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1. 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.
  • The radiological assessment of chemotherapeutic response in these patients can be complicated by irregular tumor shape and lack of relation between tumor volume and cosmetic effect.
  • METHOD: We propose that proptosis measurements and derivation of a proptosis index can be a useful adjunct to the measurement of tumor volume in the radiological assessment of chemotherapeutic response.
  • 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.
  • CONCLUSIONS: We have found that a proptosis index <1 correlates with a chemotherapeutic maintained response and an index >1 correlates with progressive disease.
  • [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
  • [Publication-country] Germany
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2. Steinbok P: Optic pathway tumors in children. J Chin Med Assoc; 2003 Jan;66(1):4-12
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  • [Title] Optic pathway tumors in children.
  • Tumors of the optic pathways are sub-divided in this review into those that arise in one or both optic nerves anterior to the chiasm (optic nerve tumors); those that arise within the chiasm and do not extend significantly into the hypothalamus (optic chiasmatic tumors) and the large exophytic tumors that involve both the optic chiasm and the hypothalamus to a lesser or greater degree (optic chiasmatic/hypothalamic tumors).
  • The management of optic chiasmatic gliomas is controversial, partly related to failure to separate out chiasmatic tumors from the chiasmatic/hypothalamic tumors.
  • The optic nerve tumors are reviewed briefly, since they rarely extend intracranially.
  • Chiasmatic tumors tend to be associated with NF1 and to behave almost like hamartomas.
  • On the other hand, chiasmatic/hypothalamic tumors grow like typical neoplasms.
  • The tumors are almost uniformly low grade astrocytomas, but growth rates may berapid, especially in infants.
  • Modern management has trended away from radical surgical resection, which has significant morbidity, to chemotherapy as the first line of treatment.
  • In this review, the clinical presentation and management of different types of optic pathway tumors are discussed.
  • [MeSH-major] Hypothalamic Neoplasms / therapy. Optic Chiasm. Optic Nerve Neoplasms / therapy
  • [MeSH-minor] Child. Glioma / diagnosis. Glioma / therapy. Humans

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  • (PMID = 12728968.001).
  • [ISSN] 1726-4901
  • [Journal-full-title] Journal of the Chinese Medical Association : JCMA
  • [ISO-abbreviation] J Chin Med Assoc
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] China (Republic : 1949- )
  • [Number-of-references] 34
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3. Lindegaard J, Heegaard S, Toft PB, Nysom K, Prause JU: Malignant transformation of a medulloepithelioma of the optic nerve. Orbit; 2010 Jun;29(3):161-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant transformation of a medulloepithelioma of the optic nerve.
  • OBJECTIVE: To describe a case of malignant transformation of a medulloepithelioma of the optic nerve.
  • A benign non-teratoid medulloepithelioma of the optic nerve was surgically removed.
  • The boy was further treated with chemotherapy and radiotherapy and is still alive 36 months after primary surgery.
  • RESULTS: Medulloepithelioma of the optic nerve is extremely rare.
  • This is the first case described of a benign medulloepithelioma of the optic nerve, later transforming into a malignant tumour.
  • One third of patients with medulloepithelioma of the optic nerve have died from direct intracranial spread or metastasis to the central nervous system (CNS).
  • CONCLUSIONS: Medulloepithelioma of the optic nerve should be considered malignant despite morphology with tendency to spread to the CNS.
  • Furthermore; it should be considered as a differential diagnosis when observing a tumour of the optic nerve in a child.
  • [MeSH-major] Cell Transformation, Neoplastic / pathology. Neoplasm Recurrence, Local / pathology. Neuroectodermal Tumors, Primitive / pathology. Optic Nerve Neoplasms / pathology
  • [MeSH-minor] Biopsy, Needle. Chemotherapy, Adjuvant. Child, Preschool. Exophthalmos / diagnosis. Exophthalmos / etiology. Eye Enucleation / methods. Follow-Up Studies. Humans. Immunohistochemistry. Magnetic Resonance Imaging / methods. Male. Orbit Evisceration / methods. Radiotherapy, Adjuvant. Risk Assessment. Stem Cell Transplantation. Treatment Outcome

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  • (PMID = 20497085.001).
  • [ISSN] 1744-5108
  • [Journal-full-title] Orbit (Amsterdam, Netherlands)
  • [ISO-abbreviation] Orbit
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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6. Filho JP, Correa ZM, Odashiro AN, Coutinho AB, Martins MC, Erwenne CM, Burnier MN Jr: Histopathological features and P-glycoprotein expression in retinoblastoma. Invest Ophthalmol Vis Sci; 2005 Oct;46(10):3478-83
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  • PURPOSE: To investigate the expression of P-glycoprotein (P-gp) in retinoblastoma specimens enucleated as a primary treatment or after conservative treatment and to correlate this expression with histopathological tumor features.
  • METHODS: Retrospective analysis was performed on retinoblastoma specimens obtained consecutively between 1993 and 2003 by enucleation either as primary treatment (group I) or after the failure of conservative treatment (group II).
  • Group I tumor differentiation was classified according to the percentage of Flexner-Wintersteiner rosettes.
  • Group II tumors, categorized as viable-appearing, regressed with a well-differentiated component (WDC), and regressed.
  • Other features, such as choroidal and optic nerve invasion, were evaluated.
  • Variables were statistically analyzed by chi(2) and Student's t-tests.
  • RESULTS: Histopathological assessment of group I revealed 65% moderately differentiated tumors, 30% well differentiated, and 5% poorly differentiated.
  • Fifteen percent had optic nerve tumor invasion only, 20% choroidal invasion only, and 55% both choroidal and optic nerve invasion.
  • Group II had 62.5% well-differentiated, regressed tumors; 25% had regressed tumors replaced by glial scarring; and 12.5% had tumors containing viable, poorly differentiated cells.
  • Approximately 18% had choroidal tumor invasion only, 6.3% optic nerve tumor invasion only, and 6.3% simultaneous optic nerve and choroidal invasion.
  • P-gp expression was observed in 60% of group I and 66.6% of group II.
  • All P-gp-positive cases in group II had a high expression.
  • P-gp was also expressed by 81.2% of well-differentiated tumors.
  • CONCLUSIONS: P-gp was expressed more frequently by well-differentiated retinoblastomas, especially those treated by chemotherapy before enucleation.
  • This finding could be related to treatment failure.
  • [MeSH-major] P-Glycoprotein / metabolism. Retinal Neoplasms / metabolism. Retinal Neoplasms / pathology. Retinoblastoma / metabolism. Retinoblastoma / pathology
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Cell Differentiation. Child, Preschool. Eye Enucleation. Female. Humans. Immunoenzyme Techniques. Infant. Male. Neoplasm Invasiveness. Treatment Failure

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  • (PMID = 16186322.001).
  • [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 / Antineoplastic Agents; 0 / P-Glycoprotein
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