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1. Pettorini BL, Inzitari R, Massimi L, Tamburrini G, Caldarelli M, Fanali C, Cabras T, Messana I, Castagnola M, Di Rocco C: The role of inflammation in the genesis of the cystic component of craniopharyngiomas. Childs Nerv Syst; 2010 Dec;26(12):1779-84
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  • BACKGROUND: Craniopharyngioma accounts for 5-10% of childhood tumors and, despite of the benign histological features, its clinical course can be malignant because of critical anatomical relationships with neural and vascular structures and the possible morbidity associated to resection.
  • Only a few studies have addressed the molecular characterization of the cyst fluid so far and the mechanisms of action of intracystic agents are not clearly understood yet.
  • METHODS: The acidic soluble proteins contained in the cystic fluid of six patients with cystic craniopharyngioma, three of them treated with intratumoral interferon-α, were analyzed.
  • Amount of peptides significantly decreased in cystic fluid during pharmacological treatment.
  • INTERPRETATION: Detection of α-defensins 1-3 excludes that cyst fluid formation can derive from disruption of blood-brain barrier and suggests the involvement of innate immune response in pathology of craniopharyngioma cyst formation.
  • [MeSH-major] Craniopharyngioma / immunology. Cysts / immunology. Inflammation / immunology. Pituitary Neoplasms / immunology

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  • (PMID = 20668862.001).
  • [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
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Immunologic Factors; 0 / Interferon-alpha; 0 / alpha-Defensins
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2. Frei-Jones M, McKinstry RC, Perry A, Leonard JR, Park TS, Rubin JB: Use of thalidomide to diminish growth velocity in a life-threatening congenital intracranial hemangioma. J Neurosurg Pediatr; 2008 Aug;2(2):125-9
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  • Infantile or capillary hemangioma is the most common vascular tumor of childhood.
  • Although the lesions are considered benign, 10% of affected children develop life-threatening complications (mortality rate 20-80% in this subgroup).
  • Vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF) have been implicated in the pathogenesis of hemangiomas, and antiangiogenesis agents are being evaluated in the treatment of these tumors.
  • Thalidomide may be an ideal therapy for life-threatening hemangiomas because it inhibits new blood vessel formation by antagonizing both the bFGF and VEGF pathways and has a more acceptable toxicity profile than other agents.
  • [MeSH-major] Angiogenesis Inhibitors / therapeutic use. Brain Neoplasms / congenital. Brain Neoplasms / drug therapy. Hemangioma, Capillary / congenital. Hemangioma, Capillary / drug therapy. Thalidomide / therapeutic use

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  • (PMID = 18671617.001).
  • [ISSN] 1933-0707
  • [Journal-full-title] Journal of neurosurgery. Pediatrics
  • [ISO-abbreviation] J Neurosurg Pediatr
  • [Language] eng
  • [Grant] United States / NCRR NIH HHS / RR / K30 RR022251; United States / NCRR NIH HHS / RR / TL1 RR024995; United States / NCRR NIH HHS / RR / UL1 RR024992; United States / NCRR NIH HHS / RR / UL1 RR024992; United States / NCRR NIH HHS / RR / UL1 RR024992-01; United States / NCATS NIH HHS / TR / UL1 TR000448
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 4Z8R6ORS6L / Thalidomide
  • [Other-IDs] NLM/ NIHMS101679; NLM/ PMC2737696
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3. Grill J, Pascal C, Chantal K: Childhood ependymoma: a systematic review of treatment options and strategies. Paediatr Drugs; 2003;5(8):533-43
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  • [Title] Childhood ependymoma: a systematic review of treatment options and strategies.
  • Childhood intracranial ependymoma have a dismal prognosis, especially in young children and when a gross total resection cannot be performed.
  • Despite some indication that benign ependymoma (WHO grade II) could show a better outcome, histology cannot be used at present to stratify treatment protocols.Craniospinal irradiation combined with posterior fossa boost has deleterious adverse effects on cognition.
  • Consequently, pediatric oncology teams have, firstly, tried to use chemotherapy to delay or avoid irradiation, and secondly, progressively reduced irradiation fields to the tumor bed without altering the prognosis.
  • Cisplatin, at a dose of 120 mg/m(2) (cumulated response rate of 34% [95% CI 19-54%]) is the only single agent that has reproducibly shown some efficacy in ependymoma.
  • Despite some combinations showing efficacy in the adjuvant setting, childhood intracranial ependymomas can, in general, be considered as chemoresistant.
  • As the use of chemotherapy with current agents is questionable, phase II studies with new agents and combinations are necessary.
  • Since the main problem of this disease is local relapse, it may not be necessary to irradiate the whole posterior fossa.
  • There is no doubt that any possible improvement in the management of this rare tumor will only be the result of well designed cooperative trials.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Brain Neoplasms / therapy. Ependymoma / therapy
  • [MeSH-minor] Adolescent. Chemotherapy, Adjuvant. Child. Child, Preschool. Drug Resistance, Neoplasm. Drug Therapy, Combination. Humans. Infant. Infant, Newborn. Time Factors

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  • (PMID = 12895136.001).
  • [ISSN] 1174-5878
  • [Journal-full-title] Paediatric drugs
  • [ISO-abbreviation] Paediatr Drugs
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 120
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4. Qaddoumi I, Sultan I, Broniscer A: Pediatric low-grade gliomas and the need for new options for therapy: Why and how? Cancer Biol Ther; 2009 Jan;8(1):4-10
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  • [Title] Pediatric low-grade gliomas and the need for new options for therapy: Why and how?
  • Pediatric low-grade gliomas are the most common tumors of the central nervous system in children, accounting for almost 50% of all childhood brain tumors.
  • This is mostly due to a lack of understanding of tumor biology at the molecular level.
  • Pediatric low-grade gliomas are not benign, and most incompletely resected tumors will progress and negatively affect quality of life.
  • The advancements made in understanding sporadic pilocytic astrocytoma and neurofibromatosis 1-associated pilocytic astrocytoma in particular have paved the way for potential targeted therapy and biological stratification.
  • Such progress in pilocytic astrocytoma needs to be consolidated and expanded to other histologic varieties of pediatric low-grade gliomas.

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  • (PMID = 19164945.001).
  • [ISSN] 1555-8576
  • [Journal-full-title] Cancer biology & therapy
  • [ISO-abbreviation] Cancer Biol. Ther.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P30 CA021765; None / None / / P30 CA021765-31; United States / NCI NIH HHS / CA / P30 CA021765-31; United States / NCI NIH HHS / CA / P30 CA21765
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] EC 2.7.11.1 / BRAF protein, human; EC 2.7.11.1 / Proto-Oncogene Proteins B-raf
  • [Number-of-references] 97
  • [Other-IDs] NLM/ NIHMS161402; NLM/ PMC2810626
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5. Hargrave D: Paediatric high and low grade glioma: the impact of tumour biology on current and future therapy. Br J Neurosurg; 2009 Aug;23(4):351-63
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Gliomas are the most common type of paediatric brain tumour and range from benign low grade gliomas which can be resected/observed to aggressive brainstem gliomas with dismal survival rates.
  • However, increasing knowledge of glioma biology is starting to impact on drug development towards targeted therapies.
  • Pilocytic astrocytoma, the most common childhood low grade brain tumour, has recently been shown to harbour an activated BRAF/MAPK/ERK pathway in the majority of cases; this represents an attractive target for new agents.
  • [MeSH-major] Brain Neoplasms. Glioma / pathology
  • [MeSH-minor] Adolescent. Adult. Astrocytoma / genetics. Astrocytoma / pathology. Astrocytoma / therapy. Child. Child, Preschool. Combined Modality Therapy. Drug Delivery Systems. Genetic Predisposition to Disease. Genome-Wide Association Study. Hamartoma Syndrome, Multiple / genetics. Humans. Infant. Neoplasm Staging. Neurofibromatosis 1 / genetics. Prognosis. Tuberous Sclerosis / genetics


6. Spalice A, Ruggieri M, Grosso S, Verrotti A, Polizzi A, Magro G, Caltabiano R, Pavone P, Del Balzo F, Platania N, Iannetti P: Dysembryoplastic neuroepithelial tumors: a prospective clinicopathologic and outcome study of 13 children. Pediatr Neurol; 2010 Dec;43(6):395-402
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Dysembryoplastic neuroepithelial tumors (DNETs) are benign intracortical masses that are typically observed in children and young adults and are classified as glioneuronal tumors (WHO grade I).
  • Large and retrospective series of patients with DNETs have been reported, but prospective studies on pediatric cohorts of patients with DNETs have been lacking.
  • In 11/13 cases, the seizures were resistant to drug therapy, and all the children had surgery consisting of extended lesionectomy coupled with neuronavigation.
  • This first prospective study with follow-up monitoring of a childhood population with DNETs confirms, on a long-term basis, that the coupled strategy of extended lesionectomy and neuronavigation has good outcome for long-term seizure control.
  • [MeSH-major] Brain Neoplasms / pathology. Cerebral Cortex / pathology. Neoplasms, Neuroepithelial / pathology. Seizures / pathology

  • MedlinePlus Health Information. consumer health - Brain Tumors.
  • MedlinePlus Health Information. consumer health - Childhood Brain Tumors.
  • MedlinePlus Health Information. consumer health - Seizures.
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  • [Copyright] Copyright © 2010 Elsevier Inc. All rights reserved.
  • (PMID = 21093729.001).
  • [ISSN] 1873-5150
  • [Journal-full-title] Pediatric neurology
  • [ISO-abbreviation] Pediatr. Neurol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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7. Shellhaas RA, Smith SE, O'Tool E, Licht DJ, Ichord RN: Mimics of childhood stroke: characteristics of a prospective cohort. Pediatrics; 2006 Aug;118(2):704-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mimics of childhood stroke: characteristics of a prospective cohort.
  • OBJECTIVES: Our goal was to describe patients with stroke mimics and to determine if clinical features predict benign diagnoses.
  • Cases in which stroke was ruled out (stroke mimics) were reviewed for clinical features and diagnostic test results and were classified "benign" if there was no structural brain lesion and there was an expectation of complete recovery.
  • Eleven patients had "benign" diagnoses (3 migraine, 3 psychogenic diagnoses, 3 musculoskeletal abnormalities, 1 delirium, and 1 episodic vital sign changes).
  • Nineteen patients had "not-benign" diagnoses (3 reversible posterior leukoencephalopathy syndrome, 3 neonatal seizures, 2 vascular anomalies, 2 inflammatory disease, 2 intracranial infection, 2 epilepsy, 2 metabolic stroke, 1 tumor, 1 drug toxicity, and 1 idiopathic intracranial hypertension).
  • Except for the presence of seizures, there were no significant differences in presentation or risk factors between benign and not-benign cases.
  • CONCLUSIONS: Many disorders mimic childhood stroke.
  • History and clinical presentation often do not distinguish the one third of patients with benign disorders from the two thirds with more serious problems, necessitating timely comprehensive investigations, especially brain MRI.
  • [MeSH-major] Brain Diseases / diagnosis. Epilepsy / diagnosis. Mental Disorders / diagnosis. Migraine Disorders / diagnosis. Stroke / diagnosis
  • [MeSH-minor] Adolescent. Brain Neoplasms / complications. Brain Neoplasms / diagnosis. Child. Child, Preschool. Cohort Studies. Consciousness Disorders / etiology. Diagnosis, Differential. Diagnostic Imaging. Female. Headache / etiology. Hemiplegia / etiology. Humans. Intracranial Hypertension / complications. Intracranial Hypertension / diagnosis. Male. Muscle Weakness / etiology. Prospective Studies. Seizures / etiology. Sensation Disorders / etiology






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