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1. Aguilar Moliner I, Costa Orvay JA, Juma K, Costa Clara JM, Cruz Martínez O, Pou Fernández J: [Optic pathway astrocytoma: an unusual cause of failure to thrive in infants]. An Pediatr (Barc); 2007 Jun;66(6):622-4
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  • [Title] [Optic pathway astrocytoma: an unusual cause of failure to thrive in infants].
  • The main objective in these patients is the early detection of an organic cause, if present.
  • We report a case of low-grade astrocytoma of the optic pathway in a 2-month-old child whose main symptoms at diagnosis were failure to thrive and anorexia.
  • Unfortunately, despite therapeutic efforts, the tumor showed local and metastatic progression refractory to chemotherapy.
  • [MeSH-major] Brain Neoplasms / diagnosis. Failure to Thrive / etiology. Optic Nerve Glioma / diagnosis

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  • (PMID = 17583627.001).
  • [ISSN] 1695-4033
  • [Journal-full-title] Anales de pediatría (Barcelona, Spain : 2003)
  • [ISO-abbreviation] An Pediatr (Barc)
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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2. 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 histological diagnosis was fibrillary astrocytoma.
  • Adjuvant treatment included one course of fractionated radiation therapy and six courses of chemotherapy.
  • 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.
  • [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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3. Bommakanti K, Panigrahi M, Yarlagadda R, Sundaram C, Uppin MS, Purohit AK: Optic chiasmatic-hypothalamic gliomas: is tissue diagnosis essential? Neurol India; 2010 Nov-Dec;58(6):833-40

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Optic chiasmatic-hypothalamic gliomas: is tissue diagnosis essential?
  • BACKGROUND: Optic chiasmatic-hypothalamic gliomas are sellar-suprasellar lesions with variable radiological features.
  • The advocated treatment is mainly primary radiotherapy without a histological diagnosis.
  • However, in developing countries, like India infective granulomas (tuberculomas) in the suprasellar region radiologically can mimic optic chiasmatic-hypothalamic gliomas.
  • PATIENTS AND METHODS: The magnetic resonance imaging (MRI) characteristics of 24 patients with either histologically proven optic chiasmatic "pilocytic astrocytoma" or radiologically suspected optic chiasmatic-hypothalamic gliomas were analyzed.
  • RESULTS: The three radiological groups were: Group-1 solid tumors with or without microcysts in 9 patients (histology: 8 pilocystic astrocytomas and 1 tuberculoma); Group-2 mixed tumors with solid and cystic components in 9 patients (histology: 7 pilocytic astrocytomas and 2 craniopharyngiomas); Group-3 ring enhancing lesions in 6 patients (all the 6 patients initially received antituberculous treatment, in 3 patients the lesion resolved and in the remaining 3 patients the lesion was subjected to biopsy as it did not resolve, the biopsy was suggestive of pilocytic astrocytoma).
  • Thus, MRI was shown to have a sensitivity of 83.33% and a specificity of 50% for diagnosing optic chiasmatic-hypothalamic gliomas.
  • CONCLUSIONS: Various lesions like craniopharyngiomas, tuberculomas can mimic optic chiasmatic-hypothalamic gliomas radiologically, and it is not possible to diagnose them with certainty on the basis of radiological findings alone.
  • Biopsy and tissue diagnosis should always be sought before instituting radiotherapy or chemotherapy for optic chiasmatic-hypothalamic gliomas.
  • [MeSH-major] Glioma / diagnosis. Hypothalamic Neoplasms / diagnosis. Optic Chiasm / pathology. Optic Nerve Neoplasms / diagnosis

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  • [CommentIn] Neurol India. 2011 Jan-Feb;59(1):144 [21339694.001]
  • (PMID = 21150045.001).
  • [ISSN] 0028-3886
  • [Journal-full-title] Neurology India
  • [ISO-abbreviation] Neurol India
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] India
  • [Chemical-registry-number] 0 / Contrast Media
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4. Koeller KK, Rushing EJ: From the archives of the AFIP: pilocytic astrocytoma: radiologic-pathologic correlation. Radiographics; 2004 Nov-Dec;24(6):1693-708
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  • [Title] From the archives of the AFIP: pilocytic astrocytoma: radiologic-pathologic correlation.
  • Pilocytic astrocytoma is the most common pediatric central nervous system glial neoplasm and the most common pediatric cerebellar tumor.
  • The cerebellum, optic nerve and chiasm, and hypothalamic region are the most common locations, but the tumor can also be found in the cerebral hemisphere, ventricles, and spinal cord.
  • Surgical resection is the treatment of choice for all tumors, except for those involving the optic pathway and hypothalamic region, which may be treated with radiation therapy and chemotherapy.
  • Accurate interpretation of imaging studies plays an essential role in directing treatment of these tumors, particularly when they arise in the optic pathway of patients with neurofibromatosis type 1.
  • [MeSH-major] Astrocytoma / pathology. Astrocytoma / radiography. Tomography, X-Ray Computed

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  • (PMID = 15537977.001).
  • [ISSN] 1527-1323
  • [Journal-full-title] Radiographics : a review publication of the Radiological Society of North America, Inc
  • [ISO-abbreviation] Radiographics
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 98
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5. 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.
  • 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

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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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6. Chikai K, Ohnishi A, Kato T, Ikeda J, Sawamura Y, Iwasaki Y, Itoh T, Sawa H, Nagashima K: Clinico-pathological features of pilomyxoid astrocytoma of the optic pathway. Acta Neuropathol; 2004 Aug;108(2):109-14

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinico-pathological features of pilomyxoid astrocytoma of the optic pathway.
  • Five cases of pilomyxoid astrocytoma (PmA) characterized by a monophasic pattern with a myxoid background were selected for a clinicopathological study from 23 cases previously diagnosed as pilocytic astrocytoma (PA).
  • All PmA patients were either infants or young children (mean age 2.1 years), and all tumors were located in the optic chiasm/hypothalamus region.
  • All cases received chemotherapy, which reduced tumor size, and the location of the tumor became confined to the optic chiasm.
  • In two cases, tumor recurrence occurred 3 and 7 years after chemotherapy.
  • Hence, we conclude that PmA might be an infantile form of PA and speculate that a subset of PmA in the optic pathway/hypothalamus originates from the optic chiasm, possibly derived from radial glia existing in the embryonic optic chiasm.
  • [MeSH-major] Astrocytoma / pathology. Hypothalamic Neoplasms / pathology. Optic Chiasm / pathology. Optic Nerve Neoplasms / pathology
  • [MeSH-minor] Child. Child, Preschool. Drug Therapy. Female. Humans. Infant. Magnetic Resonance Imaging / methods. Male. Staining and Labeling. Time Factors

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  • (PMID = 15168135.001).
  • [ISSN] 0001-6322
  • [Journal-full-title] Acta neuropathologica
  • [ISO-abbreviation] Acta Neuropathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
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7. Sawamura Y, Kamada K, Kamoshima Y, Yamaguchi S, Tajima T, Tsubaki J, Fujimaki T: Role of surgery for optic pathway/hypothalamic astrocytomas in children. Neuro Oncol; 2008 Oct;10(5):725-33

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Role of surgery for optic pathway/hypothalamic astrocytomas in children.
  • Optic pathway/hypothalamic pilocytic astrocytomas in children are usually treated with chemotherapy following a surgical biopsy.
  • In a series of 25 patients without neurofibromatosis type 1, the median age at initial treatment was 3.1 years (range, 0-15 years).
  • Twenty-three patients received chemotherapy.
  • Aims of surgery at the initiation of treatment were biopsy in 12 cases (1 stereotactic and 11 craniotomies) and debulking in 7 cases.
  • The 11 open biopsies revealed pilocytic astrocytoma; however, noticeable complications occurred in five children after the biopsies.
  • Review of preoperative MRIs showed that all had typical findings indicating pilocytic astrocytoma.
  • The open biopsy offered no noteworthy benefit for the patients despite surgical risk and delay of chemotherapy.
  • The extent of the seven resection surgeries was 70% or less removal, and postoperative adjuvant therapy was needed for six of the seven patients.
  • The remaining six children who did not undergo surgery obtained remission with chemotherapy alone.
  • In conclusion, considering the risk of open surgery and the effectiveness of chemotherapy, the role of surgical intervention is restricted to bulk-reduction surgery only when it is inevitable, especially at relapse after chemotherapy.
  • [MeSH-major] Astrocytoma / surgery. Hypothalamic Neoplasms / surgery. Optic Nerve Neoplasms / surgery
  • [MeSH-minor] Adolescent. Antineoplastic Agents / administration & dosage. Child. Child, Preschool. Combined Modality Therapy. Humans. Infant. Infant, Newborn. Magnetic Resonance Imaging. Neoplasm Recurrence, Local / surgery. Retrospective Studies

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  • (PMID = 18612049.001).
  • [ISSN] 1522-8517
  • [Journal-full-title] Neuro-oncology
  • [ISO-abbreviation] Neuro-oncology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Other-IDs] NLM/ PMC2666249
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8. 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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  • [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.
  • Two patients showed pilocytic astrocytoma in the histological study.
  • 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.
  • Despite the apparent tumoral agressivity of the magnetic resonance and magnetic resonance spectroscopy images, histological findings corresponded to benign pilocytic astrocytoma.
  • [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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9. Hsu TR, Wong TT, Chang FC, Ho DM, Tang RB, Thien PF, Chang KP: Responsiveness of progressive optic pathway tumors to cisplatin-based chemotherapy in children. Childs Nerv Syst; 2008 Dec;24(12):1457-61
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Responsiveness of progressive optic pathway tumors to cisplatin-based chemotherapy in children.
  • BACKGROUND: Though the pathology of optic pathway tumor is mostly pilocytic astrocytoma, the benign tumor behaves like malignant tumor because total resection is not feasible.
  • Adjuvant chemotherapy might be a reasonable strategy for management of these low grade tumors which keep growing.
  • We evaluate the responsiveness of optic pathway tumor to cisplatin-based chemotherapy.
  • METHODS: Patients with unresectable and progressive optic pathway tumors received conventional chemotherapy including cisplatin, etoposide, and vinblastine were enrolled in this study from 1992 to 2007.
  • Brain MRI was performed every 3 months to evaluate the objective response to chemotherapy.
  • The pathology showed pilocytic astrocytomas in 11 patients, astrocytoma in one patient, and anaplastic astrocytomas in two patients.
  • The toxicity of the cisplatin-based chemotherapy showed mild bone marrow suppression in 13 patients (81.3%), infection in nine patients (56.3%), gastrointestinal discomfort in seven patients (43.8%), renal insufficiency in two patient (12.5%), cerebral salt wasting syndrome with hyponatremia in one patient (6.25%) and high pitch hearing loss in two patients (12.5%).
  • CONCLUSION: Cisplatin-based chemotherapy is an effective regimen for control of progressive optic pathway tumors.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Astrocytoma / drug therapy. Optic Nerve Neoplasms / drug therapy
  • [MeSH-minor] Bone Marrow Diseases / chemically induced. Child. Child, Preschool. Cisplatin / administration & dosage. Cisplatin / adverse effects. Disease-Free Survival. Drug Administration Schedule. Etoposide / administration & dosage. Etoposide / adverse effects. Female. Gastrointestinal Diseases / chemically induced. Humans. Infection / chemically induced. Magnetic Resonance Imaging. Male. Treatment Outcome. Vinblastine / administration & dosage. Vinblastine / adverse effects

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  • (PMID = 18769928.001).
  • [ISSN] 1433-0350
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 5V9KLZ54CY / Vinblastine; 6PLQ3CP4P3 / Etoposide; Q20Q21Q62J / Cisplatin
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10. Kageji T, Nagahiro S, Horiguchi H, Watanabe T, Suzuya H, Okamoto Y, Kuroda Y: Successful high-dose chemotherapy for widespread neuroaxis dissemination of an optico-hypothalamic juvenile pilocytic astrocytoma in an infant: a case report. J Neurooncol; 2003 May;62(3):281-7
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  • [Title] Successful high-dose chemotherapy for widespread neuroaxis dissemination of an optico-hypothalamic juvenile pilocytic astrocytoma in an infant: a case report.
  • We report a 13-month-old boy with diencephalic syndrome (DS) due to an optico-hypothalamic juvenile pilocytic astrocytoma (JPA).
  • He received 6 courses of combined conventional-dose chemotherapy consisting of carboplatin (CBDCA), etoposide (VP-16), and cyclophosphamide (CPA) followed by high-dose chemotherapy with CBDCA, CPA, and ranimustine (MCNU) and peripheral blood stem cell transplantation (PBSCT).
  • This treatment produced tumor regression in both intracranial and spinal lesions and remarkable improvement of DS.
  • The rare combination of DS and symptomatic neuroaxis dissemination of JPA at diagnosis suggests that the behavior of some of these tumors is more aggressive and resistant to conventional-dose chemotherapy than is that of JPA without DS manifestation and dissemination.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Astrocytoma / drug therapy. Hypothalamic Neoplasms / drug therapy. Optic Nerve Neoplasms / drug therapy
  • [MeSH-minor] Carboplatin / administration & dosage. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Etoposide / administration & dosage. Humans. Infant. Magnetic Resonance Imaging. Male. Neoplasm Invasiveness. Nitrosourea Compounds / administration & dosage. Peripheral Blood Stem Cell Transplantation. Syndrome

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  • (PMID = 12777080.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Nitrosourea Compounds; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; BG3F62OND5 / Carboplatin; RYH2T97J77 / ranimustine
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11. Silva MM, Goldman S, Keating G, Marymont MA, Kalapurakal J, Tomita T: Optic pathway hypothalamic gliomas in children under three years of age: the role of chemotherapy. Pediatr Neurosurg; 2000 Sep;33(3):151-8
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  • [Title] Optic pathway hypothalamic gliomas in children under three years of age: the role of chemotherapy.
  • OBJECTIVES: Optic pathway/hypothalamic gliomas (OPHGs) tend to occur in young children.
  • Treatment options consist of surgical resection, radiation therapy (RT) and chemotherapy.
  • Due to complications induced by surgery and RT, chemotherapy has gained significant recognition for the treatment of OPHG in young children.
  • We analyzed 14 patients who were treated with chemotherapy with or without surgery.
  • Five patients had partial tumor resection and 4 had endoscopic biopsy at the time of ventriculoperitoneal shunt placement.
  • Pathological examination revealed low-grade astrocytoma in 5 and juvenile pilocytic astrocytoma in 4.
  • All patients received chemotherapy: carboplatin in 8, a combination of carboplatin and vincristine in 4 and a combination of other agents in 2.
  • RESULTS: Eight (57%) of 14 patients had a sustained reduction of tumor during the follow-up time between 15 months and 8 years.
  • Diencephalic syndrome responded to chemotherapy alone in 4 of 6 patients.
  • However, 5 others had progressive disease; 3 during the treatment and 2 following the treatment (9 months and 2 years, respectively).
  • All these 5 patients had a partial tumor resection prior to chemotherapy.
  • CONCLUSION: A majority of OPHGs responds to chemotherapy.
  • Due to slow progression of these tumors and adverse effects of other therapeutic modalities, we recommend chemotherapy as a primary treatment for OPHGs.
  • Our present data indicates that partial surgical resection does not enhance chemotherapy effectiveness for OPHGs in infants or children younger than 3 years.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Hypothalamic Neoplasms / diagnosis. Hypothalamic Neoplasms / drug therapy. Optic Nerve Glioma / diagnosis. Optic Nerve Glioma / drug therapy
  • [MeSH-minor] Astrocytoma / drug therapy. Child, Preschool. Disease-Free Survival. Female. Humans. Infant. Magnetic Resonance Imaging. Male. Retrospective Studies. Survival Analysis. Treatment Outcome

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  • [Copyright] Copyright 2000 S. Karger AG, Basel
  • (PMID = 11096362.001).
  • [ISSN] 1016-2291
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] Switzerland
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12. Colosimo C, Cerase A, Maira G: Regression after biopsy of a pilocytic opticochiasmatic astrocytoma in a young adult without neurofibromatosis. Neuroradiology; 2000 May;42(5):352-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Regression after biopsy of a pilocytic opticochiasmatic astrocytoma in a young adult without neurofibromatosis.
  • Serial MRI over 60 months demonstrated regression after biopsy of a pilocytic opticochiasmatic astrocytoma in a 20-year-old woman with no signs of neurofibromatosis, together with improvement in vision.
  • The patient did not receive radio- or chemotherapy.
  • Close MRI follow-up of optic gliomas is recommended.
  • Aggressive treatment should be limited to cases with clear clinical and radiological progression.
  • [MeSH-major] Astrocytoma / pathology. Optic Chiasm / pathology. Optic Nerve Neoplasms / pathology

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  • (PMID = 10872155.001).
  • [ISSN] 0028-3940
  • [Journal-full-title] Neuroradiology
  • [ISO-abbreviation] Neuroradiology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] GERMANY
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13. Lertchavanakul A, Baimai C, Siwanuwatn R, Nuchprayoon I, Phudhichareonrat S: Optic nerve glioma in infancy: a case report of the youngest patient in Thailand. J Med Assoc Thai; 2001 Jun;84 Suppl 1:S137-41
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Optic nerve glioma in infancy: a case report of the youngest patient in Thailand.
  • She was diagnosed as optic nerve and chiasm glioma.
  • The subtotal removal of the tumor was performed and followed by chemotherapy, with a satisfactory result.
  • To our knowledge, this reported case is the youngest patient with optic nerve and chiasm astrocytoma in Thailand.
  • [MeSH-major] Glioma / diagnosis. Optic Nerve Neoplasms / diagnosis
  • [MeSH-minor] Antineoplastic Agents / administration & dosage. Biopsy, Needle. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Infant. Neurosurgical Procedures / methods. Thailand. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 11529326.001).
  • [ISSN] 0125-2208
  • [Journal-full-title] Journal of the Medical Association of Thailand = Chotmaihet thangphaet
  • [ISO-abbreviation] J Med Assoc Thai
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Thailand
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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14. Sparagana SP, Wilkes DC, Thompson CE, Bowers DC: Optic nerve tumor in tuberous sclerosis complex is not responsive to sirolimus. Pediatr Neurol; 2010 Jun;42(6):443-6
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  • [Title] Optic nerve tumor in tuberous sclerosis complex is not responsive to sirolimus.
  • A 12-year-old girl with clinically established tuberous sclerosis complex, and without signs of neurofibromatosis type 1, developed a right retro-ocular optic nerve tumor.
  • After rapid growth for 1 year after its discovery, the optic nerve tumor demonstrated modest progression.
  • Although her left ventricular subependymal giant cell tumor demonstrated a 49% reduction in volume, the optic nerve tumor did not respond, and even underwent slight (6%) growth during the 16-month treatment.
  • The quality of this child's vision has remained normal in both eyes, and she is otherwise asymptomatic with regard to the optic nerve tumor.
  • [MeSH-major] Astrocytoma / drug therapy. Brain Neoplasms / drug therapy. Neoplasm Recurrence, Local / drug therapy. Optic Nerve Neoplasms / drug therapy. Sirolimus / therapeutic use. Tuberous Sclerosis / complications
  • [MeSH-minor] Antibiotics, Antineoplastic / therapeutic use. Child. Female. Humans. Treatment Outcome

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  • [Copyright] Copyright 2010 Elsevier Inc. All rights reserved.
  • (PMID = 20472200.001).
  • [ISSN] 1873-5150
  • [Journal-full-title] Pediatric neurology
  • [ISO-abbreviation] Pediatr. Neurol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; W36ZG6FT64 / Sirolimus
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15. van der Wal EJ, Azzarelli B, Edwards-Brown M: Malignant transformation of a chiasmatic pilocytic astrocytoma in a patient with diencephalic syndrome. Pediatr Radiol; 2003 Mar;33(3):207-10
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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 chiasmatic pilocytic astrocytoma in a patient with diencephalic syndrome.
  • We report leptomeningeal spread of a chiasmatic pilocytic astrocytoma in a child presenting with diencephalic syndrome.
  • He was treated with chemotherapy and radiation.
  • The tumor recurred with transformation into a high-grade astrocytoma.
  • Radiation therapy may have played a role in transformation of the tumor, but more research is needed to further clarify the biological behavior of this tumor.
  • [MeSH-major] Astrocytoma / secondary. Cell Transformation, Neoplastic / pathology. Diencephalon / physiopathology. Meningeal Neoplasms / secondary. Neoplasm Invasiveness / pathology. Optic Chiasm / pathology. Optic Nerve Neoplasms / pathology
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Biopsy, Needle. Child, Preschool. Combined Modality Therapy. Cranial Irradiation. Follow-Up Studies. Humans. Immunohistochemistry. Magnetic Resonance Imaging. Male. Risk Assessment. Syndrome

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  • (PMID = 12612823.001).
  • [ISSN] 0301-0449
  • [Journal-full-title] Pediatric radiology
  • [ISO-abbreviation] Pediatr Radiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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16. Bohner G, Masuhr F, Distl R, Katchanov J, Klingebiel R, Zschenderlein R, von Deimling A, van Landeghem FK: Pilocytic astrocytoma presenting as primary diffuse leptomeningeal gliomatosis: report of a unique case and review of the literature. Acta Neuropathol; 2005 Sep;110(3):306-11
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  • [Title] Pilocytic astrocytoma presenting as primary diffuse leptomeningeal gliomatosis: report of a unique case and review of the literature.
  • We describe a 25-year-old male patient with primary diffuse leptomeningeal gliomatosis (PDLG) presenting with gait ataxia, positive Lhermitte's sign, double vision, and right abducens nerve palsy.
  • Spinal leptomeningeal biopsy revealed a pilocytic astrocytoma WHO grade I.
  • Despite chemotherapy with vincristin and carboplatin, the patient died 2 months after admission.
  • A thorough autopsy showed no evidence for primary neoplasms in brain, spine and optic nerve.
  • Sequence analysis of tumor protein 53 gene (TP53) revealed a missense mutation in exon 5, and expression of phosphatase and tensin homolog (mutated in multiple advanced cancers 1) (PTEN) protein was not detected, which may have contributed to astrocytoma development.
  • To our knowledge, this is the first definitive case of pilocytic astrocytoma presenting as PDLG.
  • [MeSH-major] Astrocytoma / pathology. Meningeal Neoplasms / pathology. Meninges / pathology. Neoplasms, Neuroepithelial / pathology. Neoplasms, Unknown Primary / pathology. Subarachnoid Space / pathology
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biomarkers, Tumor / genetics. Brain / pathology. Brain / physiopathology. Diagnosis, Differential. Fatal Outcome. Humans. Magnetic Resonance Imaging. Male. Mutation / genetics. Spinal Cord / pathology. Spinal Cord / physiopathology. Spinal Cord Compression / etiology. Spinal Cord Compression / pathology. Spinal Cord Compression / physiopathology. Tumor Suppressor Protein p53 / genetics

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  • (PMID = 16003541.001).
  • [ISSN] 0001-6322
  • [Journal-full-title] Acta neuropathologica
  • [ISO-abbreviation] Acta Neuropathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / TP53 protein, human; 0 / Tumor Suppressor Protein p53
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17. Liang CL, Lu K, Liliang PC, Chen HJ: Gamma Knife surgery for optic glioma. Report of 2 cases. J Neurosurg; 2010 Dec;113 Suppl:44-7
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  • [Title] Gamma Knife surgery for optic glioma. Report of 2 cases.
  • Optic pathway/hypothalamic gliomas represent approximately 2%-5% of brain tumors in children.
  • Total excision, subtotal excision, subtotal excision followed by irradiation, radiation therapy alone, chemotherapy, and no treatment at all have been reported.
  • In this article the authors discuss the results of Gamma Knife surgery (GKS) for optic gliomas in 2 children.
  • Two pediatric patients, a boy and a girl, underwent GKS for optic gliomas at our hospital between March 2005 and August 2005.
  • The histological diagnosis was confirmed to be pilocytic astrocytoma in both cases.
  • The tumor involved the optic chiasm in 1 patient and the right optic nerve in the other patient.
  • Treatments were planned with the prescription of 11 Gy to the 50% isodose line for the optic chiasm glioma and 15 Gy to the 50% isodose line for the optic nerve glioma.
  • During the follow-up period, neither of the patients developed any endocrine dysfunction.
  • Gamma Knife surgery permits treatment of optic glioma with good tumor control and no clinically relevant morbidity.
  • With the ability to deliver a high dose to the tumor while sparing normal brain tissue, especially the optic nerve, optic chiasm, and pituitary gland, GKS should be the choice of treatment for optic gliomas.
  • [MeSH-major] Astrocytoma / surgery. Optic Nerve / surgery. Optic Nerve Glioma / surgery. Radiosurgery / instrumentation
  • [MeSH-minor] Adolescent. Child. Female. Humans. Magnetic Resonance Imaging. Male. Treatment Outcome

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  • (PMID = 21121786.001).
  • [ISSN] 1933-0693
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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18. 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.
  • On the contrary, the pineal cystic mass that was also histologically identified as a pilocytic astrocytoma showed marked enlargement within 5 months after a subtotal resection.
  • 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


19. 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.
  • 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.
  • 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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20. 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.
  • Histologic studies showed low-grade astrocytoma (WHO grade I or II) in 16 cases, anaplastic astrocytoma in 1, and oligoastrocytoma (WHO grade III) in 1.
  • Treatment included partial tumor resection in 12 patients, chemotherapy in 5, and radiotherapy in 3.
  • 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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21. Marec-Berard P, Szathmari A, Conter C, Mottolese C, Berlier P, Frappaz D: Improvement of diencephalic syndrome after partial surgery of optic chiasm glioma. Pediatr Blood Cancer; 2009 Sep;53(3):502-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Improvement of diencephalic syndrome after partial surgery of optic chiasm glioma.
  • Three cycles of chemotherapy were given, resulting in stable disease on MRI, but growth failure despite attempts at enteral feeding.
  • Pathological examination revealed a pilocytic astrocytoma.
  • [MeSH-major] Hypothalamic Diseases / etiology. Optic Chiasm. Optic Nerve Glioma / complications. Optic Nerve Glioma / surgery

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  • [Copyright] (c) 2009 Wiley-Liss,
  • (PMID = 19489055.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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22. Huber J, Sovinz P, Lackner H, Mokry M, Eder H, Urban C: Diencephalic syndrome: a frequently delayed diagnosis in failure to thrive. Klin Padiatr; 2007 Mar-Apr;219(2):91-4
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  • It is associated with neoplastic lesions of the hypothalamic-optic chiasmatic region.
  • Treatment options consist of surgical resection, radiation therapy (RT) and chemotherapy.
  • We describe the clinical course of two children suffering from diencephalic syndrome due to unresectable hypothalamic gliomas and emphasize the importance of chemotherapy as a first-line treatment.
  • Imaging of the brain showed a suprasellar mass, identified histologically as low grade pilocytic astrocytoma.
  • Both patients were treated with chemotherapy which induced tumor regression and stable disease.
  • CONCLUSIONS: Diencephalic syndrome caused by a hypothalamic/chiasmatic astrocytoma is a rare cause of failure to thrive in children so that diagnosis is frequently delayed.
  • Since most of these tumors in that specific anatomic site are regarded to be unresectable, chemotherapy including carboplatin and vincristine may reveal clinical improvement in these patients.
  • [MeSH-major] Astrocytoma / diagnosis. Failure to Thrive / etiology. Hypothalamic Diseases / diagnosis. Hypothalamic Neoplasms / diagnosis. Optic Chiasm. Optic Nerve Neoplasms / diagnosis
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Adjuvant. Child, Preschool. Combined Modality Therapy. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Hydrocephalus / etiology. Infant. Neoplasm, Residual / drug therapy

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  • (PMID = 17405074.001).
  • [ISSN] 0300-8630
  • [Journal-full-title] Klinische Pädiatrie
  • [ISO-abbreviation] Klin Padiatr
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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23. 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.
  • [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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24. Gnekow AK, Kortmann RD, Pietsch T, Emser A: Low grade chiasmatic-hypothalamic glioma-carboplatin and vincristin chemotherapy effectively defers radiotherapy within a comprehensive treatment strategy -- report from the multicenter treatment study for children and adolescents with a low grade glioma -- HIT-LGG 1996 -- of the Society of Pediatric Oncology and Hematology (GPOH). Klin Padiatr; 2004 Nov-Dec;216(6):331-42
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Low grade chiasmatic-hypothalamic glioma-carboplatin and vincristin chemotherapy effectively defers radiotherapy within a comprehensive treatment strategy -- report from the multicenter treatment study for children and adolescents with a low grade glioma -- HIT-LGG 1996 -- of the Society of Pediatric Oncology and Hematology (GPOH).
  • Early radiotherapy (RT) shall be deferred by chemotherapy (CT) within the concept of the HIT-LGG 1996 study, offering a comprehensive treatment strategy for all age groups.
  • Histology showed 132 pilocytic astrocytoma I degrees , 6 astrocytoma II degrees /nos and 2 DIGG/DIA I degrees (3 not known).
  • RESULTS: 82 children were treated at diagnosis, 68 upon clinical or radiological progression following observation times of 3.0 to 115.0 months.
  • At a median observation time of 50.1 months 21 tumors are stable, 3 regressive (2 not evaluable, 1 death).
  • At a median observation time of 44.7 months 2 children are in complete remission, 92 tumors are stable, 8 regressive, 9 progressive.
  • CONCLUSION: Within the comprehensive treatment strategy for low grade glioma HIT-LGG 1996 chemotherapy is effective to delay the need for early radiotherapy in chiasmatic-hypothalamic glioma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / therapy. Glioma / therapy. Hypothalamus. Optic Chiasm. Optic Nerve Neoplasms / therapy
  • [MeSH-minor] Adolescent. Age Factors. Antineoplastic Agents / administration & dosage. Antineoplastic Agents, Phytogenic / administration & dosage. Carboplatin / administration & dosage. Child. Child, Preschool. Combined Modality Therapy. Data Interpretation, Statistical. Female. Follow-Up Studies. Humans. Infant. Male. Radiotherapy Dosage. Time Factors. Vincristine / administration & dosage

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  • (PMID = 15565548.001).
  • [ISSN] 0300-8630
  • [Journal-full-title] Klinische Pädiatrie
  • [ISO-abbreviation] Klin Padiatr
  • [Language] eng
  • [Publication-type] Clinical Trial; Comparative Study; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Antineoplastic Agents, Phytogenic; 5J49Q6B70F / Vincristine; BG3F62OND5 / Carboplatin
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25. Akiyama H, Nakamizo S, Kawamura A, Nagashima T, Takeda H, Hasegawa D, Kosaka Y, Yoshida M: [Management of chiasmatic-hypothalamic gliomas in children: report of nine pediatric cases]. No Shinkei Geka; 2007 Nov;35(11):1079-85
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Radical resection of chiasmatic-hypothalamic glioma (CHG) carries a significant risk of morbidity and the optimum treatment remains undecided.
  • The authors reported 9 children with CHG, who were treated with surgical resection with or without postoperative chemotherapy.
  • Age at the time of diagnosis ranged from 4 months to 7.7 years (mean 3.1 years), and no patient had evidence of neurofibromatosis type 1.
  • Pathological examination revealed pilocytic astrocytomas in 7 patients, low grade astrocytoma in 1 and anaplastic astrocytoma in 1.
  • Seven patients with residual tumors received postoperative chemotherapy consisting of cisplatin, cyclophosphamide, etoposide and vincristine.
  • Although minor complications of chemotherapy were noticed in 5 patients, severe sequelae such as neuropsychological deficits or endocrinopathies did not occur, and all patients completed chemotherapy programs.
  • Additional treatments are recommended in case of incomplete tumor resections, because our experience demonstrates that the majority of the residual tumors have potential to progress.
  • Our present data suggests that the chemotherapy of the aforementioned regimen is effective in controlling CHGs after partial resections and is relatively well tolerated even in young children who are vulnerable to radiotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Glioma / surgery. Hypothalamic Neoplasms / surgery. Optic Chiasm. Optic Nerve Glioma / surgery
  • [MeSH-minor] Child. Child, Preschool. Cisplatin / administration & dosage. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Drug Administration Schedule. Etoposide / administration & dosage. Female. Humans. Infant. Male. Vincristine / administration & dosage

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  • (PMID = 18044225.001).
  • [ISSN] 0301-2603
  • [Journal-full-title] No shinkei geka. Neurological surgery
  • [ISO-abbreviation] No Shinkei Geka
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; Q20Q21Q62J / Cisplatin
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26. 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.
  • 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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27. Dasgupta B, Yi Y, Chen DY, Weber JD, Gutmann DH: Proteomic analysis reveals hyperactivation of the mammalian target of rapamycin pathway in neurofibromatosis 1-associated human and mouse brain tumors. Cancer Res; 2005 Apr 1;65(7):2755-60
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Based on the ability of the NF1 gene product (neurofibromin) to function as a GTPase activating protein for RAS, initial biologically based therapies for NF1-associated tumors focused on the use of RAS inhibitors, but with limited clinical success.
  • In an effort to identify additional targets for therapeutic drug design in NF1, we used an unbiased proteomic approach to uncover unanticipated intracellular signaling pathways dysregulated in Nf1-deficient astrocytes.
  • This mTOR pathway hyperactivation was reflected by high levels of ribosomal S6 activation in both Nf1 mutant mouse optic nerve gliomas and in human NF1-associated pilocytic astrocytoma tumors.
  • These results suggest that mTOR pathway inhibition may represent a logical and tractable biologically based therapy for brain tumors in NF1.

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  • (PMID = 15805275.001).
  • [ISSN] 0008-5472
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Neurofibromin 1; 0 / RNA, Messenger; EC 2.7.- / Protein Kinases; EC 2.7.1.- / Phosphatidylinositol 3-Kinases; EC 2.7.1.1 / MTOR protein, human; EC 2.7.1.1 / TOR Serine-Threonine Kinases; EC 2.7.1.1 / mTOR protein, mouse; EC 2.7.11.1 / Ribosomal Protein S6 Kinases, 90-kDa; W36ZG6FT64 / Sirolimus
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