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1. 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
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  • [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.
  • There were three patients without histological diagnosis; one of them had NF1.
  • 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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2. Virdis R, Sigorini M, Laiolo A, Lorenzetti E, Street ME, Villani AR, Donadio A, Pisani F, Terzi C, Garavelli L: Neurofibromatosis type 1 and precocious puberty. J Pediatr Endocrinol Metab; 2000 Jul;13 Suppl 1:841-4
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  • [Title] Neurofibromatosis type 1 and precocious puberty.
  • Since neurofibromatosis type 1 (NF1) is a well known cause of precocious puberty (PP), we reviewed 412 NF1 pediatric patients to evaluate the prevalence of PP, the association with optic pathway tumors (OPT), and other clinical, auxological and hormonal data.
  • OPT in patients with PP involved the chiasm in four patients, and the optic nerves alone in three patients.
  • The age at the onset of puberty (or better at diagnosis) ranged from 5.2 to 7.5 yr in girls (n=6) and from 7.9 to 8.9 yr in boys (n=4).
  • LHRH agonist therapy was used in only three children because in the others the predicted height at diagnosis was good, treatment was refused or the patients were referred to us too late.
  • Treatment seems to be useful in the children with younger age at the onset of puberty or with a progressive decline in predicted final height.
  • [MeSH-minor] Adolescent. Adult. Body Height / drug effects. Brain Neoplasms / complications. Child. Child, Preschool. Female. Gonadotropin-Releasing Hormone / agonists. Humans. Infant. Infant, Newborn. Male. Optic Nerve Neoplasms / complications. Visual Pathways

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  • (PMID = 10969931.001).
  • [ISSN] 0334-018X
  • [Journal-full-title] Journal of pediatric endocrinology & metabolism : JPEM
  • [ISO-abbreviation] J. Pediatr. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] ENGLAND
  • [Chemical-registry-number] 33515-09-2 / Gonadotropin-Releasing Hormone
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3. 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
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  • [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.
  • Fourteen (78%) of 18 patients had a sustained reduction of tumor size between 6 months and 17 years.
  • Two patients died, none with neurofibromatosis-1, with a hypothalamic/chiasmatic tumor with suprasellar extension and accompanying electrolyte abnormalities.
  • 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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4. 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].
  • 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.
  • Gross total removal of the tumor provides 100% cure, but surgery can be proposed only for growing tumor with severe exophthalmos and no useful field of view or blindness.
  • 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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5. 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
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  • [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).
  • A significant influence on progression-free survival was noted for age at diagnosis (P=0.04) and total dose (P=0.05).
  • 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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6. 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
  • [MeSH-minor] Craniopharyngioma / complications. Craniopharyngioma / pathology. Craniopharyngioma / radiotherapy. Humans. Lymphoma / complications. Lymphoma / pathology. Lymphoma / radiotherapy. Meningioma / complications. Meningioma / pathology. Meningioma / radiotherapy. Pituitary Neoplasms / complications. Pituitary Neoplasms / pathology. Pituitary Neoplasms / radiotherapy. Pseudolymphoma / complications. Pseudolymphoma / pathology. Pseudolymphoma / radiotherapy. Radiation Dosage

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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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7. Shinoura N, Suzuki Y, Yamada R, Tabei Y, Saito K, Yagi K: Relationships between brain tumor and optic tract or calcarine fissure are involved in visual field deficits after surgery for brain tumor. Acta Neurochir (Wien); 2010 Apr;152(4):637-42
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Relationships between brain tumor and optic tract or calcarine fissure are involved in visual field deficits after surgery for brain tumor.
  • The goal of the present study was to analyze relationships between visual field deficits and the locations of brain tumors compared with optic tracts as visualized by tractography, and compared with the calcarine fissure.
  • METHODS: Subjects comprised 11 patients with brain tumor in the occipital lobe or atrium of the lateral ventricle who underwent surgery between October 2006 and February 2009.
  • Tumors were categorized as Type A, with almost all the optic tract in the occipital lobe or atrium of the lateral ventricle running close to and stretched by the brain tumor; and Type B, with the optic tract running at least partially distant to the brain tumor and remaining unstretched.
  • RESULTS: Those type A optic tracts that were laterally compressed by brain tumors (Cases 1-3) displayed hemianopsia after surgery.
  • When the brain tumor was located rostro-medial to the calcarine fissure and optic tracts were compressed caudally by the tumor, lower quadrant hemianopsia remained after surgery (Cases 4, 5).
  • In other cases, the visual field remained or improved to normal after surgery.
  • CONCLUSION: The relationship between optic tracts or the calcarine fissure, and brain tumors in the occipital lobe or atrium of the lateral ventricle is related to visual field deficits after surgery.
  • In particular, those Type A optic tracts that are compressed laterally show hemianopsia of the visual field after surgery.
  • [MeSH-major] Brain Neoplasms / surgery. Cerebral Ventricle Neoplasms / physiopathology. Cerebral Ventricle Neoplasms / surgery. Diffusion Tensor Imaging. Hemianopsia / physiopathology. Image Processing, Computer-Assisted. Lateral Ventricles / physiopathology. Lateral Ventricles / surgery. Nerve Compression Syndromes / physiopathology. Nerve Compression Syndromes / surgery. Occipital Lobe / physiopathology. Occipital Lobe / surgery. Optic Nerve / physiopathology. Optic Nerve / surgery. Postoperative Complications / physiopathology. Visual Cortex / physiopathology. Visual Cortex / surgery. Visual Fields / physiology

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  • (PMID = 20063172.001).
  • [ISSN] 0942-0940
  • [Journal-full-title] Acta neurochirurgica
  • [ISO-abbreviation] Acta Neurochir (Wien)
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Austria
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8. van Ginkel PR, Darjatmoko SR, Sareen D, Subramanian L, Bhattacharya S, Lindstrom MJ, Albert DM, Polans AS: Resveratrol inhibits uveal melanoma tumor growth via early mitochondrial dysfunction. Invest Ophthalmol Vis Sci; 2008 Apr;49(4):1299-306
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  • [Title] Resveratrol inhibits uveal melanoma tumor growth via early mitochondrial dysfunction.
  • PURPOSE: To test the efficacy of resveratrol, a nontoxic plant product, in the treatment of uveal melanoma.
  • METHODS: The effect of oral administration and peritumor injection of resveratrol was tested on tumor growth in two animal models of uveal melanoma.
  • RESULTS: Resveratrol treatment inhibited tumor growth in animal models of uveal melanoma.
  • Since oral administration resulted in relatively low bioavailability of resveratrol, the effect of increased local levels was tested by peritumor injection of the drug.
  • This method resulted in tumor cell death and tumor regression.
  • In vitro experiments with multiple uveal melanoma cell lines demonstrate that resveratrol causes a decrease in cell viability, resulting at least in part from an increase in apoptosis through a mitochondrial pathway.
  • An early event in drug action is the direct targeting of mitochondria by resveratrol, which leads to a decrease in mitochondrial membrane potential and the eventual activation of caspase-3.
  • CONCLUSION: These data suggest that resveratrol can inhibit tumor growth and can induce apoptosis via the intrinsic mitochondrial pathway and that by further increasing bioavailability of resveratrol the potency of the drug can be increased, leading to tumor regression.
  • The nontoxic nature of the drug at levels needed for therapy make resveratrol an attractive candidate for the treatment of uveal melanoma.

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  • (PMID = 18385041.001).
  • [ISSN] 0146-0404
  • [Journal-full-title] Investigative ophthalmology & visual science
  • [ISO-abbreviation] Invest. Ophthalmol. Vis. Sci.
  • [Language] ENG
  • [Grant] United States / NEI NIH HHS / EY / P30 EY016665; United States / NCI NIH HHS / CA / CA103653-07; United States / NCI NIH HHS / CA / R01 CA103653-07; United States / NCI NIH HHS / CA / R01CA103653; United States / NEI NIH HHS / EY / P30 EY016665-02; United States / NCI NIH HHS / CA / R01 CA103653
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Phytogenic; 0 / DIABLO protein, human; 0 / Intracellular Signaling Peptides and Proteins; 0 / Mitochondrial Proteins; 0 / Stilbenes; 9007-43-6 / Cytochromes c; EC 3.4.22.- / Caspase 3; EC 3.4.22.- / Caspase 9; Q369O8926L / resveratrol
  • [Other-IDs] NLM/ NIHMS53568; NLM/ PMC2465765
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9. All-Ericsson C, Girnita L, Müller-Brunotte A, Brodin B, Seregard S, Ostman A, Larsson O: c-Kit-dependent growth of uveal melanoma cells: a potential therapeutic target? Invest Ophthalmol Vis Sci; 2004 Jul;45(7):2075-82
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  • [Title] c-Kit-dependent growth of uveal melanoma cells: a potential therapeutic target?
  • Treatment of uveal melanoma cell lines with STI571, which blocks c-kit autophosphorylation, resulted in cell death.
  • CONCLUSIONS: The results confirm that c-kit is vastly expressed in uveal melanoma, suggest that the c-kit molecular pathway may be important in uveal melanoma growth, and point to its use as a target for therapy with STI571.
  • [MeSH-major] Melanoma / metabolism. Melanoma / pathology. Proto-Oncogene Proteins c-kit / metabolism. Uveal Neoplasms / metabolism. Uveal Neoplasms / pathology
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Benzamides. Blotting, Western. Cell Division. Female. Humans. Imatinib Mesylate. Immunoenzyme Techniques. Male. Middle Aged. Paraffin Embedding. Phosphorylation / drug effects. Piperazines. Polymerase Chain Reaction. Pyrimidines / pharmacology. RNA, Messenger / metabolism. Skin Neoplasms / metabolism. Skin Neoplasms / pathology. Tumor Cells, Cultured. Tyrosine / metabolism

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  • (PMID = 15223779.001).
  • [ISSN] 0146-0404
  • [Journal-full-title] Investigative ophthalmology & visual science
  • [ISO-abbreviation] Invest. Ophthalmol. Vis. Sci.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 0 / RNA, Messenger; 42HK56048U / Tyrosine; 8A1O1M485B / Imatinib Mesylate; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
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10. Maruyama K, Morita A, Shibahara J, Nakazato Y, Kirino T: Multifocal pilocytic astrocytomas with ependymal differentiation in the bilateral medial temporal lobes: case report. Neurol Med Chir (Tokyo); 2005 Aug;45(8):411-4
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  • A 7-year-old boy presented with headache, visual disturbance, and psychomotor seizures persisting for 7 months.
  • The histological diagnosis was pilocytic astrocytoma with ependymal differentiation and a MIB-1 staining index of up to 8.0%.
  • Adjuvant chemotherapy consisting of carboplatin and vincristine was given followed by radiotherapy.
  • Neuroimaging showed that the bilateral tumors had disappeared and showed no recurrence for 29 months after the diagnosis.
  • Pilocytic astrocytoma usually presents as a solitary mass in the cerebellum or optic pathway with low proliferative activity, but should be included in the differential diagnosis of multifocal tumors arising in the bilateral temporal lobes.
  • Intensive treatment is recommended for patients with such specific neuroimaging and histological features.
  • [MeSH-major] Astrocytoma / pathology. Brain Neoplasms / pathology. Temporal Lobe / pathology
  • [MeSH-minor] Antibodies, Antinuclear. Antibodies, Monoclonal. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cell Proliferation. Child. Diagnosis, Differential. Ependyma / pathology. Hemianopsia / etiology. Hemianopsia / pathology. Hemianopsia / physiopathology. Humans. Magnetic Resonance Imaging. Male. Neoplasm Invasiveness. Neurosurgical Procedures. Paresis / etiology. Paresis / pathology. Paresis / physiopathology. Radiotherapy. Seizures / etiology. Seizures / pathology. Seizures / physiopathology. Treatment Outcome

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  • (PMID = 16127260.001).
  • [ISSN] 0470-8105
  • [Journal-full-title] Neurologia medico-chirurgica
  • [ISO-abbreviation] Neurol. Med. Chir. (Tokyo)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antibodies, Antinuclear; 0 / Antibodies, Monoclonal; 0 / MIB-1 antibody
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11. Kamada K, Todo T, Morita A, Masutani Y, Aoki S, Ino K, Kawai K, Kirino T: Functional monitoring for visual pathway using real-time visual evoked potentials and optic-radiation tractography. Neurosurgery; 2005 Jul;57(1 Suppl):121-7; discussion 121-7
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  • [Title] Functional monitoring for visual pathway using real-time visual evoked potentials and optic-radiation tractography.
  • OBJECTIVE: It has been difficult to obtain anatomic and functional information about the visual pathway during neurosurgical operations.
  • The aim of this study was to combine the information of the visual evoked potentials (VEPs) and the anatomic navigation of the optic radiation by diffusion tensor imaging-based tractography for functional monitoring of the visual pathway.
  • METHODS: The subjects were two patients with brain lesions adjacent to the visual pathway.
  • Diffusion tensor imaging-based tractography of the optic radiation was performed by selecting appropriate regions of interest and by fractional anisotropy.
  • In Patient 2, the results of optic radiation tractography were imported to a neuronavigation system to better understand the spatial relationships between the lesions and the visual pathway (functional neuronavigation).
  • RESULTS: In Patient 1, the lesion did not seem to be attached to the optic radiation, and VEP profiles remained stable during resection.
  • In Patient 2, who had a lesion adjacent to the posterior horn of the lateral ventricle, VEPs suddenly diminished when resection reached the optic radiation as illustrated on the neuronavigation system.
  • As a result, complete left hemianopia developed after surgery in Patient 2.
  • The combination of continuous VEP and optic-radiation tractography is reliable to monitor the visual function and is helpful in performing neurosurgical planning near the visual pathway.
  • [MeSH-major] Brain Neoplasms / diagnosis. Brain Neoplasms / surgery. Diffusion Magnetic Resonance Imaging / methods. Evoked Potentials, Visual. Neuronavigation / methods. Visual Pathways / pathology. Visual Pathways / physiopathology
  • [MeSH-minor] Adult. Computer Systems. Female. Humans. Magnetic Resonance Imaging / methods. Male. Middle Aged. Treatment Outcome

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  • (PMID = 15987578.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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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.
  • Advances in magnetic resonance imaging are helping to delineate tumor extent.
  • 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. Messersmith W, Oppenheimer D, Peralba J, Sebastiani V, Amador M, Jimeno A, Embuscado E, Hidalgo M, Iacobuzio-Donahue C: Assessment of Epidermal Growth Factor Receptor (EGFR) signaling in paired colorectal cancer and normal colon tissue samples using computer-aided immunohistochemical analysis. Cancer Biol Ther; 2005 Dec;4(12):1381-6
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  • [Title] Assessment of Epidermal Growth Factor Receptor (EGFR) signaling in paired colorectal cancer and normal colon tissue samples using computer-aided immunohistochemical analysis.
  • The Epidermal Growth Factor Receptor (EGFR) plays a role in multiple tumor cell processes and is targeted by several anticancer therapies.
  • Although EGFR mutations may determine tumor susceptibility in a small proportion of patients, knowledge of the EGFR signaling pathway status in tumors may help guide further drug development and hypothesis-driven combination studies.
  • We aimed to validate and apply a novel computer-aided immunohistochemical (IHC) technique to characterize the status of EGFR signaling in matched colorectal tumor and normal colon tissue samples.
  • Tissue Microarrays (TMA)were made from both cancerous and normal colorectal tissue in 18 patients and stained with antibodies against EGFR, phospho-EGFR (pEGFR), Akt, pAkt, MAPK, and pMAPK.
  • ACIS was compared against cell line Western blotting, ELISA, and visual scoring (0-3+) by a pathologist.
  • We conclude that ACIS IHC of human tissue samples is quantitative, reproducible, and correlates with Western blots and ELISA in cell line pellets as well as pathologist's scores of human samples.
  • Colorectal tumors show higher staining of pEGFR and downstream effectors compared to matched normal colorectal tissues.
  • [MeSH-major] Colon / chemistry. Colorectal Neoplasms / chemistry. Image Processing, Computer-Assisted / methods. Receptor, Epidermal Growth Factor / analysis. Signal Transduction


14. Laithier V, Grill J, Le Deley MC, Ruchoux MM, Couanet D, Doz F, Pichon F, Rubie H, Frappaz D, Vannier JP, Babin-Boilletot A, Sariban E, Chastagner P, Zerah M, Raquin MA, Hartmann O, Kalifa C, French Society of Pediatric Oncology: Progression-free survival in children with optic pathway tumors: dependence on age and the quality of the response to chemotherapy--results of the first French prospective study for the French Society of Pediatric Oncology. J Clin Oncol; 2003 Dec 15;21(24):4572-8
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  • [Title] Progression-free survival in children with optic pathway tumors: dependence on age and the quality of the response to chemotherapy--results of the first French prospective study for the French Society of Pediatric Oncology.
  • PURPOSE: To evaluate a strategy aimed at avoiding radiotherapy during first-line treatment of children with progressive optic pathway tumors (OPT), by exclusively administering multiagent chemotherapy during 16 months.
  • Chemotherapy alternating procarbazine plus carboplatin, etoposide plus cisplatin, and vincristine plus cyclophosphamide was given every 3 weeks.
  • At the time of relapse or progression, second-line chemotherapy was authorized before recourse to radiotherapy.
  • RESULTS: Objective response rate (partial response [PR] + complete response [CR]) to chemotherapy was 42%.
  • In the multivariate analysis of the 85 patients that entered onto the study, factors associated with the risk of disease progression were age younger than 1 year at diagnosis (P =.047) and absence of neurofibromatosis type 1 (P =.035).
  • In the multivariate analysis of the 74 patients that remained on study after the first cycle of chemotherapy, factors associated with the risk of disease progression were age younger than 1 year at diagnosis (P =.0053) and no objective response to chemotherapy (P =.0029).
  • Three-year PFS was 53% in the absence of an objective response to chemotherapy versus 68% after a PR or CR.
  • CONCLUSION: A significant proportion of children with OPT can avoid radiotherapy after prolonged chemotherapy.
  • Deferring irradiation with chemotherapy protocols did not compromise overall survival of the entire population or visual function.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Optic Nerve Neoplasms / drug therapy
  • [MeSH-minor] Age Factors. Chi-Square Distribution. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Factor Analysis, Statistical. Female. France. Humans. Infant. Infant, Newborn. Male. Prospective Studies. Survival Analysis

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  • (PMID = 14673044.001).
  • [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] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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15. Scaioli V, Caraceni A, Martini C, Curzi S, Capri G, Luca G: Electrophysiological evaluation of visual pathways in paclitaxel-treated patients. J Neurooncol; 2006 Mar;77(1):79-87
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  • [Title] Electrophysiological evaluation of visual pathways in paclitaxel-treated patients.
  • As paclitaxel may induce positive spontaneous visual symptoms or persistent visual loss, we carried out this electrophysiological study in an attempt to clarify the underlying pathophysiological mechanisms of visual pathway involvement.
  • Pattern visual evoked potentials (VEPs), and transient, 30 Hz flicker (FLK) and oscillatory potential (OP) white flash electroretinograms (ERGs), were recorded before treatment, after the third and sixth therapeutic cycle, and at the end of the programmed regimen.
  • Electrophysiological changes during treatment revealed the involvement of both the retina and anterior optic pathway.
  • There was only a weak correlation between visual symptoms and electrophysiology.
  • We suggest that the most likely mechanism of visual symptoms and electrophysiological changes during paclitaxel administration is vascular dysregulation in the retina, or ischemic mechanisms when the optic nerve is involved.
  • [MeSH-major] Antineoplastic Agents, Phytogenic / adverse effects. Evoked Potentials, Visual / drug effects. Paclitaxel / adverse effects. Retina / drug effects. Scotoma / chemically induced. Visual Pathways / drug effects
  • [MeSH-minor] Adult. Analysis of Variance. Antibiotics, Antineoplastic / therapeutic use. Breast Neoplasms / complications. Breast Neoplasms / drug therapy. Doxorubicin / adverse effects. Doxorubicin / therapeutic use. Electrophysiology. Female. Flicker Fusion / drug effects. Humans. Middle Aged. Optic Nerve / drug effects. Optic Nerve / physiopathology

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  • (PMID = 16132528.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / Antineoplastic Agents, Phytogenic; 80168379AG / Doxorubicin; P88XT4IS4D / Paclitaxel
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16. Johnson KS, Levin F, Chu DS: Persistent corneal epithelial defect associated with erlotinib treatment. Cornea; 2009 Jul;28(6):706-7
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  • [Title] Persistent corneal epithelial defect associated with erlotinib treatment.
  • PURPOSE: To report a case of persistent corneal epithelial erosion in a patient undergoing treatment with erlotinib for her lung cancer.
  • METHODS: Report of a 79-year-old woman who presented with a persistent corneal epithelial defect associated with infectious keratitis that waxed and waned for 5 months despite treatment.
  • RESULTS: The keratitis required a biopsy to establish the diagnosis of Staphylococcus epidermidis keratitis.
  • She discontinued the erlotinib treatment.
  • It will become increasingly important for ophthalmologists to recognize and treat side effects of chemotherapies interfering with the epithelial growth factor pathway.
  • [MeSH-major] Corneal Diseases / chemically induced. Epithelium, Corneal / drug effects. Lung Neoplasms / drug therapy. Protein Kinase Inhibitors / adverse effects. Quinazolines / adverse effects

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  • (PMID = 19512896.001).
  • [ISSN] 1536-4798
  • [Journal-full-title] Cornea
  • [ISO-abbreviation] Cornea
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Protein Kinase Inhibitors; 0 / Quinazolines; DA87705X9K / Erlotinib Hydrochloride
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17. Pancucci G, Massimi L, Caldarelli M, D'Angelo L, Sturiale C, Tamburrini G, Tufo T, Di Rocco C: [Pediatric craniopharyngioma: long-term results in 61 cases]. Minerva Pediatr; 2007 Jun;59(3):219-31
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  • AIM: The aim of this study was to analyze the long-term results of the surgical management of craniopharyngioma in children by reviewing a series of patients consecutively treated in a single institution, and to assess the efficacy of intratumoral chemotherapy with interferon-alpha.
  • RESULTS: All the 55 surviving patients enjoy a normal social life, except for 3 of them (visual and/or neurological deficits); endocrine function, compromised in 3/5 of cases, is managed by chronic hormone replacement; neuropsychological assessment is satisfactory in almost all the cases.
  • CONCLUSION: The current experience confirms the still remarkable challenges in the treatment of craniopharyngioma in childhood.
  • Intracystic chemotherapy with interferon-alpha might represent an effective option to postpone the surgical operation until the maturation of the hypothalamic-hypophyseal pathway is completed.
  • [MeSH-major] Brain / pathology. Craniopharyngioma / therapy. Hypophysectomy. Pituitary Neoplasms / therapy
  • [MeSH-minor] Adolescent. Antineoplastic Agents / therapeutic use. Chemotherapy, Adjuvant. Child. Child, Preschool. Female. Follow-Up Studies. Humans. Infant. Injections, Intralesional. Interferon-alpha / therapeutic use. Magnetic Resonance Imaging. Male. Radiotherapy, Adjuvant. Recombinant Proteins. Retrospective Studies. Treatment Outcome

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  • (PMID = 17519867.001).
  • [ISSN] 0026-4946
  • [Journal-full-title] Minerva pediatrica
  • [ISO-abbreviation] Minerva Pediatr.
  • [Language] ita
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Interferon-alpha; 0 / Recombinant Proteins; 76543-88-9 / interferon alfa-2a
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18. 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.
  • The diagnosis is seldom made before craniotomy.
  • 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
  • [Number-of-references] 29
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19. Klisovic DD, Katz SE, Effron D, Klisovic MI, Wickham J, Parthun MR, Guimond M, Marcucci G: Depsipeptide (FR901228) inhibits proliferation and induces apoptosis in primary and metastatic human uveal melanoma cell lines. Invest Ophthalmol Vis Sci; 2003 Jun;44(6):2390-8
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  • PURPOSE: Uveal melanoma (UM) is the most common primary malignant ocular tumor in adults.
  • No effective chemotherapy regimens are available for either intraocular or metastatic uveal melanoma.
  • Real-time PCR was used to study changes in bcl-2/bax gene expression.
  • However, no changes in bcl-2/bax gene expression were detected by real-time PCR.
  • The apoptosis is probably mediated through the Fas/FasL signaling pathway, whereas bcl-2 appears not to be involved.
  • [MeSH-major] Antibiotics, Antineoplastic / pharmacology. Apoptosis / drug effects. Depsipeptides. Melanoma / pathology. Peptides, Cyclic / pharmacology. Uveal Neoplasms / pathology
  • [MeSH-minor] Antigens, CD95 / genetics. Antigens, CD95 / metabolism. Blotting, Western. Butyrates / pharmacology. Caspase 3. Caspases / metabolism. Cell Cycle Proteins / genetics. Cell Cycle Proteins / metabolism. Cell Division / drug effects. Cyclin-Dependent Kinase Inhibitor p21. Cyclin-Dependent Kinase Inhibitor p27. Cyclins / genetics. Cyclins / metabolism. Dose-Response Relationship, Drug. Enzyme Inhibitors / pharmacology. Fas Ligand Protein. Flow Cytometry. Histone Deacetylase Inhibitors. Humans. Hydroxamic Acids / pharmacology. Membrane Glycoproteins / genetics. Membrane Glycoproteins / metabolism. Proto-Oncogene Proteins / genetics. Proto-Oncogene Proteins / metabolism. Proto-Oncogene Proteins c-bcl-2 / genetics. Proto-Oncogene Proteins c-bcl-2 / metabolism. RNA, Messenger / metabolism. Reverse Transcriptase Polymerase Chain Reaction. Tumor Cells, Cultured. Tumor Suppressor Proteins / genetics. Tumor Suppressor Proteins / metabolism. bcl-2-Associated X Protein

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  • (PMID = 12766035.001).
  • [ISSN] 0146-0404
  • [Journal-full-title] Investigative ophthalmology & visual science
  • [ISO-abbreviation] Invest. Ophthalmol. Vis. Sci.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / Antigens, CD95; 0 / BAX protein, human; 0 / Butyrates; 0 / CDKN1A protein, human; 0 / Cell Cycle Proteins; 0 / Cyclin-Dependent Kinase Inhibitor p21; 0 / Cyclins; 0 / Depsipeptides; 0 / Enzyme Inhibitors; 0 / FASLG protein, human; 0 / Fas Ligand Protein; 0 / Histone Deacetylase Inhibitors; 0 / Hydroxamic Acids; 0 / Membrane Glycoproteins; 0 / Peptides, Cyclic; 0 / Proto-Oncogene Proteins; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / RNA, Messenger; 0 / Tumor Suppressor Proteins; 0 / bcl-2-Associated X Protein; 147604-94-2 / Cyclin-Dependent Kinase Inhibitor p27; 3X2S926L3Z / trichostatin A; CX3T89XQBK / romidepsin; EC 3.4.22.- / CASP3 protein, human; EC 3.4.22.- / Caspase 3; EC 3.4.22.- / Caspases
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20. 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.
  • Four of the children had histologically verified pilocytic astrocytomas, and in two cases, diagnosis was made on the basis of clinical findings.
  • 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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21. 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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  • [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.
  • In the third patient, minimal surgical debulking was performed 1 year before the tumor began to shrink.
  • RESULTS: Spontaneous tumor shrinkage was noted in 12 patients.
  • In an additional patient without NF-1, a signal change within the tumor without associated shrinkage was detected.
  • 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.
  • Regression may manifest either as an overall shrinkage in tumor size, or as a signal change on magnetic resonance imaging.
  • 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


22. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • 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.
  • Objective tumor response and toxicity were evaluated by magnetic resonance imaging and neurologic and functional tests at 3-month intervals.
  • 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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23. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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24. 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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25. 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


26. Shambhu S, Vose M: High grade lymphoma in the nasopharynx presented as sudden onset of bilateral blindness. BMC Ophthalmol; 2004;4:2
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  • After treatment the patient made a complete visual recovery, with no evidence of visual sequelae and no clear reasons for this complete recovery.
  • CONCLUSION: CT and MR imaging did not demonstrate any lesions invading any part of the visual pathway or even indeed the occipital cortex.
  • [MeSH-major] Blindness / diagnosis. Lymphoma, Non-Hodgkin / pathology. Nasopharyngeal Neoplasms / pathology. Paranasal Sinus Neoplasms / pathology
  • [MeSH-minor] Amoxicillin-Potassium Clavulanate Combination / therapeutic use. Dexamethasone / therapeutic use. Drug Therapy, Combination. Female. Humans. Magnetic Resonance Imaging. Middle Aged. Neoplasm Invasiveness. Tomography, X-Ray Computed

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  • (PMID = 15102322.001).
  • [ISSN] 1471-2415
  • [Journal-full-title] BMC ophthalmology
  • [ISO-abbreviation] BMC Ophthalmol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 74469-00-4 / Amoxicillin-Potassium Clavulanate Combination; 7S5I7G3JQL / Dexamethasone
  • [Other-IDs] NLM/ PMC387828
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27. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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.
  • 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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28. 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.
  • Surgery was performed with partial removal of the intrachiasmatic part of the intrinsic tumor.
  • The histological diagnosis was fibrillary astrocytoma.
  • 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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29. 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.
  • 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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30. 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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31. 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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32. Sawamura Y, Kamoshima Y, Kato T, Tajima T, Tsubaki J: Chemotherapy with cisplatin and vincristine for optic pathway/hypothalamic astrocytoma in young children. Jpn J Clin Oncol; 2009 May;39(5):277-83
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Chemotherapy with cisplatin and vincristine for optic pathway/hypothalamic astrocytoma in young children.
  • OBJECTIVE: Optic pathway/hypothalamic astrocytomas (OPHA) in young children often show accelerated growth and require rather intensive induction chemotherapy.
  • All of them presented with progressive disease, and the tumor size was larger than 34 mm.
  • Eleven patients had visual disturbance, six had diencephalic syndrome and four had hydrocephalus.
  • All children tolerated the chemotherapy well under careful audiological monitoring.
  • CONCLUSION: Although the present series was small, this chemotherapy is a useful regimen for induction therapy in children with an aggressive deep-seated pilocytic astrocytoma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Astrocytoma / drug therapy. Brain Neoplasms / drug therapy. Hypothalamic Neoplasms / drug therapy. Visual Pathways
  • [MeSH-minor] Antineoplastic Agents / administration & dosage. Antineoplastic Agents, Phytogenic / administration & dosage. Child. Child, Preschool. Cisplatin / administration & dosage. Drug Administration Schedule. Humans. Infant. Male. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 19224939.001).
  • [ISSN] 1465-3621
  • [Journal-full-title] Japanese journal of clinical oncology
  • [ISO-abbreviation] Jpn. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Antineoplastic Agents, Phytogenic; 5J49Q6B70F / Vincristine; Q20Q21Q62J / Cisplatin
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33. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • 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.
  • Ten patients are alive and well from 2 to >14 years post-diagnosis.
  • 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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34. 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.
  • Of the 17 children in whom the tumor became significantly smaller, VA improved in 6, was stable in 3, and worse in 8.
  • 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


35. 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.
  • Surgical intervention is often required to relieve intracranial pressure and establish the histologic identity of the tumor.
  • Current multimodality therapy is relatively ineffective.
  • [MeSH-major] Astrocytoma / surgery. Brain Neoplasms / surgery. Hypothalamic Neoplasms / surgery. Neurofibromatosis 1 / surgery. Thalamic Diseases / surgery
  • [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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