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1. Espinoza JC, Haley K, Patel N, Dhall G, Gardner S, Allen J, Torkildson J, Cornelius A, Rassekh R, Bedros A, Etzl M, Garvin J, Pradhan K, Corbett R, Sullivan M, McGowage G, Stein D, Jasty R, Sands SA, Ji L, Sposto R, Finlay JL: Outcome of young children with high-grade glioma treated with irradiation-avoiding intensive chemotherapy regimens: Final report of the Head Start II and III trials. Pediatr Blood Cancer; 2016 Oct;63(10):1806-13
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  • [Title] Outcome of young children with high-grade glioma treated with irradiation-avoiding intensive chemotherapy regimens: Final report of the Head Start II and III trials.
  • PURPOSE: To report the final analysis of survival outcomes for children with newly diagnosed high-grade glioma (HGG) treated on the "Head Start" (HS) II and III protocols with chemotherapy and intent to avoid irradiation in children <6 years old.
  • PATIENTS AND METHODS: Between 1997 and 2009, 32 eligible children were enrolled in HS II and III with anaplastic astrocytoma (AA, n = 19), glioblastoma multiforme (GBM, n = 11), or other HGG (n = 2).
  • Central pathology review was completed on 78% of patients.
  • Irradiation was used for patients with residual tumor after consolidation or >6 years old or at the time of tumor progression.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Glioma / drug therapy
  • [MeSH-minor] Child. Child, Preschool. Clinical Trials as Topic. Female. Humans. Induction Chemotherapy. Infant. Infant, Newborn. Male. Neoplasm Recurrence, Local. Prognosis. Survival Rate

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  • [Copyright] © 2016 Wiley Periodicals, Inc.
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  • (PMID = 27332770.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P30 CA008748
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Keywords] NOTNLM ; Head Start / gliomas / irradiation-avoiding strategies / pediatrics
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2. Jung KW, Yoo H, Kong HJ, Won YJ, Park S, Lee SH: Population-based survival data for brain tumors in Korea. J Neurooncol; 2012 Sep;109(2):301-7
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  • [Title] Population-based survival data for brain tumors in Korea.
  • Primary brain tumors are relatively uncommon but particularly lethal cancers.
  • Although survival is useful for monitoring the effects of early cancer detection and treatment, there are few population-based estimates of survival for subjects with brain tumors, especially in Asian countries.
  • Using the Korea National Cancer Incidence Database, 4,721 newly diagnosed cases of histologically confirmed malignant primary brain tumors from 1999 to 2004 were analyzed for observed survival.
  • For trend analyses of glioblastomas, we included 2,751 glioblastoma cases diagnosed between 1999 and 2007.
  • For all ages and all brain tumor types in Korea, five-year survival was 37.5 %.
  • For each histological type of brain tumor survival of pediatric and younger adult populations was much better than that of older adults.
  • Five-year survival for glioblastoma, astrocytoma, anaplastic astrocytoma, and oligodendroglioma was 8.9, 51.6, 25.2, and 73.5 %, respectively.
  • Two-year survival for glioblastoma increased from 18.6 % for cases diagnosed in 1999-2001 to 21.3 % for cases diagnosed in 2002-2004 and to 24.7 % for cases diagnosed in 2005-2007.
  • These results may help clinicians and patients to assess long-term prognoses for brain tumors, and the data presented here could serve as master control data set for single-arm clinical trials, especially in Asian populations.
  • [MeSH-major] Astrocytoma / epidemiology. Brain Neoplasms / epidemiology. Glioblastoma / epidemiology

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  • [ISSN] 1573-7373
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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3. Antonelli M, Buttarelli FR, Arcella A, Nobusawa S, Donofrio V, Oghaki H, Giangaspero F: Prognostic significance of histological grading, p53 status, YKL-40 expression, and IDH1 mutations in pediatric high-grade gliomas. J Neurooncol; 2010 Sep;99(2):209-15
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  • [Title] Prognostic significance of histological grading, p53 status, YKL-40 expression, and IDH1 mutations in pediatric high-grade gliomas.
  • The objective of this study was to evaluate, in a series of 43 pediatric high-grade gliomas (21 anaplastic astrocytoma WHO grade III and 22 glioblastoma WHO grade IV), the prognostic value of histological grading and expression of p53 and YKL-40.
  • The prognostic stratification for histological grading showed no difference in overall (OS) and progression-free survival (PFS) between glioblastomas and anaplastic astrocytomas.
  • TP53 mutations were detected in five of 27 (18%) cases (four glioblastomas and one anaplastic astrocytoma).
  • Our results suggest that in pediatric high-grade gliomas: (i) histological grading does not have strong prognostic significance, (ii) YKL-40 overexpression is less frequent than adult high-grade gliomas and does not correlate with a more aggressive behavior, (iii) TP53 mutations but not p53 expression may correlate with a more aggressive behavior, and (iv) IDH1 mutations are absent.
  • These observations support the concept that, despite identical histological features, the biology of high-grade gliomas in children differs from that in adults, and therefore different prognostic factors are needed.
  • [MeSH-major] Astrocytoma / genetics. Astrocytoma / metabolism. Glycoproteins / metabolism. Isocitrate Dehydrogenase / genetics. Lectins / metabolism. Mutation / genetics. Tumor Suppressor Protein p53 / genetics
  • [MeSH-minor] Adipokines. Adolescent. Adult. Brain Neoplasms / genetics. Brain Neoplasms / metabolism. Brain Neoplasms / pathology. Child. Child, Preschool. Chitinase-3-Like Protein 1. DNA, Neoplasm / genetics. Female. Humans. Immunoenzyme Techniques. Infant. Infant, Newborn. Male. Neoplasm Staging. Polymerase Chain Reaction. Prognosis. Young Adult

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  • (PMID = 20174854.001).
  • [ISSN] 1573-7373
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Adipokines; 0 / CHI3L1 protein, human; 0 / Chitinase-3-Like Protein 1; 0 / DNA, Neoplasm; 0 / Glycoproteins; 0 / Lectins; 0 / TP53 protein, human; 0 / Tumor Suppressor Protein p53; EC 1.1.1.41 / Isocitrate Dehydrogenase; EC 1.1.1.42. / IDH1 protein, human
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4. Giunti L, Buccoliero AM, Pantaleo M, Lucchesi M, Provenzano A, Palazzo V, Guarducci S, Guidi M, Genitori L, Zuffardi O, Sardi I, Giglio S: Molecular characterization of paediatric glioneuronal tumours with neuropil-like islands: a genome-wide copy number analysis. Am J Cancer Res; 2016;6(12):2910-2918
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • : Paediatric glioneuronal tumour with neuropil-like islands (GTNI) is a rare neoplasm of neuronal differentiation and diffusely infiltrating astroglial and oligodendrocyte-like components.
  • The 2007 World Health Organization classification of central nervous system tumours considered it as a pattern variation of anaplastic astrocytoma.

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  • (PMID = 28042510.001).
  • [Journal-full-title] American journal of cancer research
  • [ISO-abbreviation] Am J Cancer Res
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Keywords] NOTNLM ; Database of Genomic Variants (DGV) / Glioneuronal tumour with neuropil-like islands (GTNI) / SNP/CGH array / amplification / central nervous tumours (CNS) / common genomic alteration / copy number variations (CNVs) / mosaicism / paediatric brain tumours / variation of anaplastic astrocytoma
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5. Adams H, Avendaño J, Raza SM, Gokaslan ZL, Jallo GI, Quiñones-Hinojosa A: Prognostic factors and survival in primary malignant astrocytomas of the spinal cord: a population-based analysis from 1973 to 2007. Spine (Phila Pa 1976); 2012 May 20;37(12):E727-35
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Prognostic factors and survival in primary malignant astrocytomas of the spinal cord: a population-based analysis from 1973 to 2007.
  • OBJECTIVE: Using data from the population-based cancer registries of the Surveillance, Epidemiology and End Results (SEER) program, we analyzed demographic features, tumor and treatment characteristics, as well as survival rates in patients with primary malignant astrocytomas of the spinal cord (PMASC).
  • SUMMARY OF BACKGROUND DATA: PMASC is a rare neoplasm and is considered to carry the same dismal outcome as their cerebral counterparts.
  • METHODS: The SEER data from 1973 to 2007 were reviewed for pathologically confirmed primary anaplastic astrocytomas (AA) and glioblastomas of the spinal cord (C72.0).
  • Adult patients observed markedly prolonged survival compared with the pediatric group, with a 16-month versus 9-month median survival, respectively.
  • Multivariate analysis revealed age at diagnosis, pediatric and adult age groups, sex, tumor histology, and extent of resection as significant predictors of survival.

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  • (PMID = 22609727.001).
  • [ISSN] 1528-1159
  • [Journal-full-title] Spine
  • [ISO-abbreviation] Spine
  • [Language] ENG
  • [Grant] United States / NINDS NIH HHS / NS / K08 NS055851; United States / NINDS NIH HHS / NS / K08 NS055851-05; United States / Howard Hughes Medical Institute / /
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS348653; NLM/ PMC3358669
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6. Keir ST, Dewhirst MW, Kirkpatrick JP, Bigner DD, Batinic-Haberle I: Cellular redox modulator, ortho Mn(III) meso-tetrakis(N-n-hexylpyridinium-2-yl)porphyrin, MnTnHex-2-PyP(5+) in the treatment of brain tumors. Anticancer Agents Med Chem; 2011 Feb;11(2):202-12
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Cellular redox modulator, ortho Mn(III) meso-tetrakis(N-n-hexylpyridinium-2-yl)porphyrin, MnTnHex-2-PyP(5+) in the treatment of brain tumors.
  • Despite intensive efforts to improve multimodal treatment of brain tumor, survival remains limited.
  • Identifying novel targeted therapies is therefore at the forefront of brain tumor research.
  • This study explores the utility of a manganese porphyrin in a brain tumor model.
  • The compound used is ortho isomer, mangnese(III) meso-tetrakis(N-n-hexylpyridinium-2-yl)porphyrin, MnTnHex-2-PyP(5+).
  • It is further one of the most lipophilic compound among cationic Mn(III) N-alkylpyridylporphyrins, and thus accumulates predominantly in mitochondria relative to cytosol.
  • In mitochondria, MnTnHex-2-PyP(5+) mimics our key antioxidant system, mitochondrial superoxide dismutase, MnSOD, whose overexpression has been widely shown to suppress tumor growth.
  • Importantly, MnTnHex-2-PyP(5+) crosses blood brain barrier in sufficient amounts to demonstrate efficacy in treating CNS injuries.
  • For those reasons we elected to test its effects in inhibiting brain tumor growth.
  • This study is the first report of the antitumor properties of MnTnHex-2-PyP(5+) as a single agent in adult and pediatric glioblastoma multiforme (D-54 MG, D-245 MG, D-256 MG, D-456 MG) and pediatric medulloblastoma (D-341 MED), and is the first case where a redox-able metal complex has been used in glioma therapy.
  • With mice bearing intracranial xenografts, MnTnHex-2-PyP(5+) increases median survival by 33% in adult glioblastoma multiforme (D-256 MG; p≤ 0.001) and 173% in pediatric medulloblastoma (D-341 MED, <0.001).
  • Our findings suggest that the use of Mn porphyrin-based SOD mimics, and in particular lipophilic analogues such as MnTnHex-2-PyP(5+), is a promising approach for brain tumor therapy.

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  • (PMID = 21291403.001).
  • [ISSN] 1875-5992
  • [Journal-full-title] Anti-cancer agents in medicinal chemistry
  • [ISO-abbreviation] Anticancer Agents Med Chem
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P50 CA108786; United States / NINDS NIH HHS / NS / P50 NS020023; United States / NCI NIH HHS / CA / K99CA143229; United States / NCI NIH HHS / CA / R37 CA 011898; United States / NCI NIH HHS / CA / R01 CA40355; United States / NCI NIH HHS / CA / 5P50 CA108786; United States / NCI NIH HHS / CA / R37 CA011898; United States / NCI NIH HHS / CA / R01 CA040355
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Metalloporphyrins; 0 / Mn(III) meso-tetrakis(N-n-hexylpyridinium-2-yl)porphyrin; EC 1.15.1.1 / Superoxide Dismutase; EC 1.15.1.1 / superoxide dismutase 2
  • [Other-IDs] NLM/ NIHMS353673; NLM/ PMC3357315
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7. Isaacs H Jr: Perinatal (fetal and neonatal) astrocytoma: a review. Childs Nerv Syst; 2016 Nov;32(11):2085-2096
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  • [Title] Perinatal (fetal and neonatal) astrocytoma: a review.
  • INTRODUCTION: The purpose of this review is to document the various types of astrocytoma that occur in the fetus and neonate, their locations, initial findings, pathology, and outcome.
  • Glioblastoma (GBM) was the most common neoplasm followed in order by subependymal giant cell astrocytoma (SEGA), low-grade astrocytoma, anaplastic astrocytoma, and desmoplastic infantile astrocytoma (DIA).
  • CONCLUSION: A number of patients were considered inoperable since their tumor occupied much of the intracranial cavity involving large areas of the brain.
  • High-grade astrocytomas were more common than low-grade ones in this review.
  • Fetuses and neonates with astrocytoma have a mixed prognosis ranging from as low as 20 % (GBM) to a high of 90 %.
  • [MeSH-major] Astrocytoma / congenital. Astrocytoma / pathology. Brain Neoplasms / congenital. Brain Neoplasms / pathology

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  • (PMID = 27568373.001).
  • [ISSN] 1433-0350
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Keywords] NOTNLM ; Congenital brain tumor (major topic) / Fetal astrocytoma (major topic) / Intracranial hemorrhage (major topic) / Neonatal astrocytoma (major topic) / Perinatal astrocytoma (major topic)
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8. Hoffman LM, Fouladi M, Olson J, Daryani VM, Stewart CF, Wetmore C, Kocak M, Onar-Thomas A, Wagner L, Gururangan S, Packer RJ, Blaney SM, Gajjar A, Kun LE, Boyett JM, Gilbertson RJ: Phase I trial of weekly MK-0752 in children with refractory central nervous system malignancies: a pediatric brain tumor consortium study. Childs Nerv Syst; 2015 Aug;31(8):1283-9
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  • [Title] Phase I trial of weekly MK-0752 in children with refractory central nervous system malignancies: a pediatric brain tumor consortium study.
  • PURPOSE: Amplification and high levels of NOTCH ligand expression have been identified in several types of pediatric brain tumors.
  • A phase I trial of weekly MK-0752, an oral inhibitor of gamma-secretase, was conducted in children with recurrent central nervous system (CNS) malignancies to estimate the maximum tolerated dose, dose-limiting toxicities (DLT), pharmacokinetics (PK), and pharmacodynamics of weekly MK-0752.
  • RESULTS: Ten eligible patients were enrolled (median age 8.8 years; range 3.1-19.2) with diagnoses of brain stem glioma (n = 3), ependymoma (n = 2), anaplastic astrocytoma (n = 1), choroid plexus carcinoma (n = 2), medulloblastoma (n = 1), and primitive neuroectodermal tumor (n = 1).
  • Non-dose-limiting grade 3 toxicities included lymphopenia, neutropenia, and anemia.
  • CONCLUSION: MK-0752 is well tolerated and exhibits target inhibition at 1000 and 1400 mg/m(2)/week in children with recurrent CNS malignancies.
  • [MeSH-major] Benzene Derivatives / therapeutic use. Brain Neoplasms / drug therapy. Central Nervous System Diseases / drug therapy. Enzyme Inhibitors / therapeutic use. Propionates / therapeutic use. Sulfones / therapeutic use

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  • (PMID = 25930724.001).
  • [ISSN] 1433-0350
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / R01 CA129541; United States / NCI NIH HHS / CA / R01 CA114567; United States / NCI NIH HHS / CA / P30 CA021765; United States / NCI NIH HHS / CA / P01 CA096832; United States / NCI NIH HHS / CA / U01 CA81457; United States / NCI NIH HHS / CA / UM1 CA081457
  • [Publication-type] Clinical Trial, Phase I; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / 3-(4-((4-chlorophenyl)sulfonyl)-4-(2,5-difluorophenyl)cyclohexyl)propanoic acid; 0 / Basic Helix-Loop-Helix Transcription Factors; 0 / Benzene Derivatives; 0 / Enzyme Inhibitors; 0 / Homeodomain Proteins; 0 / NOTCH1 protein, human; 0 / Propionates; 0 / Receptor, Notch1; 0 / Repressor Proteins; 0 / Sulfones; 0 / Transcription Factor HES-1; 148591-48-4 / HES5 protein, human; 149348-15-2 / HES1 protein, human; EC 3.4.- / Amyloid Precursor Protein Secretases
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9. Lai IC, Wong TT, Shiau CY, Hu YW, Ho DM, Chang KP, Guo WY, Chang FC, Liang ML, Lee YY, Chen HH, Yen SH, Chen YW: Treatment results and prognostic factors for intracranial nongerminomatous germ cell tumors: single institute experience. Childs Nerv Syst; 2015 May;31(5):683-91
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • METHODS: Thirty-nine patients with nondisseminated NGGCTs, excluding those with pure mature teratomas, were treated between January 1985 and December 2010.
  • Radiotherapy was given postoperatively or definitively with a median tumor bed dose of 54 Gy (range 30-54) with or without craniospinal irradiation.
  • All patients received ten cycles of adjuvant chemotherapy, vinblastine, bleomycin, etoposide, and cisplatin after radiotherapy, except for one with mixed anaplastic astrocytoma component who received oral temozolomide.
  • Inferior PFS was associated with lesions in the suprasellar region (p = 0.017), poor pathological features (p = 0.048), and with poor image (p < 0.0001) and tumor marker (TM) response (p = 0.003) to irradiation.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / therapy. Craniospinal Irradiation. Neoplasms, Germ Cell and Embryonal / therapy. Neurosurgical Procedures / methods

  • MedlinePlus Health Information. consumer health - Brain Tumors.
  • MedlinePlus Health Information. consumer health - Childhood Brain Tumors.
  • Hazardous Substances Data Bank. BLEOMYCIN .
  • Hazardous Substances Data Bank. CIS-DIAMINEDICHLOROPLATINUM .
  • Hazardous Substances Data Bank. ETOPOSIDE .
  • Hazardous Substances Data Bank. DACARBAZINE .
  • Hazardous Substances Data Bank. VINBLASTINE .
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  • (PMID = 25749900.001).
  • [ISSN] 1433-0350
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 11056-06-7 / Bleomycin; 5V9KLZ54CY / Vinblastine; 6PLQ3CP4P3 / Etoposide; 7GR28W0FJI / Dacarbazine; Q20Q21Q62J / Cisplatin; YF1K15M17Y / temozolomide
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10. Rosenfeld A, Listernick R, Charrow J, Goldman S: Neurofibromatosis type 1 and high-grade tumors of the central nervous system. Childs Nerv Syst; 2010 May;26(5):663-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Neurofibromatosis type 1 and high-grade tumors of the central nervous system.
  • PURPOSE: Neurofibromatosis type 1 (NF1), a common genetic disorder, predisposes patients to the development of both benign and malignant tumors.
  • Although the most common central nervous system (CNS) tumor is a low-grade pilocytic astrocytoma of the optic pathway, there have been sporadic reports of NF1 patients with more aggressive CNS lesions.
  • We investigated the incidence of aggressive CNS lesions in NF1 patients at our institution.
  • METHODS: We conducted a retrospective review of all patients with NF1 and any CNS tumor being followed in the Children's Memorial Hospital NF1 Clinic.
  • Of these, 145 (20%) patients had CNS tumors, 99 (68%) of whom had optic pathway tumors (OPTs).
  • Five patients (3%) were identified as having high-grade tumors, which consisted of anaplastic medulloblastoma (n = 1) and high-grade glioma (n = 4).
  • Three of the five patients had a history of an OPT prior to the development of their high-grade lesions.
  • CONCLUSION: High-grade CNS tumors may occur in children with NF1.
  • Although tumors in NF patients are generally benign, clinicians should have a high index of suspicion of malignancy in patients whose tumors are in an unusual location or behave in an uncharacteristically aggressive manner.
  • [MeSH-major] Brain Neoplasms / complications. Neurofibromatosis 1 / complications






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