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1. Dalla Via P, Opocher E, Pinello ML, Calderone M, Viscardi E, Clementi M, Battistella PA, Laverda AM, Da Dalt L, Perilongo G: Visual outcome of a cohort of children with neurofibromatosis type 1 and optic pathway glioma followed by a pediatric neuro-oncology program. Neuro Oncol; 2007 Oct;9(4):430-7
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  • [Title] Visual outcome of a cohort of children with neurofibromatosis type 1 and optic pathway glioma followed by a pediatric neuro-oncology program.
  • We evaluated the visual outcome of a cohort of children with neurofibromatosis type 1 (NF1) and optic pathway glioma (OPG) treated according to standardized therapeutic guidelines.
  • Treatment was instituted only in cases of progressive disease or clinical deterioration.
  • Treatment modalities were chemotherapy (based on vincristine/carboplatin) for children younger than 5 years and radiotherapy for all others.
  • At a median follow-up time of 78 months, seven patients had been treated with chemotherapy only, four with radiotherapy, and four with chemotherapy plus radiotherapy.
  • Eight patients were treated for progressive visual loss in the face of stable disease, five for tumor volume increase without visual deterioration, and two for symptomatic tumor volume increase.
  • At referral, six children had a visual acuity (VA) of < 30% in both eyes; eight children had 100% VA bilaterally.
  • At referral, the visual field (VF) could be assessed in three children: One had VF loss in both eyes, one had VF loss in one eye, and one had normal VF.
  • Among 11 children who had some visual function, three had VF loss in one eye and three in both eyes, and five had an intact VF.
  • In summary, among the 15 children treated, one had a definitive and two a mild improvement in VA.
  • In conclusion, the visual outcome of this selected cohort of NF1 patients with OPG is unsatisfactory.
  • A critical reappraisal of the therapeutic strategy adopted is needed.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / adverse effects. Neurofibromatosis 1 / therapy. Optic Nerve Glioma / therapy. Radiotherapy / adverse effects. Vision Disorders / etiology
  • [MeSH-minor] Carboplatin / administration & dosage. Carboplatin / adverse effects. Child. Child, Preschool. Combined Modality Therapy. Contrast Sensitivity / drug effects. Contrast Sensitivity / radiation effects. Evoked Potentials, Visual. Female. Humans. Infant. Male. Vincristine / administration & dosage. Vincristine / adverse effects. Visual Fields / drug effects. Visual Fields / radiation effects


2. Osztie E, Várallyay P, Doolittle ND, Lacy C, Jones G, Nickolson HS, Neuwelt EA: Combined intraarterial carboplatin, intraarterial etoposide phosphate, and IV Cytoxan chemotherapy for progressive optic-hypothalamic gliomas in young children. AJNR Am J Neuroradiol; 2001 May;22(5):818-23
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  • [Title] Combined intraarterial carboplatin, intraarterial etoposide phosphate, and IV Cytoxan chemotherapy for progressive optic-hypothalamic gliomas in young children.
  • BACKGROUND AND PURPOSE: Optic pathway and/or hypothalamic astrocytomas in children are often quiescent, but in some cases, more aggressive tumors may cause progressive visual, endocrine, and neurologic deterioration.
  • The initial treatment of these gliomas includes surgery and IV chemotherapy.
  • This report suggests a new approach using combined intraarterial and IV carboplatin-based chemotherapy for patients for whom first line treatment has already failed.
  • METHODS: Six children (mean age, 57 months) with the diagnosis of optic pathway hypothalamic gliomas, who had tumor progression after surgery and underwent IV chemotherapy, were treated monthly with intraarterially administered carboplatin, intraarterially administered etoposide phosphate, and IV administered Cytoxan.
  • Administration of the intraarterial chemotherapy required catheter placement in both internal carotid arteries at the level of C2-C3 and into one of the vertebral arteries at the level of C6-C7, with the patient under general anesthesia.
  • One patient showed mild ototoxicity, and four patients needed platelet transfusion because of hematologic toxicity of drugs.
  • CONCLUSION: These results suggest that this modality of chemotherapy (administered after failure of systemic [ie, IV] chemotherapy), of progressive optic-hypothalamic astrocytomas in young children may be an effective treatment prior to radiotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Glioma / drug therapy. Hypothalamic Neoplasms / drug therapy. Visual Pathways
  • [MeSH-minor] Antineoplastic Agents / administration & dosage. Antineoplastic Agents / adverse effects. Antineoplastic Agents / therapeutic use. Antineoplastic Agents, Alkylating / administration & dosage. Antineoplastic Agents, Alkylating / adverse effects. Antineoplastic Agents, Alkylating / therapeutic use. Antineoplastic Agents, Phytogenic / administration & dosage. Antineoplastic Agents, Phytogenic / adverse effects. Antineoplastic Agents, Phytogenic / therapeutic use. Carboplatin / administration & dosage. Carboplatin / adverse effects. Carboplatin / therapeutic use. Child. Cyclophosphamide / administration & dosage. Cyclophosphamide / adverse effects. Cyclophosphamide / therapeutic use. Etoposide / administration & dosage. Etoposide / adverse effects. Etoposide / therapeutic use. Female. Humans. Infant. Infant, Newborn. Infusions, Intra-Arterial. Injections, Intravenous. Magnetic Resonance Imaging. Male. Treatment Outcome

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  • (PMID = 11337321.001).
  • [ISSN] 0195-6108
  • [Journal-full-title] AJNR. American journal of neuroradiology
  • [ISO-abbreviation] AJNR Am J Neuroradiol
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA31770; United States / NINDS NIH HHS / NS / NS33618; United States / NINDS NIH HHS / NS / NS34608
  • [Publication-type] Case Reports; Journal Article; Research Support, U.S. Gov't, Non-P.H.S.; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Antineoplastic Agents, Alkylating; 0 / Antineoplastic Agents, Phytogenic; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; BG3F62OND5 / Carboplatin
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3. Chernov MF, Ivanov PI, Zhinzhina IV, Getmanova OY, Zabrodskaya JM, Tigliev GS: Complete recovery of visual functions after multimodality treatment for intrinsic chiasmatic-hypothalamic astrocytoma--case report. Neurol Med Chir (Tokyo); 2004 Mar;44(3):129-32
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  • [Title] Complete recovery of visual functions after multimodality treatment for intrinsic chiasmatic-hypothalamic astrocytoma--case report.
  • The visual acuity was 0.02 in both eyes along with residual visual fields and central scotomas.
  • Neuroimaging disclosed a chiasmatic-hypothalamic glioma.
  • Adjuvant treatment included one course of fractionated radiation therapy and six courses of chemotherapy.
  • Complete recovery of visual acuity occurred after 10 months, and the visual fields were restored after an additional 6 months.
  • The prognosis for recovery of vision after treatment of optic pathway gliomas mainly depends on the severity of visual loss at admission and is negatively influenced by intrinsic tumor growth, symmetrical extension, and involvement of the chiasm.
  • Despite the presence of all these factors in the present case, multimodality management resulted in the complete recovery of visual functions.
  • Surgery may be indicated in cases of intrinsic chiasmatic gliomas complicated by severe visual loss.
  • [MeSH-major] Astrocytoma / therapy. Hypothalamic Neoplasms / therapy. Optic Chiasm. Optic Nerve Neoplasms / therapy. Vision Disorders / therapy
  • [MeSH-minor] Adult. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Treatment Outcome. Visual Acuity

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  • (PMID = 15095966.001).
  • [ISSN] 0470-8105
  • [Journal-full-title] Neurologia medico-chirurgica
  • [ISO-abbreviation] Neurol. Med. Chir. (Tokyo)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
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4. Moreno L, Bautista F, Ashley S, Duncan C, Zacharoulis S: Does chemotherapy affect the visual outcome in children with optic pathway glioma? A systematic review of the evidence. Eur J Cancer; 2010 Aug;46(12):2253-9
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  • [Title] Does chemotherapy affect the visual outcome in children with optic pathway glioma? A systematic review of the evidence.
  • INTRODUCTION: Overall prognosis for optic pathway glioma (OPG) in children is excellent.
  • Little is known, however, about the effect of chemotherapy on visual outcome of these patients.
  • MATERIAL AND METHODS: A systematic review of the literature was carried out to identify all studies published in PubMed, EMBASE and Cochrane Library between 1990 and 2008 reporting visual outcome in children with OPG after chemotherapy.
  • Studies were included when they included 10 or more children with OPG, main treatment was chemotherapy and visual outcome was described.
  • Only 25 of 174 children experienced improvement in vision after chemotherapy (14.4%), responses ranged from 0% to 45.5%.
  • No study documented the duration of the visual response.
  • DISCUSSION: Published studies on childhood low grade gliomas have not shown satisfactorily whether chemotherapy improves outcome of vision in children with OPG.
  • Based on our systematic review it appears that treatment with chemotherapy does not improve resulting vision in the majority of children with OPG.
  • The data available does not allow us to assess whether vision is stabilised sufficiently prior to treatment with radiotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Optic Nerve Glioma / drug therapy. Vision Disorders / drug therapy
  • [MeSH-minor] Child. Child, Preschool. Humans. Infant. Treatment Outcome. Visual Acuity / physiology

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  • [Copyright] Copyright 2010 Elsevier Ltd. All rights reserved.
  • (PMID = 20400294.001).
  • [ISSN] 1879-0852
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
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5. Gururangan S, Fisher MJ, Allen JC, Herndon JE 2nd, Quinn JA, Reardon DA, Vredenburgh JJ, Desjardins A, Phillips PC, Watral MA, Krauser JM, Friedman AH, Friedman HS: Temozolomide in children with progressive low-grade glioma. Neuro Oncol; 2007 Apr;9(2):161-8
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  • [Title] Temozolomide in children with progressive low-grade glioma.
  • We conducted a phase II study to assess the efficacy of oral temozolomide (TMZ) in children with progressive low-grade glioma.
  • Patients received a median of nine cycles (range, 2-12 cycles) of treatment.
  • Best responses in the 26 patients (86%) with optic pathway glioma (OPG)/pilocytic astrocytoma (PA) included partial response in 3 patients (11%), minor response in 1 (4%), stable disease in 10 (38%), and progressive disease in 12 (46%).
  • Seventeen of 26 patients had progressive disease either on or off therapy, and three have died of disease.
  • [MeSH-major] Antineoplastic Agents, Alkylating / therapeutic use. Brain Neoplasms / drug therapy. Dacarbazine / analogs & derivatives. Glioma / drug therapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Drug Administration Schedule. Female. Humans. Male. Survival Analysis. Survivors. Time Factors. Treatment Outcome

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  • (PMID = 17347491.001).
  • [ISSN] 1522-8517
  • [Journal-full-title] Neuro-oncology
  • [ISO-abbreviation] Neuro-oncology
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Multicenter Study
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide
  • [Other-IDs] NLM/ PMC1871667
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6. Demaerel P, de Ruyter N, Casteels I, Renard M, Uyttebroeck A, van Gool S: Visual pathway glioma in children treated with chemotherapy. Eur J Paediatr Neurol; 2002;6(4):207-12
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  • [Title] Visual pathway glioma in children treated with chemotherapy.
  • Visual pathway gliomas occur predominantly in young children.
  • Chemotherapy has been increasingly used as a first-line treatment because of the complications caused by radiotherapy and surgery.
  • Nine children between 6 months and 9 years (median age of 4.8 years) were treated with vincristine and carboplatin according to the SIOP (Société Internationale d'Oncologie Pédiatrique) low-grade glioma 1996 protocol.
  • Five patients had evidence of neurofibromatosis type 1.
  • Magnetic resonance imaging (MRI) and ophthalmological assessment were performed during and after treatment.
  • Three patients developed progressive disease between 8 and 12 months after ceasing treatment.
  • One of them, being only 2.5 years old, was again treated by chemotherapy with partial response on MRI.
  • Patients with neurofibromatosis type 1 never developed progressive disease.
  • Our data suggest that chemotherapy is an effective treatment option in young children with visual pathway gliomas.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Brain Neoplasms / pathology. Carboplatin / therapeutic use. Glioma / drug therapy. Glioma / pathology. Vincristine / therapeutic use. Visual Pathways / pathology
  • [MeSH-minor] Child. Child, Preschool. Female. Follow-Up Studies. Humans. Infant. Magnetic Resonance Imaging. Male. Visual Acuity / physiology. Visual Fields / physiology

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  • (PMID = 12374587.001).
  • [ISSN] 1090-3798
  • [Journal-full-title] European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society
  • [ISO-abbreviation] Eur. J. Paediatr. Neurol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; BG3F62OND5 / Carboplatin
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7. Pascual-Castroviejo I, Pascual-Pascual SI, Velázquez-Fragua R, Viaño J, García-Segura JM, Botella MP: [Neurofibromatosis type 1 and optic pathway gliomas. A series of 80 patients]. Rev Neurol; 2008 May 1-15;46(9):530-6
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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 optic pathway gliomas. A series of 80 patients].
  • PATIENTS AND METHODS: From a series of 530 patients with neurofibromatosis type 1 (NF1), we performed a retrospective assessment of the long-term neurologic, visual, neuroimaging and evolution of 80 patients (15%) with optic pathway gliomas (OPG).
  • RESULTS: Image studies showed the distribution of the lesions among optic nerves, chiasm, tracts and radiations demonstrated that only 25% of the tumors involved only one optic nerve and 11.5% were located only in the chiasm, while 40% involved one or both optic nerves and chiasm, tracts and radiations.
  • Late diagnosis (after 7 years of age) of OPG was made in three patients and late progression was evident in three others who required surgical resection, radiotherapy or chemotherapy.
  • [MeSH-major] Neoplasms, Multiple Primary / diagnosis. Neurofibromatosis 1 / diagnosis. Optic Nerve Glioma / diagnosis

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  • (PMID = 18446694.001).
  • [ISSN] 1576-6578
  • [Journal-full-title] Revista de neurologia
  • [ISO-abbreviation] Rev Neurol
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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8. Campagna M, Opocher E, Viscardi E, Calderone M, Severino SM, Cermakova I, Perilongo G: Optic pathway glioma: long-term visual outcome in children without neurofibromatosis type-1. Pediatr Blood Cancer; 2010 Dec 1;55(6):1083-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Optic pathway glioma: long-term visual outcome in children without neurofibromatosis type-1.
  • BACKGROUND: Little is known about the visual outcome of children affected by an optic pathway glioma (OPG).
  • PROCEDURES: We evaluated the long-term visual outcome of 32 consecutive children affected by OPG without neurofibromatosis type-1 referred to the Pediatric Department of Padua University and managed according to standardized strategies.
  • RESULTS: Eight children received chemotherapy, 10 radiotherapy, 7 both chemotherapy and radiotherapy, whereas 7 were untreated.
  • At presentation, visual acuity (VA) was normal in 22 children (13 unilaterally and 9 bilaterally), and reduced in 10.
  • Visual field, assessed in 29 children, was normal in 9 and reduced in 20.
  • The number of children with some grade of visual impairment increased from 7 to 10 during follow-up.
  • CONCLUSIONS: The visual prognosis of children with OPG is unsatisfactory.
  • Older children treated with radiotherapy seem to have a better visual outcome than younger children.
  • Severe optic pallor at diagnosis or during follow-up may be indicative of a negative prognosis.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Optic Nerve Glioma / physiopathology. Optic Nerve Neoplasms / physiopathology. Visual Acuity / physiology. Visual Fields / physiology. Visual Pathways / physiopathology
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Neurofibromatosis 1 / physiopathology. Radiotherapy Dosage. Treatment Outcome

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  • (PMID = 20979170.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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9. Kaufman LM, Doroftei O: Optic glioma warranting treatment in children. Eye (Lond); 2006 Oct;20(10):1149-64
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Optic glioma warranting treatment in children.
  • PURPOSE: To describe cases of optic pathway glioma (OPG) warranting treatment in children.
  • Three of the patients were also ultimately diagnosed with neurofibromatosis type 1.
  • Six of the patients were treated with intravenous chemotherapy, with three patients requiring a second chemotherapy cycle.
  • One patient was successfully treated with an en-bloc optic nerve excision.
  • Both chemotherapy and en-bloc excision can be employed for treatment.
  • [MeSH-major] Optic Nerve Glioma / therapy
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child, Preschool. Disease Progression. Exophthalmos / etiology. Eye Enucleation. Female. Humans. Infant. Magnetic Resonance Imaging. Male. Neoplasm Invasiveness. Neurofibromatosis 1 / complications. Neurofibromatosis 1 / diagnosis. Neurofibromatosis 1 / pathology. Neurofibromatosis 1 / therapy. Optic Nerve / pathology. Optic Nerve / surgery. Retrospective Studies. Treatment Outcome. Vision Disorders / etiology

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  • (PMID = 17019413.001).
  • [ISSN] 0950-222X
  • [Journal-full-title] Eye (London, England)
  • [ISO-abbreviation] Eye (Lond)
  • [Language] eng
  • [Grant] United States / NEI NIH HHS / EY / EY 1792
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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10. Burzynski SR, Weaver RA, Lewy RI, Janicki TJ, Jurida GF, Szymkowski BG, Khan MI, Bestak M: Phase II study of antineoplaston A10 and AS2-1 in children with recurrent and progressive multicentric glioma : a preliminary report. Drugs R D; 2004;5(6):315-26
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  • [Title] Phase II study of antineoplaston A10 and AS2-1 in children with recurrent and progressive multicentric glioma : a preliminary report.
  • OBJECTIVE: To evaluate the response rates, survival and toxicity of treatment with antineoplaston A10 and AS2-1 (ANP) in the first 12 children enrolled in our studies diagnosed with incurable recurrent and progressive multicentric glioma.
  • In one case of visual pathway glioma, a biopsy was not performed due to a dangerous location.
  • The average duration of intravenous ANP therapy was 16 months and the average dosage of A10 was 7.95 g/kg/day and of AS2-1 was 0.33 g/kg/day.
  • CONCLUSION: The results of the present study are favourable in comparison with radiation therapy and chemotherapy.
  • We believe that confirmation of these results through further studies may introduce a new promising treatment for incurable paediatric brain tumours.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Benzeneacetamides / therapeutic use. Brain Neoplasms / drug therapy. Glioma / drug therapy. Glutamine / analogs & derivatives. Glutamine / therapeutic use. Phenylacetates / therapeutic use. Piperidones / therapeutic use
  • [MeSH-minor] Adolescent. Astrocytoma / drug therapy. Astrocytoma / pathology. Child. Child, Preschool. Disease Progression. Drug Combinations. Drug Therapy, Combination. Female. Humans. Infant. Magnetic Resonance Imaging. Male. Neoplasm Recurrence, Local / prevention & control. Survival Analysis. Treatment Outcome

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  • (PMID = 15563234.001).
  • [ISSN] 1174-5886
  • [Journal-full-title] Drugs in R&D
  • [ISO-abbreviation] Drugs R D
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] New Zealand
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzeneacetamides; 0 / Drug Combinations; 0 / Phenylacetates; 0 / Piperidones; 0RH81L854J / Glutamine; 104624-98-8 / antineoplaston AS 2-1; 91531-30-5 / antineoplaston A10
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11. Pepin SM, Lessell S: Anterior visual pathway gliomas: The last 30 years. Semin Ophthalmol; 2006 Jul-Sep;21(3):117-24
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  • [Title] Anterior visual pathway gliomas: The last 30 years.
  • Modern neuroimaging provides excellent characterization of anterior visual pathway gliomas, often obviating the need for biopsy of the tumor.
  • Management remains controversial, but if there is progression, chemotherapy is preferred for young patients.
  • A mouse model of NF-1 with optic pathway gliomas has the potential to provide important insights into the development of gliomas as well as serving as a model for their effective treatment.
  • [MeSH-major] Glioma / pathology. Optic Nerve Neoplasms / pathology. Visual Pathways / pathology
  • [MeSH-minor] Animals. Antineoplastic Agents / therapeutic use. Disease Models, Animal. Humans. Neurofibromatosis 1 / drug therapy. Neurofibromatosis 1 / pathology. Neurofibromatosis 1 / radiotherapy. Optic Chiasm / drug effects. Optic Chiasm / pathology. Optic Chiasm / radiation effects. Radiotherapy, Conformal

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  • (PMID = 16912009.001).
  • [ISSN] 0882-0538
  • [Journal-full-title] Seminars in ophthalmology
  • [ISO-abbreviation] Semin Ophthalmol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 55
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12. Liu GT: Optic gliomas of the anterior visual pathway. Curr Opin Ophthalmol; 2006 Oct;17(5):427-31
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  • [Title] Optic gliomas of the anterior visual pathway.
  • PURPOSE OF REVIEW: To review advances in the diagnosis and management of optic-pathway gliomas made within the past 5 years.
  • RECENT FINDINGS: Important papers regarding optic-pathway gliomas have been published recently in the following areas: neuroimaging, natural history and how the presence of neurofibromatosis type 1 affects it, unusual presentations, visual prognosis, and treatment with fractionated stereotactic radiotherapy.
  • SUMMARY: The diagnosis and treatment of optic-pathway gliomas has been aided greatly by a greater understanding of the natural history of these tumors and their prognosis related to the presence of neurofibromatosis type 1.
  • Newer radiation techniques that spare surrounding tissues are being used to treat optic-pathway gliomas, but chemotherapy has become the first-line treatment modality.
  • [MeSH-major] Glioma / pathology. Optic Chiasm / pathology. Optic Nerve Neoplasms / pathology. Visual Pathways / pathology

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  • (PMID = 16932058.001).
  • [ISSN] 1040-8738
  • [Journal-full-title] Current opinion in ophthalmology
  • [ISO-abbreviation] Curr Opin Ophthalmol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 25
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13. Peng F, Juhasz C, Bhambhani K, Wu D, Chugani DC, Chugani HT: Assessment of progression and treatment response of optic pathway glioma with positron emission tomography using alpha-[(11)C]methyl-L-tryptophan. Mol Imaging Biol; 2007 May-Jun;9(3):106-9
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  • [Title] Assessment of progression and treatment response of optic pathway glioma with positron emission tomography using alpha-[(11)C]methyl-L-tryptophan.
  • PURPOSE: To report the utility of positron emission tomography (PET) with alpha-[(11)C]methyl-L-tryptophan (AMT) for monitoring progression and response to treatment of an isolated optic pathway glioma (OPG) in a 16-year-old girl.
  • PROCEDURES: Positron emission tomography scanning of the brain was performed 20 minutes after intravenous administration of AMT.
  • The tracer uptake was dramatically decreased on the images obtained after chemotherapy.
  • Subsequently, AMT-PET revealed a new tumor lesion of increased AMT uptake when the patient developed vision problems and MRI showed no significant interval morphological changes.
  • CONCLUSIONS: Positron emission tomography with alpha-[(11)C]methyl-L-tryptophan may be useful for monitoring progression and response to treatment of OPGs, which needs to be further investigated in a prospective study of more patients, including those with neurofibromatosis.
  • [MeSH-major] Carbon Radioisotopes. Optic Nerve Glioma / diagnostic imaging. Positron-Emission Tomography / methods. Radiopharmaceuticals. Tryptophan / analogs & derivatives

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  • (PMID = 17318667.001).
  • [ISSN] 1536-1632
  • [Journal-full-title] Molecular imaging and biology : MIB : the official publication of the Academy of Molecular Imaging
  • [ISO-abbreviation] Mol Imaging Biol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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14. Stieber VW: Radiation therapy for visual pathway tumors. J Neuroophthalmol; 2008 Sep;28(3):222-30
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  • [Title] Radiation therapy for visual pathway tumors.
  • The multimodality management of visual pathway tumors frequently involves radiation.
  • Most commonly, photons are delivered via multiple focused beams aimed at the tumor while sparing adjacent tissues.
  • The dose can be delivered in multiple treatments (radiation therapy) or in a single treatment (radiosurgery).
  • Children with visual pathway gliomas should be treated with chemotherapy alone, delaying the use of radiation therapy until progression.
  • Definitive radiation therapy of optic nerve sheath meningiomas results in stable vision in most patients.
  • Radiation therapy or radiosurgery for pituitary tumors can result in control of both tumor growth and hormone hypersecretion.
  • Postoperative radiation therapy or radiosurgery of craniopharyngiomas significantly improves local control rates compared with surgery alone.
  • Radiation therapy is highly effective for eradicating orbital pseudolymphoma and lymphoma.
  • The risk of complications from radiation treatment is dependent on the organ at risk, the cumulative dose it receives, and the dose delivered per fraction.
  • [MeSH-major] Optic Nerve / radiation effects. Optic Nerve Diseases / radiotherapy. Optic Nerve Glioma / radiotherapy. Radiotherapy / methods

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  • (PMID = 18769290.001).
  • [ISSN] 1536-5166
  • [Journal-full-title] Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
  • [ISO-abbreviation] J Neuroophthalmol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 106
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15. Wabbels B, Demmler A, Seitz J, Woenckhaus M, Bloss HG, Lorenz B: Unilateral adult malignant optic nerve glioma. Graefes Arch Clin Exp Ophthalmol; 2004 Sep;242(9):741-8
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  • [Title] Unilateral adult malignant optic nerve glioma.
  • INTRODUCTION: Adult malignant optic nerve gliomas are rare and rapidly fatal visual pathway tumours.
  • They represent a clinical entity different from the more common childhood benign optic nerve gliomas, which are frequently associated with neurofibromatosis I.
  • CASE REPORT: A 61-year-old woman presented with rapidly progressing right vision loss, lower altitudinal visual field defect and papilloedema.
  • MRI showed intraorbital and intracranial swelling of the right optic nerve.
  • Resection of the intracranial part of the right optic nerve up to the chiasm revealed anaplastic astrocytoma grade III.
  • Clinical and MRI evaluation of the left eye and optic nerve were normal at all times.
  • DISCUSSION: Unilateral adult malignant glioma of the optic nerve is exceptional.
  • The final diagnosis was only confirmed by optic nerve biopsy.
  • To date, 44 case reports of adult malignant optic nerve glioma have been published, either malignant astrocytoma or glioblastoma.
  • These tumours can mimic optic neuritis in their initial presentation.
  • On MRI images, malignant glioma cannot be distinguished from optic nerve enlargement due to other causes.
  • Although radiotherapy appears to prolong life expectancy, all presently available treatment options (radiation, surgery, radio-chemotherapy) are of limited value.
  • [MeSH-major] Optic Nerve Glioma / pathology. Optic Nerve Neoplasms / pathology
  • [MeSH-minor] Fatal Outcome. Female. Humans. Magnetic Resonance Imaging. Middle Aged. Papilledema / diagnosis. Vision Disorders / diagnosis. Visual Fields

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  • (PMID = 15085353.001).
  • [ISSN] 0721-832X
  • [Journal-full-title] Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie
  • [ISO-abbreviation] Graefes Arch. Clin. Exp. Ophthalmol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Germany
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16. Lee AG: Neuroophthalmological management of optic pathway gliomas. Neurosurg Focus; 2007;23(5):E1
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Neuroophthalmological management of optic pathway gliomas.
  • The growth rate of optic pathway gliomas (OPGs) is unpredictable and quite variable, especially in children with neurofibromatosis Type 1 (NF1).
  • Typically, only symptomatic and/or radiographically growing tumors require treatment, and observation is the accepted first-line option.
  • Although both chemotherapy and radiotherapy can stabilize growth or even decrease the size of tumors, chemotherapy, especially in younger patients, has fewer side effects than radiation therapy (such as secondary tumors, radiation necrosis, and Moyomoya disease) and is generally considered the first-line treatment for progressive lesions in younger patients.
  • The tumor location defines prognosis in OPGs; optic nerve gliomas (ONG) have the lowest rate of complications and death, and optic chiasm and retrochiasmal gliomas the highest.
  • Although the major complication of an OPG is visual loss, hypothalamic involvement can lead to death.
  • Resection is an option for ONGs but is generally reserved for tumors confined to the optic nerve with poor or no vision, or for patients with severe, cosmetically unappealing proptosis, producing severe pain or exposure keratopathy in a blind eye.
  • The approach to a patient with OPG must be individualized based on tumor location, radiographic or clinical progression, the presence of NF1, and a risk-benefit comparison for treatment.
  • [MeSH-major] Glioma / diagnosis. Glioma / therapy. Optic Nerve Neoplasms / diagnosis. Optic Nerve Neoplasms / therapy
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Child. Disease Progression. Humans. Magnetic Resonance Imaging. Neoplasm Recurrence, Local. Neurofibromatosis 1 / complications. Optic Chiasm. Practice Guidelines as Topic

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  • (PMID = 18004957.001).
  • [ISSN] 1092-0684
  • [Journal-full-title] Neurosurgical focus
  • [ISO-abbreviation] Neurosurg Focus
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 80
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17. Massimino M, Spreafico F, Cefalo G, Riccardi R, Tesoro-Tess JD, Gandola L, Riva D, Ruggiero A, Valentini L, Mazza E, Genitori L, Di Rocco C, Navarria P, Casanova M, Ferrari A, Luksch R, Terenziani M, Balestrini MR, Colosimo C, Fossati-Bellani F: High response rate to cisplatin/etoposide regimen in childhood low-grade glioma. J Clin Oncol; 2002 Oct 15;20(20):4209-16
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  • [Title] High response rate to cisplatin/etoposide regimen in childhood low-grade glioma.
  • PURPOSE: The aim of this study was to avoid radiotherapy and to induce an objective response in children with low-grade glioma (LGG) using a simple chemotherapy regimen based on cisplatin and etoposide.
  • Tumor originated in the visual pathway in 29 patients, in the temporal lobe in two, in the frontal lobe in two, and in the spine in one.
  • Eight children were affected by neurofibromatosis type 1.
  • CONCLUSION: Cisplatin and etoposide combined treatment is one of the most active regimens for LGG in children and allows avoidance of radiotherapy in the vast majority of patients.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Central Nervous System Neoplasms / drug therapy. Glioma / drug therapy

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  • (PMID = 12377964.001).
  • [ISSN] 0732-183X
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; Q20Q21Q62J / Cisplatin
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18. Lena G, Pech-Gourg G, Scavarda D, Klein O, Paz-Paredes A: [Optic nerve glioma in children]. Neurochirurgie; 2010 Apr-Jun;56(2-3):249-56
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  • [Title] [Optic nerve glioma in children].
  • [Transliterated title] Gliome du nerf optique chez l'enfant.
  • Optic pathway gliomas are rare tumors accounting for 3-5% of brain tumors in children; 90% are observed in children.
  • Association with NF 1 is classical and the incidence of NF 1 in patients with optic pathway gliomas is estimated at 30-58%.
  • Exophthalmos and loss of visual acuity or blindness are the usualpresentation in progressive disease.
  • Chemotherapy must be proposed as first-line treatment for growing tumor with moderate exophthalmos and useful vision when the tumor is strictly confined in the orbit.
  • The place of radiotherapy for pure intraorbital gliomas has not been defined and proton beam therapy has to be evaluated.
  • [MeSH-major] Glioma / epidemiology. Optic Nerve Neoplasms / epidemiology
  • [MeSH-minor] Adult. Antineoplastic Agents / therapeutic use. Blindness / etiology. Brain / pathology. Child. Child, Preschool. Exophthalmos / drug therapy. Exophthalmos / epidemiology. Exophthalmos / surgery. Female. Humans. Incidence. Infant. Magnetic Resonance Imaging. Male. Neurofibromatosis 1 / complications. Neurofibromatosis 1 / epidemiology. Neurofibromatosis 1 / pathology. Neurofibromatosis 1 / surgery. Orbit / pathology. Tomography, X-Ray Computed. Visual Acuity

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  • [Copyright] Copyright 2010 Elsevier Masson SAS. All rights reserved.
  • (PMID = 20303553.001).
  • [ISSN] 1773-0619
  • [Journal-full-title] Neuro-Chirurgie
  • [ISO-abbreviation] Neurochirurgie
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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19. Shofty B, Constantini S, Freedman S, Ben-Sira L, Kesler A: [Optic pathway gliomas--current position and future directions]. Harefuah; 2010 Nov;149(11):721-5, 748

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Optic pathway gliomas--current position and future directions].
  • Optic pathway gliomas (OPG) are the most common primary tumors of the visual pathways, and constitute 1% of all brain tumors and 5% of all brain tumors in children.
  • Among Neurofibromatosis type 1 patients (a hereditary genetic disorder which is characterized by higher incidence of tumors from a neurocutaneous origin) it is the most frequent tumor and it constitutes between 15 to 20 percent of all nervous system tumors.
  • These tumors are stable most of the time and remain indolent for many years after diagnosis, especially in patients suffering from Neurofibromatosis type 1.
  • However, amongst some of the patients suffering from OPG, these tumors develop progressive characteristics and can cause visual disturbances, endocrine dysfunction, blindness and even death.
  • Patients with aggressive tumors will need treatment, which can be either surgery, chemotherapy or radiation therapy.
  • Today, the treating physicians face substantial difficulty in estimating the course the tumor will take, choosing the right candidates for oncological treatment and the type of therapy most suited to the case, due to lack of reliable information in the relevant literature.
  • This article characterizes the tumors, presents updates from recent literature, as well as recommendations for treatment and follow-up.
  • [MeSH-major] Neurofibromatosis 1 / surgery. Optic Nerve Glioma / surgery
  • [MeSH-minor] Adolescent. Child. Glioma / drug therapy. Glioma / radiography. Glioma / radiotherapy. Glioma / surgery. Humans. Magnetic Resonance Imaging

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  • (PMID = 21250414.001).
  • [ISSN] 0017-7768
  • [Journal-full-title] Harefuah
  • [ISO-abbreviation] Harefuah
  • [Language] heb
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Israel
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20. Binning MJ, Liu JK, Kestle JR, Brockmeyer DL, Walker ML: Optic pathway gliomas: a review. Neurosurg Focus; 2007;23(5):E2
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Optic pathway gliomas: a review.
  • Optic pathway gliomas represent approximately 3-5% of childhood intracranial tumors.
  • They usually occur in children during the first decade of life and are seen in 11-30% of patients with neurofibromatosis Type 1 (NF1).
  • Although these tumors are typically low-grade gliomas, the clinical course and natural history are highly variable, making treatment paradigms difficult.
  • Overall, however, they are often indolent tumors that can be observed over time for progression without initial treatment, especially in patients with NF1.
  • Chemotherapy is the first-line treatment for progressive tumors, and radiation therapy is reserved for patients with progressive disease who are older than 5-7 years.
  • Surgery is reserved for large tumors causing mass effect or hydrocephalus and tumors confined to the orbit or unilateral optic nerve.
  • [MeSH-major] Glioma / diagnosis. Glioma / therapy. Optic Nerve Neoplasms / diagnosis. Optic Nerve Neoplasms / therapy
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Child. Child, Preschool. Diagnostic Imaging / methods. Female. Humans. Male. Prognosis. Radiotherapy, Adjuvant. Visual Acuity

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  • [CommentOn] Neurosurg Focus. 2005 Jun 15;18(6A):E7 [16048293.001]
  • (PMID = 18004964.001).
  • [ISSN] 1092-0684
  • [Journal-full-title] Neurosurgical focus
  • [ISO-abbreviation] Neurosurg Focus
  • [Language] eng
  • [Publication-type] Comment; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 85
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21. Walker D: Recent advances in optic nerve glioma with a focus on the young patient. Curr Opin Neurol; 2003 Dec;16(6):657-64
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Recent advances in optic nerve glioma with a focus on the young patient.
  • PURPOSE OF REVIEW: Optic tract low-grade gliomas are one of the commonest category of neoplasm presenting in childhood and early adolescence.
  • Recent reports covering their aetiology, imaging techniques, the application of novel radiotherapy techniques and chemotherapy are reported.
  • Increasing numbers of trials of chemotherapy agents are demonstrating efficacy in this tumour category.
  • SUMMARY: Visual pathway low-grade astrocytomas of childhood are the subject of diverse research into diagnostic aetiological and treatment aspects aimed at tailoring diagnostic and treatment procedures more precisely to the needs of the young patient for this tumour type.
  • [MeSH-major] Genetic Predisposition to Disease / genetics. Neurofibromatosis 1 / complications. Optic Nerve / pathology. Optic Nerve Glioma / genetics. Optic Nerve Glioma / pathology
  • [MeSH-minor] Child. Drug Therapy / standards. Humans. Neoplasm Invasiveness / pathology. Neoplasm Invasiveness / radiography. Pedigree. Predictive Value of Tests. Radiotherapy / standards

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  • (PMID = 14624073.001).
  • [ISSN] 1350-7540
  • [Journal-full-title] Current opinion in neurology
  • [ISO-abbreviation] Curr. Opin. Neurol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 20
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22. Suárez JC, Viano JC, Zunino S, Herrera EJ, Gomez J, Tramunt B, Marengo I, Hiramatzu E, Miras M, Pena M, Sonzini Astudillo B: Management of child optic pathway gliomas: new therapeutical option. Childs Nerv Syst; 2006 Jul;22(7):679-84

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Management of child optic pathway gliomas: new therapeutical option.
  • OBJECTIVE: To present our experience in the treatment of child optic pathway gliomas in the last 25 years.
  • The most frequent symptoms have been ophthalmologic and visual alterations in all 17 patients, endocrine alterations in 10, and neurological signs in 6.
  • One of the patients presented neurofibromatosis type 1 (NF1), another patient had Down syndrome.
  • Diagnosed using computed tomography or/and magnetic resonance imaging, histological studies showed pilocytic astrocytomas in 13 cases and a fibrillary astrocytoma grade II in 1 case.
  • The treatment consisted of surgery, external beam radiotherapy, chemotherapy, and brachytherapy with iodine 125, separately or combined.
  • Five patients died; the causes were secondary tumors in two children, tumor recurrence in one, sepsis secondary to respiratory and urinary tract infections in the child with Down syndrome, and finally, hydrocephaly due to hyperproteinorachia of tumor origin in one.
  • CONCLUSION: Chemotherapy and brachytherapy are therapeutic methods to be considered, especially in children under 5.
  • Marsupialization of the residual cyst into the ventricular system postradio or oncolytic treatment through endoscopic or stereotactic techniques is useful in the treatment of endocranial hypertension and/or hypothalamic compression in these patients.
  • [MeSH-major] Glioma / therapy. Optic Nerve Glioma / therapy. Optic Nerve Neoplasms / therapy
  • [MeSH-minor] Child. Child, Preschool. Female. Humans. Infant. Magnetic Resonance Imaging / methods. Male. Neurosurgery. Radiotherapy. Tomography, X-Ray Computed / methods

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  • (PMID = 16389565.001).
  • [ISSN] 0256-7040
  • [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] Clinical Trial; Comparative Study; Journal Article
  • [Publication-country] Germany
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23. Jaing TH, Lin KL, Tsay PK, Hsueh C, Hung PC, Wu CT, Tseng CK: Treatment of optic pathway hypothalamic gliomas in childhood: experience with 18 consecutive cases. J Pediatr Hematol Oncol; 2008 Mar;30(3):222-4

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment of optic pathway hypothalamic gliomas in childhood: experience with 18 consecutive cases.
  • The aim of this study was to present our 17-year experience (1989 to 2006) in the treatment of optic pathway/hypothalamic gliomas (OPHG) in 18 children younger than 17 years (median age, 66 mo).
  • OPHG was diagnosed using computed tomography and/or magnetic resonance imaging.
  • Treatment included partial tumor resection in 12 patients, chemotherapy in 5, and radiotherapy in 3.
  • Ophthalmologic and visual alterations occurred in 12 patients, endocrine alterations in 6, and neurologic signs in 5.
  • All treatment modalities led to tumor shrinkage and stabilization for a variable period, but none of them totally eradicated the tumor.
  • Because progression of these tumors is slow and associated with endocrinopathy, we recommend chemotherapy as a primary treatment of OPHG if the disease progresses.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Hypothalamic Neoplasms / therapy. Optic Nerve Glioma / therapy. Visual Pathways / pathology
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Disease Progression. Female. Follow-Up Studies. Humans. Infant. Magnetic Resonance Imaging. Male. Predictive Value of Tests. Retrospective Studies. Survival Rate. Treatment Outcome

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  • (PMID = 18376285.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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24. Jahraus CD, Tarbell NJ: Optic pathway gliomas. Pediatr Blood Cancer; 2006 May 1;46(5):586-96
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Optic pathway gliomas.
  • Optic pathway gliomas represent approximately 5% of all pediatric intracranial tumors.
  • Their propensity to occur in very young children and infants further complicates selection of therapy.
  • Historically, surgery and radiotherapy have played a primary role in management, however, in the last 15 years, chemotherapy has evolved into the first-line treatment of choice.
  • Nonetheless, chemotherapy frequently fails, but serves to delay implementation of radiotherapy or surgery until the child has progressed neuropsychologically.
  • An overall favorable prognosis for this tumor emphasizes the need for careful selection of therapy.
  • Herein, we review the major features of optic pathway glioma, including epidemiology, pathology, therapeutic interventions, outcome, and treatment sequelae.
  • [MeSH-major] Cranial Nerve Neoplasms. Optic Nerve Glioma
  • [MeSH-minor] Combined Modality Therapy. Humans. Treatment Outcome

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  • (PMID = 16411210.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 110
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25. Kortmann RD, Timmermann B, Taylor RE, Scarzello G, Plasswilm L, Paulsen F, Jeremic B, Gnekow AK, Dieckmann K, Kay S, Bamberg M: Current and future strategies in radiotherapy of childhood low-grade glioma of the brain. Part I: Treatment modalities of radiation therapy. Strahlenther Onkol; 2003 Aug;179(8):509-20
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Current and future strategies in radiotherapy of childhood low-grade glioma of the brain. Part I: Treatment modalities of radiation therapy.
  • BACKGROUND: Treatment of childhood low-grade gliomas is a challenging issue owing to their low incidence and the lack of consensus about "optimal" treatment approach.
  • MATERIAL AND METHODS: Reports in the literature spanning 60 years of radiation therapy, including orthovoltage, megavoltage and recently modern high-precision treatments, were reviewed with respect to visual function, survival, prognostic factors, dose prescriptions, target volumes, and treatment techniques.
  • Based on these experiences, future strategies in the management of childhood low-grade glioma are presented.
  • Even with the shortcomings of the reports available in the literature, primarily concerning indications, age at treatment, dose response, timing and use of "optimal" treatment fields, radiation therapy continues to play an important role in the management of these tumors achieving long-term survival rates up to 80% or more.
  • Particularly in gliomas of the visual pathway, high local tumor control and improved or stable visual function is achieved in approximately 90% of cases.
  • Data on dose-response relationships recommend dose prescriptions between 45 and 54 Gy with standard fractionation.
  • There is consensus now to employ radiation therapy in older children in case of progressive disease only, regardless of tumor location and histologic subtype.
  • Recent advances in treatment techniques, such as 3-D treatment planning and various "high-precision" treatments achieved promising initial outcome, however with limited patient numbers and short follow-ups.
  • CONCLUSIONS: Radiation therapy is an effective treatment modality in children with low-grade glioma regarding tumor control and improvement and/or preservation of neurologic function or vision, respectively.
  • More prospective studies are needed to address the impact of modern radiation therapy technologies (including intensity-modulated radiotherapy) on outcome especially in the very young and to define the role of radiation therapy as a part of a comprehensive treatment approach.
  • [MeSH-major] Brain Neoplasms / radiotherapy. Glioma / radiotherapy. Neurofibromatoses / radiotherapy. Optic Nerve Neoplasms / radiotherapy
  • [MeSH-minor] Adolescent. Adult. Age Factors. Astrocytoma / drug therapy. Astrocytoma / radiotherapy. Astrocytoma / surgery. Brachytherapy. Cerebellar Neoplasms / drug therapy. Cerebellar Neoplasms / radiotherapy. Cerebellar Neoplasms / surgery. Child. Child, Preschool. Clinical Trials as Topic. Combined Modality Therapy. Disease-Free Survival. Dose Fractionation. Dose-Response Relationship, Radiation. Follow-Up Studies. Humans. Hypothalamus. Medulloblastoma / drug therapy. Medulloblastoma / radiotherapy. Medulloblastoma / surgery. Optic Chiasm. Postoperative Care. Prognosis. Protons / therapeutic use. Radiotherapy Dosage. Radiotherapy Planning, Computer-Assisted. Radiotherapy, Conformal. Retrospective Studies. Survival Analysis. Time Factors. Vision, Ocular. Visual Pathways

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  • (PMID = 14509949.001).
  • [ISSN] 0179-7158
  • [Journal-full-title] Strahlentherapie und Onkologie : Organ der Deutschen Röntgengesellschaft ... [et al]
  • [ISO-abbreviation] Strahlenther Onkol
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Protons
  • [Number-of-references] 77
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26. Diaz RJ, Laughlin S, Nicolin G, Buncic JR, Bouffet E, Bartels U: Assessment of chemotherapeutic response in children with proptosis due to optic nerve glioma. Childs Nerv Syst; 2008 Jun;24(6):707-12
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Assessment of chemotherapeutic response in children with proptosis due to optic nerve glioma.
  • INTRODUCTION: Some children with optic pathway gliomas present with proptosis related to intraorbital tumor extension.
  • A series of six patients with proptosis and the diagnosis of an optic nerve tumor from an optic pathway glioma registry demonstrate by case example the correlation between the proptosis index and the clinical and radiographic response to chemotherapy.
  • [MeSH-major] Drug Therapy / methods. Exophthalmos / drug therapy. Exophthalmos / etiology. Optic Nerve Glioma / complications. Optic Nerve Neoplasms / complications. Outcome Assessment (Health Care) / methods
  • [MeSH-minor] Child, Preschool. Female. Humans. Infant. Magnetic Resonance Imaging / methods. Male. Retrospective Studies. Tomography Scanners, X-Ray Computed. Vision, Ocular / physiology

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  • (PMID = 18157537.001).
  • [ISSN] 0256-7040
  • [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
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27. Moharir M, London K, Howman-Giles R, North K: Utility of positron emission tomography for tumour surveillance in children with neurofibromatosis type 1. Eur J Nucl Med Mol Imaging; 2010 Jul;37(7):1309-17
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Utility of positron emission tomography for tumour surveillance in children with neurofibromatosis type 1.
  • PURPOSE: There is little consensus regarding optimal surveillance of optic pathway glioma (OPG) and plexiform neurofibroma (PNF) in childhood neurofibromatosis type 1 (NF1). (18)F-2-Fluoro-2-deoxy-D: -glucose (FDG) positron emission tomography and computed tomography (PET/CT) is employed in the surveillance of adult PNFs; but its utility has neither been specifically studied in children with PNFs nor in children with OPG.
  • FDG-avidity reduced from grade 3 to grade 1 in two symptomatic OPGs following chemotherapy and this was associated with clinical improvement.
  • As in adults, PET/CT is useful for the detection of malignant transformation in PNFs in children with NF1.
  • [MeSH-major] Neurofibromatosis 1 / diagnostic imaging. Positron-Emission Tomography
  • [MeSH-minor] Adult. Child. Child, Preschool. Female. Fluorodeoxyglucose F18. Humans. Male. Neurofibroma, Plexiform / diagnostic imaging. Optic Nerve Glioma / diagnostic imaging. Retrospective Studies. Tomography, X-Ray Computed

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  • (PMID = 20179923.001).
  • [ISSN] 1619-7089
  • [Journal-full-title] European journal of nuclear medicine and molecular imaging
  • [ISO-abbreviation] Eur. J. Nucl. Med. Mol. Imaging
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0Z5B2CJX4D / Fluorodeoxyglucose F18
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28. Allen JC: Initial management of children with hypothalamic and thalamic tumors and the modifying role of neurofibromatosis-1. Pediatr Neurosurg; 2000 Mar;32(3):154-62
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Optic pathway/hypothalamus gliomas (OPG) arise primarily from a slower-growing juvenile pilocytic astrocytoma, and thalamic gliomas arise primarily from a fibrillary astrocytoma which can become clinically and histologically more aggressive.
  • The major therapeutic challenge for these patients is to maximize their quality of life by preserving visual and endocrine function while minimizing treatment-related morbidity.
  • Treatment is often initiated at diagnosis in infants and toddlers who have a major visual impairment or the diencephalic syndrome.
  • The judicious application of chemotherapy may serve to forestall the need for radiotherapy or surgery.
  • However, over 90% of children with OPG without NF-1 will require some form of therapy.
  • Current multimodality therapy is relatively ineffective.
  • The bithalamic variant behaves similarly to a pontine glioma.
  • [MeSH-minor] Child. Child, Preschool. Humans. Hypothalamus / pathology. Infant. Magnetic Resonance Imaging. Neoadjuvant Therapy. Optic Nerve Glioma / diagnosis. Optic Nerve Glioma / surgery. Thalamus / pathology

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  • [Copyright] Copyright 2000 S. Karger AG, Basel
  • (PMID = 10867564.001).
  • [ISSN] 1016-2291
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Switzerland
  • [Number-of-references] 21
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29. Parsa CF, Hoyt CS, Lesser RL, Weinstein JM, Strother CM, Muci-Mendoza R, Ramella M, Manor RS, Fletcher WA, Repka MX, Garrity JA, Ebner RN, Monteiro ML, McFadzean RM, Rubtsova IV, Hoyt WF: Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging. Arch Ophthalmol; 2001 Apr;119(4):516-29
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging.
  • OBJECTIVE: To demonstrate spontaneous regression of large, clinically symptomatic optic pathway gliomas in patients with and without neurofibromatosis type 1 (NF-1).
  • Serial documentation of tumor signal and size, using magnetic resonance imaging in 11 patients and computed tomography in 2 patients, was used to evaluate clinically symptomatic optic pathway gliomas.
  • All tumors met radiologic criteria for the diagnosis of glioma and 4 patients had biopsy confirmation of their tumors.
  • In 3 patients, some attempt at therapy had been made many years before regression occurred.
  • In one of these, radiation treatment had been given 19 years before tumor regression, while in another, chemotherapy had been administered 5 years before signal changes in the tumor.
  • Tumor regression was associated with improvement in visual function in 10 of 13 patients, stability of function in 1, and deterioration in 2.
  • CONCLUSIONS: Large, clinically symptomatic optic gliomas may undergo spontaneous regression.
  • A variable degree of improvement in visual function may accompany regression.
  • The possibility of spontaneous regression of an optic glioma should be considered in the planning of treatment of patients with these tumors.
  • [MeSH-major] Brain Neoplasms / physiopathology. Neoplasm Regression, Spontaneous. Neurofibromatosis 1 / physiopathology. Optic Nerve Glioma / physiopathology
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Female. Humans. Infant. Magnetic Resonance Imaging. Male. Tomography, X-Ray Computed


30. Grabenbauer GG, Schuchardt U, Buchfelder M, Rödel CM, Gusek G, Marx M, Doerr HG, Fahlbusch R, Huk WJ, Wenzel D, Sauer R: Radiation therapy of optico-hypothalamic gliomas (OHG)--radiographic response, vision and late toxicity. Radiother Oncol; 2000 Mar;54(3):239-45

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Radiation therapy of optico-hypothalamic gliomas (OHG)--radiographic response, vision and late toxicity.
  • BACKGROUND: Management strategies for optic pathway gliomas include observation, surgery, irradiation, chemotherapy and a combination of these modalities.
  • It has been the policy of our University Hospital to consider radiation as the standard treatment for progressive optic pathway gliomas.
  • This report describes the clinical presentation, treatment patterns and outcome with special emphasis on the long term functional status of patients with optico-hypothalamic gliomas (OHG).
  • PATIENTS AND METHODS: Between 1975 and 1997, 25 patients with OHG were treated by radiation therapy (RT) following surgery or biopsy.
  • Age adjusted radiation doses ranged from 45 to 60 Gy with a single fraction size of 1.6-2 Gy.
  • Endpoints of the study were: radiographic response, survival, progression-free survival and time to endocrinologic toxicity as well as the visual function during follow-up.
  • The median follow-up time was 9 years (range, 1.5-23 years).
  • As for visual acuity, nine patients had an improvement, another 13 patients a stable situation and three patients a measurable deterioration.
  • Visual field deficits improved in three, remained unchanged in 16 patients and worsened in only one patient.
  • CONCLUSION: Postoperative RT with a total dose above 45 Gy should be considered as standard treatment in OHG with documented progression.
  • [MeSH-major] Glioma / radiotherapy. Hypothalamic Neoplasms / radiotherapy. Optic Nerve Glioma / radiotherapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Follow-Up Studies. Humans. Infant. Magnetic Resonance Imaging. Male. Prognosis. Radiation Injuries. Survival Rate. Tomography, X-Ray Computed. Visual Acuity / radiation effects

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  • (PMID = 10738082.001).
  • [ISSN] 0167-8140
  • [Journal-full-title] Radiotherapy and oncology : journal of the European Society for Therapeutic Radiology and Oncology
  • [ISO-abbreviation] Radiother Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] IRELAND
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31. Ogura T, Adachi J, Nishikawa R, Hirose T, Matsutani M: Synchronous optic and pineal pilocytic astrocytomas in a paediatric patient with neurofibromatosis type 1. Pediatr Neurosurg; 2004 Nov-Dec;40(6):301-5
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  • [Title] Synchronous optic and pineal pilocytic astrocytomas in a paediatric patient with neurofibromatosis type 1.
  • A 12-year-old girl with neurofibromatosis type 1 presented with headache, visual acuity and visual field disturbance.
  • Computed tomography and magnetic resonance imaging revealed an enhanced solid mass involving her right optic nerve and optic chiasm, and a cystic lesion in the pineal region that had resulted in obstructive hydrocephalus.
  • An open biopsy of the right optic nerve tumour was performed, and it was histologically identified as a pilocytic astrocytoma.
  • Local irradiation of 50 Gy to the optic pathway tumour was performed, and the tumour has remained stable for more than 29 months.
  • Chemotherapy with cisplatin and vincristine was performed after a second surgery, and the pineal tumour has not re-grown in 18 months.
  • To our knowledge, this is the first case report to describe synchronous optic and pineal pilocytic astrocytomas associated with neurofibromatosis type 1.
  • [MeSH-major] Astrocytoma / etiology. Brain Neoplasms / etiology. Neurofibromatosis 1 / complications. Optic Nerve Glioma / etiology. Pineal Gland


32. Moschovi M, Alexiou GA, Papakonstantopoulou I, Gavra M, Sfakianos G, Prodromou N: Efficacy of carboplatin plus vincristine in an optic pathway glioma. Klin Padiatr; 2010 Nov;222(6):395-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Efficacy of carboplatin plus vincristine in an optic pathway glioma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Optic Nerve Glioma / drug therapy
  • [MeSH-minor] Child, Preschool. Female. Follow-Up Studies. Humans. Hypothalamus / pathology. Magnetic Resonance Imaging. Optic Chiasm / pathology. Optic Nerve / pathology. Remission Induction. Treatment Outcome

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  • (PMID = 20806167.001).
  • [ISSN] 1439-3824
  • [Journal-full-title] Klinische Pädiatrie
  • [ISO-abbreviation] Klin Padiatr
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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