patients similarly to pediatric population

2019 ◽  
Vol 21 (Supplement_3) ◽  
pp. iii85-iii86
Author(s):  
M Massimino ◽  
M Sunyach ◽  
L Gandola ◽  
E Pecori ◽  
F Spreafico ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Pediatric Population ◽  
Pediatric Brain Tumor ◽  
Molecular Classification ◽  
Adult Brain ◽  
Adult Patients ◽  
Current Therapy ◽  
Published Data ◽  
Adult Medulloblastoma ◽  
Standard Risk

Abstract BACKGROUND MBL is the most common malignant pediatric brain tumor but represents 1% of adult brain tumors. Recent molecular classification suggests that MBL is not the same disease in children and adults. For standard risk pediatric medulloblastomas current therapy includes CSI at reduced doses (23.4Gy) associated with chemotherapy. Most adult patients with similar risk factors still receive CSI at 36 Gy±chemotherapy (CT): in the adult series treated according to the HIT protocol (CSI 35.2 Gy + boost to 55.2 Gy to posterior fossa followed in most patients by maintenance CT with lomustine, vincristine and cisplatin) a 73% 5-year PFS was reported {Friedrich, Eur J Cancer 2013}, so far the best published data. Hence retrospective experience of 23.4 Gy together with CT for adult patients in some institutions is worthwhile reporting. MATERIAL AND METHODS We gathered M0 patients, aged over 18 years with medulloblastomas and no/minimal post-surgical residues/no biological negative factor, between 1996–2018 in Centre Léon Bérard of-Lyon and Fondazione IRCCS Istituto Nazionale dei Tumori-Milan. RESULTS Forty-four patients were included, median age 26 (18–48) years,20 females. Median follow-up 90 months(10–227). Thirty-six and 8 received 23.4Gy and 30Gy CSI, respectively, + posterior fossa/tumor bed boost and CT in all: pre-RT (carbo/VPx2 courses or 8 drugs-in-one day x 2 courses (13 patients)) and/or post-RT (carbo/VPx2 courses in 11 and 8 drugs-in-one day x 2 courses in 10; CDDP/VCR/CCNU x 8courses in other 22). The 5/10 year PFS and OS were respectively 80.9±6.5%/76.8±7.4% and 88.4±5.5%/73.1±8.5%. Median progression time was 44 months. Relapses (8) were local (4), local+CSF or spine or bone in one instance each and bone only in one. Among variable considered, higher CSI dose than 23.4 Gy, pre-RT CT did not influence PFS, while females had a trend to better PFES and OS (P=0.07). CONCLUSION These combined series present results comparable to - or even better than- those obtained after high CSI doses highlighting the need for treatment redefinition in adults.

scholarly journals LINC-06. OBSERVATION ONLY IN A PATIENT WITH SUSPECTED LOW GRADE GLIOMA. SHOULD NEUROSURGERY ALWAYS BE THE FIRST STEP IN LOW AND MIDDLE INCOME COUNTRIES?

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii379-iii379
Author(s):  
Carlos Leal - Cavazos ◽  
Jose Arenas-Ruiz ◽  
Oscar Vidal-Gutierrez
Keyword(s):  
Brain Mri ◽  
Internal Capsule ◽  
Pediatric Brain Tumor ◽  
Clinical History ◽  
Low Grade ◽  
Large Tumor ◽  
Middle Income ◽  
Dismal Prognosis ◽  
Close Observation ◽  
The Right

Abstract BACKGROUND Low grade gliomas (LGGs) are the most frequent pediatric brain tumor and they comprise a variety of histologies. Complete surgery is curative but sometimes its location makes it difficult. Recent publications highlight the excellent long-term outcomes of patients with LGGs with complete and incomplete resected tumors. Current strategies are focused on reducing risks of treatment related sequelae. METHOD We describe a patient with a suspected LGG managed by close observation. We describe the case of a 6 year old female with 5 months history of focal onset seizures. During this time a brain MRI was requested and tumor was evidenced. After “tumor diagnosis” was made family visited a handful of private neurosurgeons with a uniformly dismal prognosis and high risk morbidity from procedures offered. When first seen at our Hospital, the clinical history seemed compatible with a LGG and seizures well controlled with antiepileptic drugs. Neurological examination was completely normal. MRI showed a large tumor (7x5x5 cm) hypointense on T1, hyperintense on T2, without contrast enhancement, involving the right temporal lobe white matter, insula, internal capsule, hipoccampus, thalamus and mesencephalus with middle cerebral artery encasement. Interval imaging was proposed and after 4.5 years since diagnosis the tumor has been stable and patient clinically excellent. CONCLUSION Overall survival in pediatric LGGs is excellent and risk of sequelae should always be part of multidisciplinary team considerations. In centers with significant neurosurgical morbidity, biopsy of large tumors that are compatible with LGG may not be required in selected cases.

scholarly journals Pathobiology ofHelicobacter pyloriInfection in Children

1999 ◽  
Vol 13 (7) ◽  
pp. 599-603 ◽  
Author(s):  
Robert H Riddell
Keyword(s):  
Intestinal Metaplasia ◽  
Pediatric Population ◽  
Paediatric Population ◽  
Lymphoid Hyperplasia ◽  
Host Responses ◽  
Low Grade ◽  
Cell Axis ◽  
Age Related ◽  
H Pylori ◽  
Time Required

In the paediatric population, the associations ofHelicobacter pyloriwith gastritis, gastric ulcer, duodenitis and duodenal ulcer, and with duodenal gastric surface metaplasia and disorders of the D cell-G cell axis resulting in hypergastrinemia, are well established and in many ways resemble their counterparts in adults. Eradication ofH pyloriinvariably results in the reversal of these diseases with time. There are also suggestions that gastric surface metaplasia is more extensive in children withH pylori, and may be the site of duodenalH pyloriinfection and associated duodenal erosions or ulcers. There is no consensus as to whetherH pyloriin children is more or less severe than in adults. In one paediatric cohort,H pyloriwas associated with increased intensity of inflammation, while other studies suggest that acute inflammation may be less intense in children overall but that chronic inflammation may be increased in intensity, including lymphoid hyperplasia, which in turn may correlate with endoscopic nodularity. Lymphoid hyperplasia and nodular gastritis appear to be more frequent in children than in adults and usually regress followingH pylorieradication. However, in children, other diseases or morphological abnormalities, including some loss of glands (atrophy), occasionally intestinal metaplasia, lymphoproliferative diseases including low grade mucosal-associated lymphoid tissue lymphoma, lymphocytic gastritis and hypertrophic gastritis/Menetrier’s disease, are much less frequently associated withH pylorithan in adults. Other associations are rarely seen in children, primarily because the time required for these to develop takes the individual to adulthood; for example, while intestinal metaplasia occurs in the pediatric population, the complications of adenoma/dysplasia and carcinoma are rare. In adults, inflammatory and hyperplastic polyps, atrophic gastritis and pernicious anemia, and in some patients granulomas (granulomatous gastritis), may also be associated withH pyloriinfection. Greater awareness of the spectrum of diseases associated withH pylorimay well lead to their increased recognition in the paediatric population. Some diseases, particularly Crohn’s disease, but also human immunodeficiency virus infection, have a negative association withH pylorithat appears not to be simply a result of the excess antibiotic therapy that these patients receive. These variations in association and reactions toH pylori, some of which are age-related, may allow the different host responses toH pylorithat occur in humans to be examined.

scholarly journals LGG-28. NOVEL BRAF INTRAGENIC DELETION IN A GLIOMA: A CASE REPORT OF A PEDIATRIC PATIENT

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii371-iii372
Author(s):  
Valerie Cruz Flores ◽  
Maxine Sutcliffe ◽  
Thomas Geller ◽  
Ignacio Gonzalez Gomez ◽  
Stephanie Smith ◽  
...  
Keyword(s):  
Pediatric Patient ◽  
Pilocytic Astrocytoma ◽  
Pediatric Population ◽  
Binding Pocket ◽  
Genetic Alterations ◽  
Low Grade ◽  
Braf Gene ◽  
Intragenic Deletion ◽  
Whole Genome Microarray ◽  
Intragenic Deletions

Abstract BACKGROUND Numerous variant BRAF genetic alterations have been associated with malignancies. BRAF activating fusions/mutations are frequently present in low grade gliomas. BRAF intragenic deletions have been reported in melanoma, but have not previously been reported in gliomas. OBJECTIVE To report a BRAF intragenic deletion in a pediatric patient with recurrent low-grade glioma. RESULTS A 3-year-old female underwent a complete resection of a posterior fossa pilocytic astrocytoma. She had recurrences at age 4, and then at age 9; pathology was consistent with pilocytic astrocytoma. Microarray analysis on sample from the first recurrence showed one region of loss encompassing 86 Kbp within the BRAF gene. The deletion breakpoints are within intron 1 and 9, resulting in loss of exons 2 through 9, inclusive. This has been previously described melanoma, but appears to be a novel finding in glioma. It is hypothesized that, since the loss retains the kinase and ATP binding pocket domains but deletes the N-terminal conserved region 1 and 2 (CR1, CR2) of the BRAF gene, it is likely functionally similar to the loss and activation resulting from the more usually described KIAA1549 and BRAF gene fusion. CONCLUSION This is the first BRAF intragenic deletion involving exons 2–9 reported in a glioma. Although 86kbp is small using whole genome microarray technology, it is large using sequencing strategies, and a targeted sequencing approach to investigate the BRAF gene would not readily identify this deletion. It is speculated that the deletion may be under ascertained in the pediatric population.

scholarly journals PATH-21. TELOMERE LENGTH ANALYSIS OF CNS TUMORS IN THE PEDIATRIC BRAIN TUMOR ATLAS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii428-iii428
Author(s):  
Jo Lynn Rokita ◽  
Krutika Gaonkar ◽  
Heba Ijaz ◽  
Daniel Miller ◽  
Tasso Karras ◽  
...  
Keyword(s):  
Brain Tumor ◽  
Gene Mutations ◽  
Pediatric Brain Tumor ◽  
High Grade Glioma ◽  
Pediatric Brain Tumors ◽  
Low Grade ◽  
Alternative Lengthening ◽  
Length Analysis ◽  
Telomere Lengthening ◽  
Pediatric Brain

Abstract Subsets of pediatric cancers, including high grade glioma (pHGG), have high rates of uniquely long telomeres, associated with ATRX gene mutations and alternative lengthening of telomeres (ALT). Ultimately, these cancers may benefit from a therapy stratification approach. In order to identify and further characterize pediatric brain tumors with telomere lengthening (TL), we determined the intratelomeric content in silico from paired WGS of 918 tumors from CBTTC Pediatric Brain Tumor Atlas (PBTA). The results were highly concordant with experimental assays to determine ALT in a subset of 45 pHGG tumors from the set. Overall, 13% of the PBTA cohort had telomere lengthening. We confirmed the highest rate of TL (37%) in the pHGG cohort (37/100 tumors; 30/82 patients). There was no statistical difference in age, gender or survival in subset analysis. As expected, the patient pHGG tumors with telomere lengthening were enriched for ATRX mutations (60%, q= 1.76e-3). However, 6 tumors without ATRX mutation also had normal protein expression, suggesting a different mechanism of inactivation or TL. The pHGG tumors with telomere lengthening had increased mutational burden (q=8.98e-3) and included all known pHGG cases (n=6) in the cohort with replication repair deficiencies. Of interest, the second highest rate of telomere lengthening was 9 subjects (24%) in the craniopharyngioma cohort. None of the craniopharyngioma tumors had ATRX mutations or low ATRX expression, and 55% of those with TL had CTNNB1 mutations. Finally, lower rates of telomere lengthening were found in medulloblastoma (10%), ependymoma (10%), low grade astrocytoma (8%) and ganglioglioma (7/47, 15%).

Adult brain low-grade astrocytomas: survival after surgery and radiotherapy

2001 ◽  
Vol 22 (3) ◽  
pp. 233-238 ◽  
Author(s):  
V. M. Arienti ◽  
A. Botturi ◽  
A. Boiardi ◽  
G. Broggi ◽  
M. Collice ◽  
...  
Keyword(s):  
Adult Brain ◽  
Low Grade

scholarly journals Neurotrophic Receptor Tyrosine Kinase 2 (NTRK2) Alterations in Low-Grade Gliomas: Report of a Novel Gene Fusion Partner in a Pilocytic Astrocytoma and Review of the Literature

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Siobhan S. Pattwell ◽  
Eric Q. Konnick ◽  
Yajuan J. Liu ◽  
Rebecca A. Yoda ◽  
Laligam N. Sekhar ◽  
...  
Keyword(s):  
Pilocytic Astrocytoma ◽  
Gene Fusion ◽  
Pediatric Population ◽  
Neurofibromatosis Type ◽  
Genetic Alterations ◽  
Neurotrophin Receptor ◽  
Low Grade ◽  
Fusion Partner ◽  
Tyrosine Kinase 2 ◽  
Cns Neoplasms

Pilocytic astrocytoma is a low-grade glial neoplasm of the central nervous system (CNS) that tends to occur in the pediatric population and less commonly presents in adults. Hereditary pilocytic astrocytoma is often associated with germline genetic alterations in the tumor suppressor NF1, the gene responsible for the syndrome neurofibromatosis type 1. Sporadic pilocytic astrocytoma frequently harbors somatic alterations in BRAF, with rare pilocytic astrocytomas containing alterations in FGFR1 and NTRK2. NTRK2 encodes for the protein tropomyosin receptor kinase B (TrkB), which is a neurotrophin receptor with high affinity for Brain-Derived Neurotrophic Factor (BDNF), and plays a role in several physiological functions of neurons, including cell survival and differentiation. In this report, we describe a novel PML-NTRK2 gene fusion occurring in an adult sporadic pilocytic astrocytoma and review the biology and implications of specific NTRK2 mutations occurring in CNS neoplasms.

Exploiting Laboratory Insights to Improve Outcomes of Pediatric Central Nervous System Tumors

2016 ◽  
pp. e540-e546 ◽  
Author(s):  
Giles W. Robinson ◽  
Hendrik Witt ◽  
Adam Resnick
Keyword(s):  
Brain Tumor ◽  
Molecular Mechanisms ◽  
Therapeutic Potential ◽  
Pediatric Brain Tumor ◽  
Pediatric Brain Tumors ◽  
Low Grade ◽  
Short Period ◽  
Tumor Management ◽  
The Impact ◽  
Pediatric Brain

Over a relatively short period of time, owing to improvements in biotechnology, our ability to identify the molecular mechanisms within pediatric brain tumors has dramatically increased. These findings have reshaped the way that we describe these diseases and have provided insights into how to better treat these often devastating diseases. Although still far from reaching the full therapeutic potential these advancements hold, the impact of these findings is steadily taking hold of pediatric brain tumor management. In this article, we summarize the major discoveries within three common pediatric brain tumor categories; medulloblastoma, ependymoma, and low-grade glioma. We discuss the current impact of these findings on treatment and the direction these findings may take the field of pediatric neuro-oncology.

Polyps and Masses of the Pediatric Urinary Bladder: A 21-Year Pathology Review

2011 ◽  
Vol 14 (6) ◽  
pp. 438-444 ◽  
Author(s):  
Alison R. Huppmann ◽  
Bruce R. Pawel
Keyword(s):  
Urinary Bladder ◽  
Pediatric Population ◽  
Low Grade ◽  
Nephrogenic Adenoma ◽  
Patient Demographics ◽  
Urothelial Tumors ◽  
Histologic Types ◽  
Cystitis Cystica ◽  
Pathology Review ◽  
Made In

Although not uncommon in adults, bladder tumors are rare in children. In addition, the histologic types of tumors seen in the pediatric population differ from those seen in adults. Although rhabdomyosarcoma is the most common pediatric bladder tumor, many other benign, malignant, and reactive lesions can be encountered. All may present clinically as a mass or polyp in the bladder. This study was designed to describe the pathology and patient demographics of pediatric bladder masses, because there are few studies describing these entities. Retrospectively reviewing our experience over a 21-year period, we identified 98 specimens from 65 patients with polyps or masses in the urinary bladder. As expected, the most frequent diagnosis was rhabdomyosarcoma. This was followed by fibroepithelial polyp and a variety of additional nonurothelial tumors. Only 7 urothelial tumors were identified, including 1 low-grade papillary urothelial carcinoma. Inflammatory lesions, such as cystitis cystica and nephrogenic adenoma, were invariably associated with an irritating factor when a history was provided. Our findings emphasize that diagnoses made in the pediatric urinary bladder are distinct from those in adults, although a wide variety of lesions may still be seen.

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