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1. 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.
  • Neuroimaging disclosed a chiasmatic-hypothalamic glioma.
  • 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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2. Osztie E, Várallyay P, Doolittle ND, Lacy C, Jones G, Nickolson HS, Neuwelt EA: Combined intraarterial carboplatin, intraarterial etoposide phosphate, and IV Cytoxan chemotherapy for progressive optic-hypothalamic gliomas in young children. AJNR Am J Neuroradiol; 2001 May;22(5):818-23
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  • [Title] Combined intraarterial carboplatin, intraarterial etoposide phosphate, and IV Cytoxan chemotherapy for progressive optic-hypothalamic gliomas in young children.
  • BACKGROUND AND PURPOSE: Optic pathway and/or hypothalamic astrocytomas in children are often quiescent, but in some cases, more aggressive tumors may cause progressive visual, endocrine, and neurologic deterioration.
  • The initial treatment of these gliomas includes surgery and IV chemotherapy.
  • This report suggests a new approach using combined intraarterial and IV carboplatin-based chemotherapy for patients for whom first line treatment has already failed.
  • METHODS: Six children (mean age, 57 months) with the diagnosis of optic pathway hypothalamic gliomas, who had tumor progression after surgery and underwent IV chemotherapy, were treated monthly with intraarterially administered carboplatin, intraarterially administered etoposide phosphate, and IV administered Cytoxan.
  • 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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3. Fouladi M, Wallace D, Langston JW, Mulhern R, Rose SR, Gajjar A, Sanford RA, Merchant TE, Jenkins JJ, Kun LE, Heideman RL: Survival and functional outcome of children with hypothalamic/chiasmatic tumors. Cancer; 2003 Feb 15;97(4):1084-92
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  • [Title] Survival and functional outcome of children with hypothalamic/chiasmatic tumors.
  • BACKGROUND: The management of children with hypothalamic (H) and/or chiasmatic (C) tumors remains controversial.
  • We evaluated the impact of clinical and neuroimaging parameters and primary therapy on overall (OS) and progression-free (PFS) survival and on neuroendocrine and neurocognitive outcome in children with H and/or C tumors.
  • RESULTS: Thirty-six patients received irradiation or chemotherapy immediately postdiagnosis and 37 were observed.
  • The 6-year PFS rates for the irradiation, chemotherapy, and observation groups were 69 +/- 16%, 12 +/- 11%, and 37 +/- 9%, respectively.
  • Mean serial intelligence quotient (IQ) scores were 86 and 86 at diagnosis and at 6 years later, respectively.
  • Patients younger than 5 years old had a lower mean IQ score at diagnosis (79.1) than older patients (96.3; P = 0.003).
  • Patients who were irradiated at diagnosis had a significantly higher cumulative incidence of endocrinopathy at 3 years (P = 0.008).
  • Initial treatment with radiation and the presence of intracranial NF1 lesions were positive predictors of PFS.
  • Mean IQ is significantly compromised at diagnosis, but does not change over time or with irradiation.
  • We recommend observation in asymptomatic patients, platinum-based chemotherapy in younger patients, and irradiation in older symptomatic patients.
  • [MeSH-major] Glioma / drug therapy. Glioma / radiotherapy. Hypothalamic Neoplasms / drug therapy. Hypothalamic Neoplasms / radiotherapy
  • [MeSH-minor] Adolescent. Age Factors. Astrocytoma / drug therapy. Astrocytoma / radiotherapy. Child. Child, Preschool. Endocrine System Diseases / etiology. Female. Humans. Infant. Intelligence Tests. Male. Treatment Outcome

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  • [Copyright] Copyright 2003 American Cancer Society
  • (PMID = 12569610.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / PA30CA 21765
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
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4. Gupta DK, Satyarthee GD, Sharma MC, Mahapatra AK: Hypothalamic glioma presenting with seizures. a case report and review of the literature. Pediatr Neurosurg; 2006;42(4):249-53
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  • [Title] Hypothalamic glioma presenting with seizures. a case report and review of the literature.
  • A rare case of hypothalamic glioma in a 7-year-old boy presenting with seizures and diabetes insipidus is reported.
  • Near total decompression of the hypothalamic glioma was done successfully using subfrontal approach.
  • Postoperative radiotherapy/chemotherapy was not given in view of tumor histology (low grade glioma), patient's age and tumor location.
  • [MeSH-major] Epilepsy, Tonic-Clonic / etiology. Glioma / diagnosis. Hypothalamic Neoplasms / diagnosis

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  • [Copyright] Copyright (c) 2006 S. Karger AG, Basel.
  • (PMID = 16714868.001).
  • [ISSN] 1016-2291
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
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5. 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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  • [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).
  • BACKGROUND: Low grade gliomas arise in all CNS-locations and age groups, chiasmatic-hypothalamic tumors occur especially in young children.
  • 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.
  • PATIENTS: 198 of 905 protocol patients (21.9 %) had a chiasmatic (34), chiasmatic-hypothalamic (144) or hypothalamic (20) primary tumor, median age at diagnosis 3.6 years (0.2-16.3 y.), 54 had neurofibromatosis (27.3 %), 108 female (54.5 %).
  • The initial neurosurgical intervention resulted in 5 complete, 26 subtotal, 45 partial resections, 67 biopsies; 55 children had a diagnosis on the basis of neuroradiologic findings.
  • 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).
  • 44/123 tumors were progressive after median 22.5 months, 37 with a chiasmatic-hypothalamic primary, 16/44 were irradiated.
  • At a median observation time of 44.7 months 2 children are in complete remission, 92 tumors are stable, 8 regressive, 9 progressive.
  • Within the CT-group children with an age at diagnosis < 1 year and non-pilocytic histology are at increased risk for early progression.
  • 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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6. Takasu M, Takeshita S, Tanitame N, Tamura A, Mori M, Fujihara M, Ito K: Case report. Primary hypothalamic third ventriclular Burkitt's lymphoma: a case report with emphasis on differential diagnosis. Br J Radiol; 2010 Feb;83(986):e43-7
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  • [Title] Case report. Primary hypothalamic third ventriclular Burkitt's lymphoma: a case report with emphasis on differential diagnosis.
  • A patient with primary Burkitt-type lymphoma of the central nervous system is presented.
  • A hypothalamic-third ventricular tumour in a man 71 years of age was diagnosed histologically as Burkitt's lymphoma.
  • Primary Burkitt's lymphoma of the hypothalamic region is extremely rare and has not been previously reported in adults.
  • [MeSH-major] Burkitt Lymphoma / diagnosis. Glioma / diagnosis. Hypothalamic Neoplasms / diagnosis
  • [MeSH-minor] Adult. Aged. Child. Child, Preschool. Diabetes Insipidus / diagnosis. Diabetes Insipidus / drug therapy. Diagnosis, Differential. Humans. Magnetic Resonance Imaging. Male. Third Ventricle. Tomography, X-Ray Computed

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  • (PMID = 20139257.001).
  • [ISSN] 1748-880X
  • [Journal-full-title] The British journal of radiology
  • [ISO-abbreviation] Br J Radiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC3473536
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7. 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.
  • They were grouped into three groups on the basis of radiological features and treated with a suspected diagnosis.
  • The final diagnosis was correlated with preoperative diagnosis, and the feasibility of managing these lesions without a histopathological confirmation is discussed.
  • 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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8. Mishra KK, Squire S, Lamborn K, Banerjee A, Gupta N, Wara WM, Prados MD, Berger MS, Haas-Kogan DA: Phase II TPDCV protocol for pediatric low-grade hypothalamic/chiasmatic gliomas: 15-year update. J Neurooncol; 2010 Oct;100(1):121-7
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  • [Title] Phase II TPDCV protocol for pediatric low-grade hypothalamic/chiasmatic gliomas: 15-year update.
  • To report long-term results for children with low-grade hypothalamic/chiasmatic gliomas treated on a phase II chemotherapy protocol.
  • Between 1984 and 1992, 33 children with hypothalamic/chiasmatic LGGs received TPDCV chemotherapy on a phase II prospective trial.
  • Age at diagnosis was significantly associated with relapse and survival (P = 0.004 for PFS and P = 0.037 for OS).
  • Upfront TPDCV for children with hypothalamic/chiasmatic LGGs resulted in 15-year OS of 71.2% and 15-year PFS of 23.4%.

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  • (PMID = 20221671.001).
  • [ISSN] 1573-7373
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA 82103; United States / NCI NIH HHS / CA / P30 CA082103; United States / NCI NIH HHS / CA / P50 CA097257; United States / NINDS NIH HHS / NS / P01 NS042927; United States / NINDS NIH HHS / NS / P01 NS-42927-27A2
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 35S93Y190K / Procarbazine; 5J49Q6B70F / Vincristine; 7BRF0Z81KG / Lomustine; FTK8U1GZNX / Thioguanine; LJ2P1SIK8Y / Mitolactol
  • [Other-IDs] NLM/ PMC2951507
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9. 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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  • [Title] [Management of chiasmatic-hypothalamic gliomas in children: report of nine pediatric cases].
  • 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.
  • 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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10. Badawi MA, Salih F, Al-Humaidi AA, El Khalifa MY, Elhadd TA: Bone marrow hypoplasia responsive to testosterone therapy in a patient with panhypopituitarism: need for adherence to androgen replacement. Endocr Pract; 2008 Mar;14(2):229-32
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Bone marrow hypoplasia responsive to testosterone therapy in a patient with panhypopituitarism: need for adherence to androgen replacement.
  • OBJECTIVE: To describe the case of a young Saudi male patient with long-term panhypopituitarism and pancytopenia attributable to poor adherence to androgen replacement therapy, which resolved after institution of testosterone treatment and recurred after another interval of poor adherence to recommended therapy.
  • METHODS: We present the clinical and laboratory data before and after treatment with testosterone.
  • RESULTS: After resection of a hypothalamic glioma, panhypopituitarism developed in a 14-year-old Saudi boy.
  • After treatment of sepsis, the pancytopenia persisted.
  • The patient's family indicated that he had not been taking his testosterone therapy.
  • Maintenance therapy with testosterone once weekly for 3 weeks and then once every 3 weeks resulted in improved hematologic findings.
  • The patient again discontinued his testosterone treatment, and the hematologic abnormalities recurred but were again corrected after supervised testosterone therapy.
  • CONCLUSION: This case emphasizes the importance of androgen replacement therapy in patients with hypopituitarism, not only for sexual potency, bone strength, and quality of life but also for normal bone marrow function.
  • [MeSH-major] Androgens / therapeutic use. Bone Marrow / drug effects. Hormone Replacement Therapy. Hypopituitarism / drug therapy
  • [MeSH-minor] Adolescent. Bone Marrow Diseases / drug therapy. Bone Marrow Diseases / etiology. Bone Marrow Diseases / pathology. Humans. Male. Pancytopenia / complications. Pancytopenia / drug therapy. Pancytopenia / pathology. Testosterone / therapeutic use

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  • (PMID = 18308664.001).
  • [ISSN] 1934-2403
  • [Journal-full-title] Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists
  • [ISO-abbreviation] Endocr Pract
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Androgens; 3XMK78S47O / Testosterone
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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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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.
  • 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. Packer RJ: Chemotherapy: low-grade gliomas of the hypothalamus and thalamus. Pediatr Neurosurg; 2000 May;32(5):259-63
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Chemotherapy: low-grade gliomas of the hypothalamus and thalamus.
  • Chemotherapy is an increasing component of the management of diencephalic gliomas.
  • However, decisions concerning the institution of treatment should be based on the goals of treatment.
  • (2) whether the child has neurofibromatosis type 1;.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Glioma / drug therapy. Hypothalamic Neoplasms / drug therapy. Supratentorial Neoplasms / drug therapy
  • [MeSH-minor] Adult. Age Factors. Chemotherapy, Adjuvant. Child. Clinical Trials as Topic. Disease-Free Survival. Humans. Patient Selection. Severity of Illness Index

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  • (PMID = 10965273.001).
  • [ISSN] 1016-2291
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 20
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13. Ronghe M, Hargrave D, Bartels U, Tabori U, Vaidya S, Chandler C, Kulkarni A, Bouffet E: Vincristine and carboplatin chemotherapy for unresectable and/or recurrent low-grade astrocytoma of the brainstem. Pediatr Blood Cancer; 2010 Sep;55(3):471-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Vincristine and carboplatin chemotherapy for unresectable and/or recurrent low-grade astrocytoma of the brainstem.
  • BACKGROUND: Radiotherapy remains a widely accepted postoperative treatment modality for unresectable or recurrent low-grade glioma (LGG).
  • However, there is increasing evidence to suggest that chemotherapy can delay and may obviate the need for radiotherapy in progressive/recurrent LGG.
  • The majority of the published experience is in children with hypothalamic/optic chiasmatic lesions and little information is available regarding its use in LGG of the brainstem.
  • PROCEDURE: We describe clinical characteristics and course of children with LGG of the brainstem who received carboplatin-based chemotherapy in two institutions over 10 years (1996-2006).
  • Vincristine and carboplatin were first-line chemotherapy regimen used in all patients.
  • RESULTS: In this series, there were 16 children (9 males) with median age at diagnosis of 4.2 years (range 0.5-8).
  • Eight children were treated at diagnosis while the remaining eight received chemotherapy after either radiological progression or clinical deterioration.
  • After a median follow-up of 57 months (range 20-136) from initiation of chemotherapy all children are alive and 11 remain progression free (1 complete response, 8 with partial response + minor response, and 2 stable diseases).
  • CONCLUSIONS: The efficacy of this chemotherapy regimen in this series supports its role in children with progressive unresectable LGG of brainstem.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Astrocytoma / drug therapy. Brain Stem. Brain Stem Neoplasms / drug therapy
  • [MeSH-minor] Carboplatin / administration & dosage. Child. Child, Preschool. Disease Progression. Female. Humans. Infant. Male. Neoplasm Recurrence, Local / drug therapy. Vincristine / administration & dosage

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  • [Copyright] 2010 Wiley-Liss, Inc.
  • (PMID = 20535831.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; BG3F62OND5 / Carboplatin
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14. Peyrl A, Azizi A, Czech T, Gruber-Olipitz M, Jones N, Haberler C, Prayer D, Autzinger E, Slavc I: Tumor stabilization under treatment with imatinib in progressive hypothalamic-chiasmatic glioma. Pediatr Blood Cancer; 2009 Apr;52(4):476-80
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Tumor stabilization under treatment with imatinib in progressive hypothalamic-chiasmatic glioma.
  • BACKGROUND: Hypothalamic-chiasmatic gliomas (HCG) account for up to 20% of tumors in patients under the age of 3 years.
  • While most children respond to chemotherapy, alternative treatment approaches are needed for those with progressive disease refractory to chemotherapy.
  • PROCEDURE: Six patients (median age: 5.5 years) with progressive HCG were treated with imatinib for 3-29 months at a median daily oral dose of 270 mg/m(2).
  • Disease control lasted from 5 to 46 months and was sustained longer in comparison to their last prior chemotherapy.
  • CONCLUSIONS: We conclude that imatinib has possible activity in progressive HCG and may present an additional therapeutic option for patients who are too young or whose tumor is too extensive to receive radiotherapy.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Glioma / drug therapy. Hypothalamic Neoplasms / drug therapy. Optic Nerve Glioma / drug therapy. Piperazines / therapeutic use. Pyrimidines / therapeutic use
  • [MeSH-minor] Benzamides. Child, Preschool. Female. Humans. Imatinib Mesylate. Immunohistochemistry. Infant. Male. Neoplasm Recurrence, Local / drug therapy. Optic Chiasm / drug effects. Optic Chiasm / pathology. Treatment Outcome

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  • [Copyright] Copyright 2008 Wiley-Liss, Inc.
  • (PMID = 19061223.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate
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15. Bartels UK, Hawkins C, Ma J, Ray A, Dirks P, Rutka J, Bouffet E: Microvessel density predicts behavior in pediatric optic pathway/hypothalamic gliomas. J Clin Oncol; 2004 Jul 15;22(14_suppl):1556

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Microvessel density predicts behavior in pediatric optic pathway/hypothalamic gliomas.
  • : 1556 Background: Optic pathway/hypothalamic gliomas (OPHG) are predominately low-grade tumors.
  • Those with sufficient tissue for staining were included in the study.
  • These factors were evaluated with respect to progression-free survival (PFS) and outcome of treatment.
  • 30 required additional treatment (16 chemotherapy, 8 radiation, 6 chemotherapy + radiation).
  • CONCLUSIONS: Our findings suggest that angiogenesis is important in low-grade glioma and MD rather than MIB-1 has a prognostic value in OPHGs.

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  • (PMID = 28015451.001).
  • [ISSN] 1527-7755
  • [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
  • [Publication-country] United States
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16. Vanhauwaert DJ, Clement F, Van Dorpe J, Deruytter MJ: Chordoid glioma of the third ventricle. Acta Neurochir (Wien); 2008 Nov;150(11):1183-91
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Chordoid glioma of the third ventricle.
  • BACKGROUND: Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle with both glial and chordoid features.
  • Since there is no detailed information available on the outcome after surgery and adjuvant treatment, we reviewed the literature.
  • METHODS: A literature search through PUBMED revealed 50 cases of chordoid glioma.
  • We reviewed the available literature and studied in detail the presenting symptoms, mortality and postoperative complications in relation to the extent of resective surgery, as well as the importance of adjuvant treatment.
  • Non-fatal postoperative complications are hypothalamic disorders and mental alterations.
  • Gross total resection is the treatment of choice since no recurrence has been reported after macroscopically complete resection, but this is often difficult because of the location and adherence to the hypothalamus.
  • There is no report on the use of chemotherapy in the treatment of chordoid gliomas.
  • More information about the optimal treatment strategy is needed, and more reports are also needed.
  • [MeSH-major] Cerebral Ventricle Neoplasms / pathology. Cerebral Ventricle Neoplasms / surgery. Glioma / pathology. Glioma / surgery. Third Ventricle / pathology
  • [MeSH-minor] Female. Humans. Hypothalamic Diseases / etiology. Hypothalamic Diseases / pathology. Hypothalamic Diseases / physiopathology. Male. Neoplasm Invasiveness / pathology. Neoplasm Invasiveness / physiopathology. Neurosurgical Procedures / adverse effects. Neurosurgical Procedures / methods. Neurosurgical Procedures / mortality. Postoperative Complications / mortality. Postoperative Complications / physiopathology. Radiosurgery / methods. Radiosurgery / standards. Sex Distribution

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  • (PMID = 18936876.001).
  • [ISSN] 0942-0940
  • [Journal-full-title] Acta neurochirurgica
  • [ISO-abbreviation] Acta Neurochir (Wien)
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Austria
  • [Number-of-references] 39
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17. Kim RJ, Janss A, Shanis D, Homan S, Moshang T Jr: Adult heights attained by children with hypothalamic/chiasmatic glioma treated with growth hormone. J Clin Endocrinol Metab; 2004 Oct;89(10):4999-5002
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adult heights attained by children with hypothalamic/chiasmatic glioma treated with growth hormone.
  • Hypothalamic/chiasmatic gliomas (H/CG) in children are commonly accompanied by endocrine dysfunction due to mass effects of the tumor itself or as a consequence of tumor therapy, with GH deficiency (GHD) being the most common disorder.
  • For GH-treated patients, the mean initial height was -0.7 +/- 0.3 (+/-se).
  • The mean initial and final height SDS for the non-GHD patients were 0.6 (se = 0.4) and 0.0 (se = 0.4), respectively.
  • GH treatment for H/CG patients restores much of their growth potential and improves adult height to within normal limits.
  • [MeSH-major] Body Height / drug effects. Glioma / drug therapy. Human Growth Hormone / therapeutic use. Hypothalamic Neoplasms / drug therapy

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  • (PMID = 15472197.001).
  • [ISSN] 0021-972X
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 12629-01-5 / Human Growth Hormone
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18. Fernandes JK, Klein MJ, Ater JL, Kuttesch JF, Vassilopoulou-Sellin R: Triiodothyronine supplementation for hypothalamic obesity. Metabolism; 2002 Nov;51(11):1381-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Triiodothyronine supplementation for hypothalamic obesity.
  • Patients with suprasellar lesions develop profound hypothalamic obesity and listlessness with no effective treatment.
  • A 10.6-year-old boy (optic glioma) was gaining 6 kg/yr for 4 years; after T(3) supplement, he lost 4.3 kg over 11 months.
  • We suggest that T(3) may serve as a simple and effective supplement, which can promote weight loss and improve the well being of these patients with hypothalamic obesity.
  • [MeSH-major] Brain Neoplasms / complications. Hypothalamic Diseases / complications. Hypothalamic Diseases / drug therapy. Obesity / etiology. Triiodothyronine / therapeutic use. Weight Loss
  • [MeSH-minor] Adult. Child. Female. Humans. Male. Treatment Outcome


19. Siffert J, Allen JC: Late effects of therapy of thalamic and hypothalamic tumors in childhood: vascular, neurobehavioral and neoplastic. Pediatr Neurosurg; 2000 Aug;33(2):105-11
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Late effects of therapy of thalamic and hypothalamic tumors in childhood: vascular, neurobehavioral and neoplastic.
  • The late effects in children with hypothalamic and thalamic tumors relate to the effects of the tumor on the surrounding brain, the effects of surgery, radiotherapy (RT) and, to a lesser extent, chemotherapy.
  • The prevention of late effects is an integral part of current treatment strategies.
  • Early diagnosis, a rational use of surgery, and deferral of RT are the mainstays of the modern treatment in these patients.
  • The improvement of RT techniques and the use of radioprotective compounds may further help spare normal brain tissue.
  • A better understanding of chemotherapy use and the development of newer agents may increase efficacy, reduce side effects and allow deferral of RT in a greater percentage of patients.
  • [MeSH-major] Brain Neoplasms / pathology. Brain Neoplasms / radiotherapy. Glioma / pathology. Hypothalamus / pathology. Hypothalamus / radiation effects. Neoplasms, Radiation-Induced / pathology. Thalamus / pathology. Thalamus / radiation effects. Visual Pathways / pathology. Visual Pathways / radiation effects

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  • [Copyright] Copyright 2000 S. Karger AG, Basel.
  • (PMID = 11070438.001).
  • [ISSN] 1016-2291
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
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20. Grabenbauer GG, Schuchardt U, Buchfelder M, Rödel CM, Gusek G, Marx M, Doerr HG, Fahlbusch R, Huk WJ, Wenzel D, Sauer R: Radiation therapy of optico-hypothalamic gliomas (OHG)--radiographic response, vision and late toxicity. Radiother Oncol; 2000 Mar;54(3):239-45

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Radiation therapy of optico-hypothalamic gliomas (OHG)--radiographic response, vision and late toxicity.
  • BACKGROUND: Management strategies for optic pathway gliomas include observation, surgery, irradiation, chemotherapy and a combination of these modalities.
  • It has been the policy of our University Hospital to consider radiation as the standard treatment for progressive optic pathway gliomas.
  • This report describes the clinical presentation, treatment patterns and outcome with special emphasis on the long term functional status of patients with optico-hypothalamic gliomas (OHG).
  • PATIENTS AND METHODS: Between 1975 and 1997, 25 patients with OHG were treated by radiation therapy (RT) following surgery or biopsy.
  • Age adjusted radiation doses ranged from 45 to 60 Gy with a single fraction size of 1.6-2 Gy.
  • Endpoints of the study were: radiographic response, survival, progression-free survival and time to endocrinologic toxicity as well as the visual function during follow-up.
  • The median follow-up time was 9 years (range, 1.5-23 years).
  • A significant influence on progression-free survival was noted for age at diagnosis (P=0.04) and total dose (P=0.05).
  • Nine out of 13 (69%) patients aged below 10 years compared with 3/12 (25%) patients aged above 10 years experienced hypothalamic-pituitary deficiency (P=0.008) during follow-up.
  • 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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21. Massimi L, Tufo T, Di Rocco C: Management of optic-hypothalamic gliomas in children: still a challenging problem. Expert Rev Anticancer Ther; 2007 Nov;7(11):1591-610

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Management of optic-hypothalamic gliomas in children: still a challenging problem.
  • Optic pathway-hypothalamic gliomas (OPHGs) are rare, often unresectable tumors that mostly occur in childhood.
  • Their biological behavior is unpredictable, although they tend to follow an aggressive clinical course in infants and a benign course in children with neurofibromatosis type 1.
  • Chemotherapy is increasingly used in the management of OPHGs, especially in infants, to delay radiotherapy.
  • Carboplatin and vincristine are the most frequently used drugs, although several chemotherapeutic agents in different combinations are currently employed with good results.
  • [MeSH-major] Hypothalamic Neoplasms / therapy. Optic Nerve Glioma / therapy
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Combined Modality Therapy. Humans

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  • (PMID = 18020927.001).
  • [ISSN] 1744-8328
  • [Journal-full-title] Expert review of anticancer therapy
  • [ISO-abbreviation] Expert Rev Anticancer Ther
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 148
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22. Jaing TH, Lin KL, Tsay PK, Hsueh C, Hung PC, Wu CT, Tseng CK: Treatment of optic pathway hypothalamic gliomas in childhood: experience with 18 consecutive cases. J Pediatr Hematol Oncol; 2008 Mar;30(3):222-4

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment of optic pathway hypothalamic gliomas in childhood: experience with 18 consecutive cases.
  • The aim of this study was to present our 17-year experience (1989 to 2006) in the treatment of optic pathway/hypothalamic gliomas (OPHG) in 18 children younger than 17 years (median age, 66 mo).
  • OPHG was diagnosed using computed tomography and/or magnetic resonance imaging.
  • Treatment included partial tumor resection in 12 patients, chemotherapy in 5, and radiotherapy in 3.
  • All treatment modalities led to tumor shrinkage and stabilization for a variable period, but none of them totally eradicated the tumor.
  • 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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23. 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.
  • The histological diagnosis was confirmed to be pilocytic astrocytoma in both cases.
  • 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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24. 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.
  • MRI showed a chiasma of the hypothalamic mass.
  • Three cycles of chemotherapy were given, resulting in stable disease on MRI, but growth failure despite attempts at enteral feeding.
  • [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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25. 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.
  • [Title] Initial management of children with hypothalamic and thalamic tumors and the modifying role of neurofibromatosis-1.
  • Optic pathway/hypothalamus gliomas (OPG) arise primarily from a slower-growing juvenile pilocytic astrocytoma, and thalamic gliomas arise primarily from a fibrillary astrocytoma which can become clinically and histologically more aggressive.
  • The major therapeutic challenge for these patients is to maximize their quality of life by preserving visual and endocrine function while minimizing treatment-related morbidity.
  • Treatment is often initiated at diagnosis in infants and toddlers who have a major visual impairment or the diencephalic syndrome.
  • The judicious application of chemotherapy may serve to forestall the need for radiotherapy or surgery.
  • However, over 90% of children with OPG without NF-1 will require some form of therapy.
  • Current multimodality therapy is relatively ineffective.
  • The bithalamic variant behaves similarly to a pontine glioma.
  • [MeSH-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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26. Steinbok P: Optic pathway tumors in children. J Chin Med Assoc; 2003 Jan;66(1):4-12
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  • 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.
  • On the other hand, chiasmatic/hypothalamic tumors grow like typical neoplasms.
  • 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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27. Ceppa EP, Bouffet E, Griebel R, Robinson C, Tihan T: The pilomyxoid astrocytoma and its relationship to pilocytic astrocytoma: report of a case and a critical review of the entity. J Neurooncol; 2007 Jan;81(2):191-6
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  • Pilomyxoid astrocytoma (PMA) is a rare glioma that shares histopathological similarities with pilocytic astrocytoma (PA).
  • Radiographic studies at 6 months of age (age at initial presentation) revealed a large hypothalamic lesion occupying proximal portions of the optic nerves, chiasm and right posterior optic tract.
  • The first biopsy obtained after two chemotherapy regimens was consistent with a diagnosis of PMA.
  • Some even suggested a different cell type, such as the tanycytic cell as the origin for PMA.
  • [MeSH-major] Astrocytoma / pathology. Hypothalamic Neoplasms / pathology. Myxoma / pathology
  • [MeSH-minor] Child. Diagnosis, Differential. Female. Humans. Magnetic Resonance Imaging

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  • (PMID = 16850101.001).
  • [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
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28. Rilliet B, Vernet O: Gliomas in children: a review. Childs Nerv Syst; 2000 Nov;16(10-11):735-41
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  • The different types of gliomas encountered in the pediatric population are reviewed, taking account of the most recent contributions on this subject.
  • Surgery, with all its recent refinements, remains the best treatment for the majority of benign gliomas providing they can be removed without unacceptable sequelae.
  • The role of chemotherapy has emerged recently for the treatment of nonresectable low-grade gliomas, such as hypothalamic-chiasmatic tumors, especially for infants, in whom the adverse effects of radiotherapy can be severe and irreversible.
  • [MeSH-major] Brain Neoplasms / surgery. Glioma / surgery

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  • (PMID = 11151725.001).
  • [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; Review
  • [Publication-country] Germany
  • [Number-of-references] 55
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29. Lerner SE, Huang GJ, McMahon D, Sklar CA, Oberfield SE: Growth hormone therapy in children after cranial/craniospinal radiation therapy: sexually dimorphic outcomes. J Clin Endocrinol Metab; 2004 Dec;89(12):6100-4
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  • [Title] Growth hormone therapy in children after cranial/craniospinal radiation therapy: sexually dimorphic outcomes.
  • Radiation therapy (RT) to the craniospinal region in childhood affects final height.
  • The use of GH treatment (GHRx) in children after cranial or craniospinal RT results in variable improvement in final height.
  • The median age at diagnosis was 5.4 yr, the median RT to the hypothalamic-pituitary axis was 40 Gy, the median spinal RT dose in 13 of 19 of the subjects treated was 36 Gy, and the median years post-RT to GHRx was 4.8 yr.
  • Adjuvant chemotherapy was used in 12 of 19 patients.
  • All but one (optic glioma) had a lesion anatomically distant from the suprasellar region.
  • The effects of age at diagnosis, sex, L-T4 or GnRH agonist use, conventional vs. hyperfractionated RT, spinal RT, dose of spinal or cranial RT, chemotherapy, peak stimulated GH, dose and duration of GHRx, age at GHRx, time interval between RT and GHRx initiation, bone age, and height SDS at the start of GHRx were also assessed.
  • Y1girls best correlated with younger age at diagnosis and im vs. sc GHRx.
  • Y2girls best correlated with delayed bone age and younger age at diagnosis [Y1girls = -9.95 + 0.38 (age in years at diagnosis) + 3.11[GH method (1 = i.m.
  • ; 2 = s.c. )]; r2 = 0.898; P = 0.02; Y2girls = -3.54 + 1.8 (bone age - age in years) + 0.334 (age at diagnosis in years); r2= 0.956; P = 0.02].
  • Both Y1boys and Y2boys were strongly associated with spinal RT and younger age at diagnosis or treatment [Y1boys = -11.22 + 4.65 [spinal RT (1 = yes; 2 = no)] + 0.396 (age in years at diagnosis); r2= 0.64, P = 0.01; Y2boys = -6.32 + 0.23 (age in years at GH start) + 1.75 [spinal RT (1 = yes; 2 = no)]; r2= 0.646; P < 0.01].
  • Intramuscular vs. sc use of GHRx is likely to be simply a surrogate marker for earlier methods of treatment.
  • [MeSH-major] Body Height / drug effects. Growth Hormone / therapeutic use. Head and Neck Neoplasms / drug therapy. Head and Neck Neoplasms / radiotherapy. Hypothalamo-Hypophyseal System / radiation effects. Sex Characteristics. Spine / radiation effects
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Cohort Studies. Female. Humans. Infant. Male. Retrospective Studies. Treatment Outcome

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  • [ErratumIn] J Clin Endocrinol Metab. 2005 Jan;90(1):16
  • (PMID = 15579765.001).
  • [ISSN] 0021-972X
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 9002-72-6 / Growth Hormone
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30. Johannesen TB, Lien HH, Hole KH, Lote K: Radiological and clinical assessment of long-term brain tumour survivors after radiotherapy. Radiother Oncol; 2003 Nov;69(2):169-76
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  • BACKGROUND AND PURPOSE: Late adverse effects of therapeutic brain radiotherapy (RT) may develop after long latency periods and our objective was to assess long-term brain tumour survivors following RT to large partial brain volumes.
  • Fraction dose was 1.8 Gy to a median total dose of 54 Gy (range: 45.0-59.4 Gy).
  • In 25 patients the hypothalamic and pituitary area had been included in the RT field.
  • Patients treated with intra-arterial chemotherapy and patients at higher age at follow-up had significantly more grade 3 changes.
  • [MeSH-major] Brain / radiation effects. Brain Neoplasms / radiotherapy. Glioma / radiotherapy. Radiation Injuries

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  • (PMID = 14643954.001).
  • [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; Research Support, Non-U.S. Gov't
  • [Publication-country] Ireland
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31. Saran FH, Baumert BG, Khoo VS, Adams EJ, Garré ML, Warrington AP, Brada M: Stereotactically guided conformal radiotherapy for progressive low-grade gliomas of childhood. Int J Radiat Oncol Biol Phys; 2002 May 1;53(1):43-51
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  • PURPOSE: To describe the rationale, technique, and early results of stereotactically guided conformal radiotherapy (SCRT) in the treatment of progressive or inoperable low-grade gliomas (LGGs) of childhood.
  • Tumors were located at the optic chiasm (n = 9), third ventricle (n = 2), hypothalamus, craniocervical junction, and pineal region (each n = 1).
  • Four patients received chemotherapy before SCRT.
  • The treatment technique consisted of 4 isocentric, noncoplanar, conformal, fixed fields.
  • Treatment was delivered in 30-33 daily fractions to a total dose of 50-55 Gy.
  • One child with optic chiasm glioma had local progression at 25 months, and 1 developed diffuse leptomeningeal disease without local progression at 27 months.
  • The frequency of delayed hypothalamic-pituitary axis dysfunction reflects tumor location adjacent to the hypothalamus and pituitary.
  • Additional follow-up is required to demonstrate that SCRT contributes to a reduction in treatment-related late toxicity, while maintaining the local control achieved with conventionally delivered RT in children with progressive LGGs.

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  • (PMID = 12007940.001).
  • [ISSN] 0360-3016
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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32. Sievert AJ, Fisher MJ: Pediatric low-grade gliomas. J Child Neurol; 2009 Nov;24(11):1397-408
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  • Surgery is the mainstay of therapy.
  • Overall survival rates for patients whose tumors are completely resected are 90% or greater, 10 years from diagnosis.
  • Conversely, most optic pathway/hypothalamic, deep midline, and brain stem gliomas have minimal potential for resection; these tumors can be difficult to treat and deserve special attention.
  • Combination chemotherapy is currently recommended as front-line adjuvant treatment for progressive or recurrent tumors.

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  • (PMID = 19841428.001).
  • [ISSN] 1708-8283
  • [Journal-full-title] Journal of child neurology
  • [ISO-abbreviation] J. Child Neurol.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA133173-02; United States / NCI NIH HHS / CA / R21 CA133173; United States / NCI NIH HHS / CA / R21 CA133173-02
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 146
  • [Other-IDs] NLM/ NIHMS208342; NLM/ PMC2917804
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33. 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.
  • 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.
  • Although the major complication of an OPG is visual loss, hypothalamic involvement can lead to death.
  • 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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34. 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.
  • 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.
  • 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.
  • 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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35. Starzyk J, Pituch-Noworolska A, Pietrzyk JA, Urbanik A, Kroczka S, Drozdz R, Wójcik M: [Non-structural abnormalities of CNS function resulting in coincidence of endocrinopathies, epilepsy and psychoneurologic disorders in children and adolescents]. Przegl Lek; 2010;67(11):1127-31
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • BACKGROUND: In the population of children and adolescents, epilepsy affects approximately 1% of cases, nonepileptic seizures are seen in approximately 3%, and endocrine disorders are several times more common.
  • 2) Presentation of diagnostic and therapeutic difficulties in these patients, and 3) An attempt at defining the common etiology of both disorders.
  • RESULTS: Various forms of epilepsy (symptomatic or idiopathic) and other psychoneurological disorders (disorders of behavior and emotions, obsession-compulsion syndromes, stereotypias, aggression, autoaggression, or hypothalamic obesity) coincident with one or more endocrine disorders, such as growth disorders, disorders of pubertal development, obesity, thyroid diseases, adrenal diseases, hyperprolactinemia, hypoparathyroidism and ion metabolism disorders were diagnosed in 49 patients.
  • The group included: i) children after cranial irradiation and chemotherapy due to medulloblastoma (3 patients), oligodenroglioma (1 patient), ependymoma (1 patient), optic chiasm glioma (2 patients), suprasellar germinal tumor (1 patient), ii) children with Hashimoto encephalopathy (2 patients), iii) children with Prader-Willi syndrome (20 patients), with Klinefelter syndrome (10 patients), with Albright syndrome (9 patients).
  • In those patients, the etiology of both endocrine disorders, epilepsy and neuropsychiatric disorders was suspected to be common, and the diagnosis was usually delayed.
  • Cranial irradiation and chemotherapy, encephalopathy associated with Hashimoto disease and some of the syndromes with the chromosomal and genetic background are the causes of non-structural CNS abnormalities and coincidence of endocrinopathies, epilepsy and psychoneurologic disorders.
  • All of the above-mentioned manifestations may be symptoms of structural CNS abnormalities and their early treatment determines the child's future.
  • 4. In the diagnosis of Hashimoto's encephalopathy, a decisive factor is exclusion of structural, infectious, traumatic and metabolic causes, intoxications, epilepsy and presence of neuropsychiatric symptoms in patients with high level of against TPO antibodies.
  • In cases of steroids resistance, a good therapeutic effect may be achieved by plasmapheresis, Rituximab therapy and progestagene inhibition of the menstrual cycle.






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