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1. Broniscer A, Gajjar A: Supratentorial high-grade astrocytoma and diffuse brainstem glioma: two challenges for the pediatric oncologist. Oncologist; 2004;9(2):197-206
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  • [Title] Supratentorial high-grade astrocytoma and diffuse brainstem glioma: two challenges for the pediatric oncologist.
  • These neoplasms predominantly involve the supratentorial hemispheres or the pons, in which case the tumors are usually called diffuse brainstem gliomas.
  • The diagnosis of supratentorial neoplasms is dependent on their histologic appearance.
  • Older children (>3 years) with supratentorial neoplasms undergo a multimodality treatment comprised of surgical resection, radiation therapy, and chemotherapy.
  • The addition of chemotherapy seems to improve the survival of a subset of these children, particularly those with glioblastoma multiforme.
  • The diagnosis of a diffuse brainstem glioma is based upon typical imaging, dispensing with the need for surgery in the majority of cases.
  • Radiation therapy is the mainstay of treatment for children with diffuse brainstem gliomas.
  • The role of chemotherapy for these children is not clear, and it is, in general, employed in the context of an investigational study.
  • Less than 10% of children with diffuse brainstem gliomas survive 2 years.
  • Because the outcome for patients with either type of tumor is poor when standard multimodality therapy is used, these children are ideal candidates for innovative treatment approaches.
  • [MeSH-major] Brain Neoplasms / genetics. Brain Neoplasms / therapy. Glioma / genetics. Glioma / therapy. Neoplasm Recurrence, Local
  • [MeSH-minor] Astrocytoma / physiopathology. Astrocytoma / therapy. Brain Stem Neoplasms / genetics. Brain Stem Neoplasms / physiopathology. Brain Stem Neoplasms / therapy. Child. Disease Progression. Humans. Prognosis. Treatment Outcome


2. Broniscer A, Laningham FH, Kocak M, Krasin MJ, Fouladi M, Merchant TE, Kun LE, Boyett JM, Gajjar A: Intratumoral hemorrhage among children with newly diagnosed, diffuse brainstem glioma. Cancer; 2006 Mar 15;106(6):1364-71
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  • [Title] Intratumoral hemorrhage among children with newly diagnosed, diffuse brainstem glioma.
  • BACKGROUND: Children with diffuse brainstem glioma (BSG) commonly undergo novel therapies because their outcome is poor with radiation therapy (RT).
  • METHODS: All available brain imaging studies and medical records of 48 consecutive patients with newly diagnosed BSG treated at the study institution over a 10-year interval (1992-2002) were reviewed.
  • Treatment was comprised of RT and various regimens of conventional chemotherapy; none of these patients received biologic agents.
  • At the time of last follow-up, all patients had died of tumor progression.
  • RESULTS: The authors reviewed 319 imaging studies (251 magnetic resonance imaging scans and 68 computed tomography scans).
  • IH was present in 6.25% of patients at the time of diagnosis.
  • All cases of IH at the time of diagnosis and 78% of symptomatic cases that developed after diagnosis were located in necrotic areas.
  • CONCLUSIONS: Although IH is uncommon at the time of diagnosis, symptomatic IH may occur among nearly 20% of children after the diagnosis of BSG.
  • [MeSH-major] Brain Stem Neoplasms / diagnosis. Cerebral Hemorrhage / diagnosis. Glioma / diagnosis
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Agents / therapeutic use. Child. Child, Preschool. Combined Modality Therapy. Cranial Irradiation. Female. Humans. Magnetic Resonance Imaging. Male. Necrosis. Prognosis. Retrospective Studies. Survival Rate. Treatment Outcome

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  • [Copyright] (c) 2006 American Cancer Society.
  • (PMID = 16463390.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P30 CA 21765
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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3. Broniscer A, Laningham FH, Sanders RP, Kun LE, Ellison DW, Gajjar A: Young age may predict a better outcome for children with diffuse pontine glioma. Cancer; 2008 Aug 01;113(3):566-72
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  • [Title] Young age may predict a better outcome for children with diffuse pontine glioma.
  • BACKGROUND: Because diffuse pontine glioma (DPG) is rare among young children, the outcome of affected patients is unknown.
  • Inclusion followed standard magnetic resonance imaging criteria for the diagnosis of DPG.
  • RESULTS: The median age at diagnosis in 10 patients was 2.2 years (range, 0.8-2.7 years).
  • The median interval between the onset of symptoms and diagnosis was 2.5 months.
  • Histologic confirmation was attempted in 2 patients who had atypical radiologic features at diagnosis.
  • All patients received therapy, which consisted of radiation therapy (RT) (n = 2), RT and chemotherapy (n = 6), or chemotherapy only (n = 2).
  • CONCLUSIONS: Children aged <3 years with DPG potentially may fare better than older patients with the same diagnosis despite the use of similar therapy.
  • [MeSH-major] Brain Stem Neoplasms / diagnosis. Glioma / diagnosis
  • [MeSH-minor] Age Factors. Child, Preschool. Disease-Free Survival. Early Diagnosis. Female. Humans. Infant. Male. Prognosis. Retrospective Studies. Treatment Outcome

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  • [Copyright] (c) 2008 American Cancer Society
  • (PMID = 18484645.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P30 CA021765; United States / NCI NIH HHS / CA / P30 CA21765
  • [Publication-type] Evaluation Studies; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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4. Packer RJ, Krailo M, Mehta M, Warren K, Allen J, Jakacki R, Villablanca JG, Chiba A, Reaman G: Phase 1 study of concurrent RMP-7 and carboplatin with radiotherapy for children with newly diagnosed brainstem gliomas. Cancer; 2005 Sep 15;104(6):1281-7
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  • BACKGROUND: Ninety percent of children with diffuse intrinsic brainstem tumors will die within 18 months of diagnosis.
  • RMP-7 is a bradykinin analog that causes selective permeability of the blood-brain-tumor interface.
  • Local radiotherapy (5940 centigrays) was given within 4 hours of completion of drug delivery.
  • Duration of treatment was escalated in a stepwise, weekly fashion, in cohorts of 3, until there was treatment-limiting toxicity or until radiotherapy was completed.
  • RESULTS: One child died early in treatment of progressive disease and was not assessable for toxicity.
  • Treatment for 3, 4, or 5 weeks was tolerated well, with mild flushing, tachycardia, nausea, emesis, dizziness, and abdominal pain.
  • Of 3 children treated at the full duration of therapy (33 doses over 7 wks), 1 developed dose-limiting hepatotoxicity and neutropenia.
  • The estimated median survival period was 328 days, and 1 patient remained disease progression free > 400 days from initiation of treatment.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Stem Neoplasms / therapy. Carboplatin / administration & dosage. Glioma / therapy
  • [MeSH-minor] Adolescent. Bradykinin / administration & dosage. Bradykinin / adverse effects. Bradykinin / analogs & derivatives. Child. Child, Preschool. Combined Modality Therapy. Humans

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  • [Copyright] Copyright 2005 American Cancer Society.
  • (PMID = 16078267.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase I; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 159768-75-9 / RMP 7; BG3F62OND5 / Carboplatin; S8TIM42R2W / Bradykinin
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5. Korones DN: Treatment of newly diagnosed diffuse brain stem gliomas in children: in search of the holy grail. Expert Rev Anticancer Ther; 2007 May;7(5):663-74
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  • [Title] Treatment of newly diagnosed diffuse brain stem gliomas in children: in search of the holy grail.
  • Diffuse brain stem glioma is the most devastating of pediatric malignancies.
  • Virtually all children with this disease die within 1-2 years of diagnosis.
  • Attempts to improve survival using radiation, chemotherapy and biologic agents have yet to culminate in meaningful advances.
  • Recent advances in molecular biology have led to the development of more targeted therapies, which are now being introduced in clinical trials for children with brain stem glioma.
  • Real strides in improving the lives of children with brain stem glioma may finally be within our grasp.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Astrocytoma / radiotherapy. Brain Stem Neoplasms / radiotherapy
  • [MeSH-minor] Chemotherapy, Adjuvant. Child. Humans. Neoadjuvant Therapy. Radiotherapy / methods. Stem Cell Transplantation

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  • (PMID = 17492930.001).
  • [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
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 73
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6. Hashizume R, Ozawa T, Dinca EB, Banerjee A, Prados MD, James CD, Gupta N: A human brainstem glioma xenograft model enabled for bioluminescence imaging. J Neurooncol; 2010 Jan;96(2):151-9
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  • [Title] A human brainstem glioma xenograft model enabled for bioluminescence imaging.
  • Despite the use of radiation and chemotherapy, the prognosis for children with diffuse brainstem gliomas is extremely poor.
  • There is a need for relevant brainstem tumor models that can be used to test new therapeutic agents and delivery systems in pre-clinical studies.
  • We report the development of a brainstem-tumor model in rats and the application of bioluminescence imaging (BLI) for monitoring tumor growth and response to therapy as part of this model.
  • To evaluate if this model would allow detection of therapeutic response, rats bearing brainstem U-87 MG or GS2 glioblastoma xenografts were treated with the DNA methylating agent temozolomide (TMZ).
  • TMZ treatment groups were included for experiments involving U-87 MG and GS2 cells, and in each case TMZ delayed tumor growth, as indicated by BLI monitoring, and significantly extended survival of animal subjects.
  • Our results demonstrate the development of a brainstem tumor model in athymic rats, in which tumor growth and response to therapy can be accurately monitored by BLI.
  • This model is well suited for pre-clinical testing of therapeutics that are being considered for treatment of patients with brainstem tumors.
  • [MeSH-major] Brain Stem Neoplasms / diagnosis. Glioma / diagnosis. Luciferases. Luminescent Agents
  • [MeSH-minor] Animals. Antineoplastic Agents, Alkylating / therapeutic use. Cell Line, Tumor. Dacarbazine / analogs & derivatives. Dacarbazine / therapeutic use. Diagnostic Imaging. Disease Models, Animal. Humans. In Situ Nick-End Labeling / methods. Kaplan-Meier Estimate. Male. Neoplasm Transplantation / methods. Rats. Rats, Nude. Time Factors. Xenograft Model Antitumor Assays / methods

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  • (PMID = 19585223.001).
  • [ISSN] 1573-7373
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Grant] United States / NINDS NIH HHS / NS / K08 NS055061; United States / NCI NIH HHS / CA / R01 CA107268
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 0 / Luminescent Agents; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide; EC 1.13.12.- / Luciferases
  • [Other-IDs] NLM/ PMC2808534
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7. Broniscer A, Iacono L, Chintagumpala M, Fouladi M, Wallace D, Bowers DC, Stewart C, Krasin MJ, Gajjar A: Role of temozolomide after radiotherapy for newly diagnosed diffuse brainstem glioma in children: results of a multiinstitutional study (SJHG-98). Cancer; 2005 Jan 1;103(1):133-9
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  • [Title] Role of temozolomide after radiotherapy for newly diagnosed diffuse brainstem glioma in children: results of a multiinstitutional study (SJHG-98).
  • BACKGROUND: The role of chemotherapy in the treatment of children with newly diagnosed diffuse brainstem glioma is uncertain.
  • In the current study, the authors tested the efficacy of temozolomide treatment after radiotherapy (RT) in this setting.
  • An optional window therapy regimen consisting of 2 cycles of intravenous irinotecan (10 doses of 20 mg/m2 per day separated by 2 days of rest per cycle) was delivered over 6 weeks and was followed by conventionally fractionated RT.
  • RESULTS: Thirty-three patients (median age at diagnosis, 6.4 years) were enrolled.
  • Of the 16 patients who received window therapy, 6 had irinotecan treatment discontinued due to clinical progression (n=5) or toxicity (n=1); the remaining 10 experienced disease stabilization after 2 cycles.
  • CONCLUSIONS: The administration of temozolomide after RT did not alter the poor prognosis associated with newly diagnosed diffuse brainstem glioma in children.
  • [MeSH-major] Antineoplastic Agents, Alkylating / therapeutic use. Brain Stem Neoplasms / drug therapy. Camptothecin / analogs & derivatives. Dacarbazine / analogs & derivatives. Dacarbazine / therapeutic use. Glioma / drug therapy
  • [MeSH-minor] Administration, Oral. Adolescent. Child. Child, Preschool. Female. Humans. Infusions, Intravenous. Male. Treatment Outcome

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  • (PMID = 15565574.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P30 CA21765
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 7673326042 / irinotecan; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide; XT3Z54Z28A / Camptothecin
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8. Saurez G, Cabanas R, Zaldívar M, Garnier T, Iglesias B, Piedra P, Castillo MR, Longchong M, Iznaga N, Lage A: Clinical experience with nimotuzumab in cuban pediatric patients with brain tumors, 2005 to 2007. MEDICC Rev; 2009 Jul;11(3):27-33
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  • [Title] Clinical experience with nimotuzumab in cuban pediatric patients with brain tumors, 2005 to 2007.
  • Introduction Nimotuzumab, developed in Cuba, is a humanized monoclonal antibody that targets the epidermal growth factor receptor (EGFR).
  • It has been evaluated in malignant brain tumors in adults and children, and shown to be therapeutically safe and effective in terms of increased survival and improved quality of life.
  • Objective Describe nimotuzumab's safety profile and clinical benefits in terms of disease control and survival in pediatric patients with progressive or recurrent primary brain tumors who were included in an expanded access program.
  • Between December 2005 and December 2007, 22 patients were included, all of whom had an histological and/or radiological diagnosis of progressive or recurrent primary brain tumor, classified as high-grade malignant glioblastoma (n=6), diffuse brain stem glioma (n=6), ependymoblastoma (n=5), low-grade glioma (n=4), or thalamic tumor (n=1); life expectancy of at least 4 weeks; and a Karnofsky or Lansky Performance Status score of ≥50.
  • Therapeutic protocols were followed for administration as monotherapy or in combination with chemotherapy and/or radiotherapy.
  • Results Nimotuzumab was well tolerated in all therapeutic modalities, even with prolonged exposure.

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  • (PMID = 21483304.001).
  • [ISSN] 1555-7960
  • [Journal-full-title] MEDICC review
  • [ISO-abbreviation] MEDICC Rev
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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9. Hall WA, Doolittle ND, Daman M, Bruns PK, Muldoon L, Fortin D, Neuwelt EA: Osmotic blood-brain barrier disruption chemotherapy for diffuse pontine gliomas. J Neurooncol; 2006 May;77(3):279-84
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Osmotic blood-brain barrier disruption chemotherapy for diffuse pontine gliomas.
  • The prognosis for patients with diffuse pontine gliomas (DPG) remains poor.
  • New aggressive innovative treatments are necessary to treat this disease.
  • From 1984 to 1998, eight patients (4M/4F), median age 11 years, with DPG were treated with monthly osmotic blood-brain barrier disruption (BBBD) chemotherapy using intraarterial carboplatin or methotrexate and intravenous cytoxan and etoposide.
  • Three patients had radiation therapy before BBBD chemotherapy and four afterwards.
  • Two patients had chemotherapy (tamoxifen, topotecan) before BBBD chemotherapy and two afterwards.
  • In general, patients were evaluated with MR imaging every 3 months to monitor for a response to treatment.
  • The median number of chemotherapy cycles that were administered by BBBD was 10, mean 10.
  • Three patients also received one, two, or three cycles of intraarterial chemotherapy without BBBD.
  • The median time to tumor progression was 15 months with the range from <1 to 40 months.
  • The median survival from the time of diagnosis was 27 months, ranging from 7 to 80 months.
  • The median survival time from the first BBBD or intraarterial treatment was 16.5 months, ranging from 5 to 69 months.
  • Although the sample size is small, the TTP and survival times are longer than those previously reported in other DPG series.
  • In addition, the ability to demonstrate stable disease or partial responses in DPG on MR imaging argues for the therapeutic benefit of BBBD chemotherapy.
  • The enhanced delivery of chemotherapy afforded by osmotic BBBD supports the further examination of this treatment modality for patients with DPG.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / pharmacokinetics. Blood-Brain Barrier / metabolism. Brain Stem Neoplasms / drug therapy. Drug Delivery Systems / methods. Glioma / drug therapy
  • [MeSH-minor] Adolescent. Adult. Carboplatin / administration & dosage. Child. Child, Preschool. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Disease-Free Survival. Etoposide / administration & dosage. Female. Humans. Male. Methotrexate / administration & dosage. Osmosis / drug effects. Retrospective Studies. Treatment Outcome

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  • (PMID = 16314949.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Grant] United States / NINDS NIH HHS / NS / NS33618; United States / NINDS NIH HHS / NS / NS34608; United States / NINDS NIH HHS / NS / NS44687
  • [Publication-type] Journal Article; Multicenter Study; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; BG3F62OND5 / Carboplatin; YL5FZ2Y5U1 / Methotrexate
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10. Qaddoumi I, Ezam N, Swaidan M, Jaradat I, Mansour A, Abuirmeileh N, Bouffet E, Al-Hussaini M: Diffuse pontine glioma in Jordan and impact of up-front prognosis disclosure with parents and families. J Child Neurol; 2009 Apr;24(4):460-5
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  • [Title] Diffuse pontine glioma in Jordan and impact of up-front prognosis disclosure with parents and families.
  • For patients with diffuse pontine glioma, our institution offers local radiotherapy and supportive care only.
  • To investigate the effectiveness of this policy, we retrospectively reviewed records of patients with diffuse pontine glioma treated at the institution over a 49-month period.
  • The median age at diagnosis was 7 years.
  • We also found that diffuse pontine glioma warrants further study in developing countries.
  • [MeSH-major] Brain Stem Neoplasms / diagnosis. Brain Stem Neoplasms / psychology. Caregivers / psychology. Disclosure / statistics & numerical data. Glioma / diagnosis. Glioma / psychology. Pons / pathology
  • [MeSH-minor] Adolescent. Age Factors. Age of Onset. Arabs / ethnology. Arabs / psychology. Attitude to Death / ethnology. Cause of Death. Child. Child, Preschool. Disease Progression. Drug Therapy / statistics & numerical data. Family. Female. Humans. Jordan / ethnology. Magnetic Resonance Imaging. Male. Neoplasm Invasiveness. Palliative Care / psychology. Palliative Care / statistics & numerical data. Prognosis. Quality of Life. Radiotherapy / statistics & numerical data. Resuscitation Orders. Retrospective Studies. Survival Rate. Terminal Care. Treatment Outcome

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  • (PMID = 19074045.001).
  • [ISSN] 1708-8283
  • [Journal-full-title] Journal of child neurology
  • [ISO-abbreviation] J. Child Neurol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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11. Burzynski SR, Lewy RI, Weaver RA, Axler ML, Janicki TJ, Jurida GF, Paszkowiak JK, Szymkowski BG, Khan MI, Bestak M: Phase II study of antineoplaston A10 and AS2-1 in patients with recurrent diffuse intrinsic brain stem glioma: a preliminary report. Drugs R D; 2003;4(2):91-101
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  • [Title] Phase II study of antineoplaston A10 and AS2-1 in patients with recurrent diffuse intrinsic brain stem glioma: a preliminary report.
  • OBJECTIVE: A phase II study of antineoplaston A10 and AS2-1 was conducted to evaluate the antineoplastic activity in patients with recurrent diffuse intrinsic brain stem glioma.
  • PATIENTS AND METHODS: This report describes the results of treatment of the first 12 patients admitted to the study.
  • The median duration of treatment was 6 months and the average dosage of antineoplaston A10 was 11.3 g/kg/day and of antineoplaston AS2-1 0.4 g/kg/day.
  • Currently, of all 12 patients, two (17%) were alive and tumour free for over 5 years since initial diagnosis; one was alive for more than 5 years, and another for more than 4 years from the start of treatment.
  • CONCLUSION: The results of this study compared favourably with the responses of patients treated with radiation therapy and chemotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Benzeneacetamides / adverse effects. Benzeneacetamides / therapeutic use. Brain Stem Neoplasms / drug therapy. Glioma / drug therapy. Glutamine / adverse effects. Glutamine / analogs & derivatives. Glutamine / therapeutic use. Phenylacetates / adverse effects. Phenylacetates / therapeutic use. Piperidones / adverse effects. Piperidones / therapeutic use
  • [MeSH-minor] Adolescent. Adult. Cerebral Angiography. Child. Drug Combinations. Humans. Karnofsky Performance Status. Risk Assessment / methods. Time Factors

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  • (PMID = 12718563.001).
  • [ISSN] 1174-5886
  • [Journal-full-title] Drugs in R&D
  • [ISO-abbreviation] Drugs R D
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] New Zealand
  • [Chemical-registry-number] 0 / Benzeneacetamides; 0 / Drug Combinations; 0 / Phenylacetates; 0 / Piperidones; 0RH81L854J / Glutamine; 104624-98-8 / antineoplaston AS 2-1; 91531-30-5 / antineoplaston A10
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12. Gururangan S, McLaughlin CA, Brashears J, Watral MA, Provenzale J, Coleman RE, Halperin EC, Quinn J, Reardon D, Vredenburgh J, Friedman A, Friedman HS: Incidence and patterns of neuraxis metastases in children with diffuse pontine glioma. J Neurooncol; 2006 Apr;77(2):207-12
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  • [Title] Incidence and patterns of neuraxis metastases in children with diffuse pontine glioma.
  • PURPOSE: We performed a retrospective study of patients with diffuse pontine glioma (DPG) who suffered neuraxis metastasis (NM) and characterized the incidence, clinical features, radiologic findings, and patterns of disease dissemination.
  • METHODS: Magnetic resonance imaging (MRI) of brain and spine was used to assess NM.
  • Some patients also underwent magnetic resonance spectroscopy (MRS) (6 patients) and fluorodeoxyglucose positron emission tomography (FDG-PET) scans (13 patients) to further evaluate areas of metastatic disease.
  • RESULTS: Between 1986 and 2003, 18 of 96 patients (17.3%) with DPG developed NM.
  • The median age at diagnosis was 8 years (range, 4-17).
  • All patients had adjuvant chemotherapy and/or focal radiotherapy at diagnosis.
  • The NM occurred at a median of 15 months from diagnosis of DPG (range, 3-96).
  • Three patterns of NM were seen on MRI of brain and spine in these patients; 8 (39%) had parenchymal (PM), 4 (22%) leptomeningeal (PM), 2 (11%) subependymal, and in 5 a combination of two or more patterns.
  • Three patients also had histologic confirmation of malignant glioma at the site of NM.
  • Despite salvage therapy, all 18 patients have died of disease at a median of 5 months (range, 0.5-20) from diagnosis of neuraxis spread.
  • CONCLUSION: Our study emphasizes the need for screening patients with DPG for NM at the time of recurrence.
  • [MeSH-major] Brain Neoplasms / secondary. Brain Stem Neoplasms / pathology. Glioma / secondary. Spinal Cord Neoplasms / secondary
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Combined Modality Therapy. Female. Fluorodeoxyglucose F18. Humans. Incidence. Magnetic Resonance Imaging. Male. Positron-Emission Tomography. Retrospective Studies. Survival Analysis

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  • (PMID = 16568209.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article
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  • [Chemical-registry-number] 0Z5B2CJX4D / Fluorodeoxyglucose F18
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13. Yamada K, Miura M, Miyayama H, Sakashita N, Kochi M, Ushio Y: Diffuse brainstem glioma in a patient with Laurence-Moon-(Bardet-)Biedl syndrome. Pediatr Neurosurg; 2000 Dec;33(6):323-7
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  • [Title] Diffuse brainstem glioma in a patient with Laurence-Moon-(Bardet-)Biedl syndrome.
  • An autopsy case of a patient with diffuse brainstem glioma associated with Laurence-Moon-(Bardet-)Biedl syndrome is described.
  • She developed dizziness, headache and consequent consciousness disturbance.
  • By means of combined chemotherapy and radiation, she survived for 15 months.
  • Histopathological diagnosis for postmortem specimens obtained from the brainstem was glioblastoma multiforme.
  • [MeSH-major] Brain Stem Neoplasms / complications. Glioblastoma / complications. Laurence-Moon Syndrome / complications
  • [MeSH-minor] Adolescent. Adult. Autopsy. Brain / pathology. Fatal Outcome. Female. Humans. Magnetic Resonance Imaging

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  • [Copyright] Copyright 2001 S. Karger AG, Basel
  • (PMID = 11182644.001).
  • [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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14. Parbel S, Vlaho S, Gebhardt B, Porto L, Hattingen E, Klingebiel T, Böhles H, Kieslich M: [Diagnostic difficulties in encephalitis and glioma]. Klin Padiatr; 2007 Jul-Aug;219(4):222-4
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  • [Title] [Diagnostic difficulties in encephalitis and glioma].
  • The differential diagnosis between cerebral glioma and infective lesions can be very difficult to distinguish by MRI only.
  • MRI showed a diffuse mesencephalic and pontine swelling without contrast medium enhancement possibly pointing to an infective lesion.
  • After initial improvement associated with antibiotic, antiviral and dexamethasone treatment the process relapsed progessively.
  • Brain biopsy revealed anaplastic astrocytoma (WHO III).
  • Despite of radiation and chemotherapy the tumordisease deteriorated and the patient died because of progressive brainstem infiltration one year later.
  • CONCLUSION: This case report shows that cerebral glioma can mimick infective brain disease and that MR-spectroscopy is an important non-invasive tool in this differential diagnosis.
  • [MeSH-major] Astrocytoma / diagnosis. Brain Stem Neoplasms / diagnosis. Cerebellar Neoplasms / diagnosis. Encephalitis / diagnosis. Magnetic Resonance Spectroscopy. Pons
  • [MeSH-minor] Aspartic Acid / analogs & derivatives. Aspartic Acid / analysis. Child. Choline / analysis. Creatine / analysis. Diagnosis, Differential. Humans. Magnetic Resonance Imaging. Male

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  • (PMID = 16865652.001).
  • [ISSN] 0300-8630
  • [Journal-full-title] Klinische Pädiatrie
  • [ISO-abbreviation] Klin Padiatr
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 30KYC7MIAI / Aspartic Acid; 997-55-7 / N-acetylaspartate; MU72812GK0 / Creatine; N91BDP6H0X / Choline
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15. Chiang KL, Chang KP, Lee YY, Huang PI, Hsu TR, Chen YW, Chang FC, Wong TT: Role of temozolomide in the treatment of newly diagnosed diffuse brainstem glioma in children: experience at a single institution. Childs Nerv Syst; 2010 Aug;26(8):1035-41
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  • [Title] Role of temozolomide in the treatment of newly diagnosed diffuse brainstem glioma in children: experience at a single institution.
  • PURPOSE: The purpose of this study was to assess the efficacy of TMZ on diffuse brainstem glioma, either concomitant with radiotherapy or as an adjuvant treatment after radiotherapy in children.
  • METHODS AND MATERIALS: Eighteen children (median age at diagnosis was 8.3 years) meet the following criteria:.
  • (1) newly diagnosed diffuse brainstem glioma;.
  • They were divided into two groups according to treatment modalities: a radiotherapy alone followed by adjuvant TMZ (RT+TMZ) group received conventional radiation after initial diagnosis, and a concomitant chemoradiotherapy followed by adjuvant TMZ (CCRT+TMZ) group received concurrent chemotherapy during radiation with TMZ (75 mg/M(2)/day).
  • In addition, TMZ either as adjuvant therapy or as CCRT did not improve the prognosis of the patients with newly diagnosed diffuse brainstem glioma.
  • [MeSH-major] Antineoplastic Agents, Alkylating / therapeutic use. Brain Stem Neoplasms / drug therapy. Dacarbazine / analogs & derivatives. Glioma / drug therapy
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Adjuvant. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Humans. Kaplan-Meier Estimate. Male. Radiotherapy. Retrospective Studies. Treatment Outcome

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  • (PMID = 20217098.001).
  • [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] 0 / Antineoplastic Agents, Alkylating; 7GR28W0FJI / Dacarbazine; YF1K15M17Y / temozolomide
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16. Leblond P, Vinchon M, Bernier-Chastagner V, Chastagner P: [Diffuse intrinsic brain stem glioma in children: current treatment and future directions]. Arch Pediatr; 2010 Feb;17(2):159-65
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  • [Title] [Diffuse intrinsic brain stem glioma in children: current treatment and future directions].
  • [Transliterated title] Gliomes infiltrants du tronc cérébral chez l'enfant : traitement actuel et perspectives.
  • Despite the numerous clinical trials undertaken, the prognosis of children with diffuse brain stem glioma remains very poor.
  • This review examines the different strategies for the treatment of malignant brain stem glioma such as radiation therapy, concurrent radiochemotherapy, and classical cytotoxic drugs, with a particular focus on the novel targeted and antiangiogenic drugs recently introduced in pediatric oncology.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Brain Stem Neoplasms / drug therapy. Brain Stem Neoplasms / radiotherapy. Glioma / drug therapy. Glioma / radiotherapy
  • [MeSH-minor] Angiogenesis Inhibitors / therapeutic use. Child. Combined Modality Therapy. Cranial Irradiation. Humans. Prognosis. Radiotherapy, Adjuvant

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  • [Copyright] Copyright 2009 Elsevier Masson SAS. All rights reserved.
  • (PMID = 20018494.001).
  • [ISSN] 1769-664X
  • [Journal-full-title] Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie
  • [ISO-abbreviation] Arch Pediatr
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Antineoplastic Agents
  • [Number-of-references] 54
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17. Benesch M, Lackner H, Moser A, Kerbl R, Schwinger W, Oberbauer R, Eder HG, Mayer R, Wiegele K, Urban C: Outcome and long-term side effects after synchronous radiochemotherapy for childhood brain stem gliomas. Pediatr Neurosurg; 2001 Oct;35(4):173-80
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  • [Title] Outcome and long-term side effects after synchronous radiochemotherapy for childhood brain stem gliomas.
  • Between 1993 and 1999, 11 children with histologically confirmed diffuse and exophytic brain stem glioma (BSG) were treated with intensive induction chemotherapy and simultaneous external beam irradiation.
  • Chemotherapy was performed according to the German/Austrian Pediatric Brain Tumor Study HIT '91 and included two cycles of ifosfamide (days 1-3), etoposide (days 4-6), methotrexate (days 15 and 22), cisplatin (days 29-31) and cytarabine (days 29-31), separated by a 3-week interval.
  • Maintenance chemotherapy with carmustine, carboplatin and vincristine (8 cycles over a 1-year period) was given in those patients who responded clinically or radiographically to induction chemotherapy.
  • Six of 11 patients showed an objective reduction in tumor size on magnetic resonance imaging and 4 of 11 are alive in good general condition >22, >22, >90 and >92 months, respectively, after diagnosis without radiographic evidence of tumor progression (1 complete remission, 2 partial remissions, 1 stable disease), but suffer from moderate to severe long-term side effects.
  • Three patients died due to disease progression after having achieved a partial remission which lasted 5, 6 and 18 months, respectively, whereas only short-term stabilization was observed in 4 patients who died within 1 year after diagnosis.
  • This intensive combined modality treatment was toxic but yielded objective responses in more than 50% and long-term survivors in one third of childhood BSG patients.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / adverse effects. Brain / drug effects. Brain / radiation effects. Brain Stem Neoplasms / drug therapy. Brain Stem Neoplasms / radiotherapy. Cranial Irradiation / adverse effects. Glioma / drug therapy. Glioma / radiotherapy. Radiation Injuries / etiology
  • [MeSH-minor] Child. Child, Preschool. Combined Modality Therapy. Disease Progression. Female. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Survival Rate

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  • [Copyright] Copyright 2001 S. Karger AG, Basel
  • (PMID = 11694794.001).
  • [ISSN] 1016-2291
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
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18. Bouffet E, Raquin M, Doz F, Gentet JC, Rodary C, Demeocq F, Chastagner P, Lutz P, Hartmann O, Kalifa C: Radiotherapy followed by high dose busulfan and thiotepa: a prospective assessment of high dose chemotherapy in children with diffuse pontine gliomas. Cancer; 2000 Feb 1;88(3):685-92
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  • [Title] Radiotherapy followed by high dose busulfan and thiotepa: a prospective assessment of high dose chemotherapy in children with diffuse pontine gliomas.
  • BACKGROUND: The role of high dose chemotherapy (HDC) in patients with pediatric brain tumors currently is ill-defined.
  • The purpose of this pilot study was to assess the feasibility and the benefit of HDC after radiotherapy in a group of children with newly diagnosed diffuse pontine gliomas.
  • METHODS: Patients eligible for study were ages 3-18 years with diffuse intrinsic tumors arising in the pons, who were not treated previously with radiotherapy or chemotherapy.
  • Survival was the endpoint, and the statistical procedure was based on sequential subgroup analysis.
  • RESULTS: Thirty-six patients were entered on to the study, 12 of whom underwent stereotactic biopsy or open surgery at the time of diagnosis.
  • The median survival time was 10 months for the study group.
  • The median survival time in the subgroup of patients who received HDC was 10 months (range, 3-26 months).
  • CONCLUSIONS: For patients with diffuse pontine gliomas, survival using this aggressive treatment modality does not appear to be any better than that reported for conventional radiotherapy.
  • [MeSH-major] Antineoplastic Agents, Alkylating / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Stem Neoplasms / radiotherapy. Busulfan / administration & dosage. Glioma / radiotherapy. Thiotepa / administration & dosage
  • [MeSH-minor] Adolescent. Biopsy. Bone Marrow Transplantation. Cause of Death. Chemotherapy, Adjuvant. Child. Child, Preschool. Combined Modality Therapy. Disease Progression. Feasibility Studies. Female. Humans. Male. Pilot Projects. Prospective Studies. Stereotaxic Techniques. Survival Rate. Transplantation, Autologous. Treatment Outcome

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  • [Copyright] Copyright 2000 American Cancer Society.
  • (PMID = 10649264.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 905Z5W3GKH / Thiotepa; G1LN9045DK / Busulfan
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19. Frazier JL, Lee J, Thomale UW, Noggle JC, Cohen KJ, Jallo GI: Treatment of diffuse intrinsic brainstem gliomas: failed approaches and future strategies. J Neurosurg Pediatr; 2009 Apr;3(4):259-69
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment of diffuse intrinsic brainstem gliomas: failed approaches and future strategies.
  • Diffuse intrinsic pontine gliomas constitute ~ 60-75% of tumors found within the pediatric brainstem.
  • Magnetic resonance imaging is usually sufficient to establish the diagnosis and obviates the need for surgical biopsy in most cases.
  • Standard therapy involves radiotherapy, which produces transient neurological improvement with a progression-free survival benefit, but provides no improvement in overall survival.
  • Clinical trials have been conducted to assess the efficacy of chemotherapeutic and biological agents in the treatment of diffuse pontine gliomas.
  • In this review, the authors discuss recent studies in which systemic therapy was administered prior to, concomitantly with, or after radiotherapy.
  • For future perspective, the discussion includes a rationale for stereotactic biopsies as well as possible therapeutic options of local chemotherapy in these lesions.
  • [MeSH-major] Brain Stem Neoplasms / drug therapy. Brain Stem Neoplasms / radiotherapy. Glioma / drug therapy. Glioma / radiotherapy
  • [MeSH-minor] Animals. Antineoplastic Agents / administration & dosage. Chemotherapy, Adjuvant. Child. Drug Delivery Systems. Humans. Radiotherapy, Adjuvant. Rats. Treatment Failure

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  • [CommentIn] J Neurosurg Pediatr. 2010 Jan;5(1):140-1; author reply 141-2 [20043750.001]
  • (PMID = 19338403.001).
  • [ISSN] 1933-0707
  • [Journal-full-title] Journal of neurosurgery. Pediatrics
  • [ISO-abbreviation] J Neurosurg Pediatr
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 130
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20. Sharp JR, Bouffet E, Stempak D, Gammon J, Stephens D, Johnston DL, Eisenstat D, Hukin J, Samson Y, Bartels U, Tabori U, Huang A, Baruchel S: A multi-centre Canadian pilot study of metronomic temozolomide combined with radiotherapy for newly diagnosed paediatric brainstem glioma. Eur J Cancer; 2010 Dec;46(18):3271-9
Hazardous Substances Data Bank. DACARBAZINE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A multi-centre Canadian pilot study of metronomic temozolomide combined with radiotherapy for newly diagnosed paediatric brainstem glioma.
  • PURPOSE: Survival rates for paediatric diffuse intrinsic brainstem glioma (DIBSG) are dismal.
  • Metronomic dosing of temozolomide (TMZ) combined with standard radiotherapy may improve survival by increasing the therapeutic index and anti-angiogenic effect of TMZ.
  • Treatment was continued until tumour progression or unacceptable toxicity occurred.
  • For patients who consented, plasma and urine samples were collected at diagnosis, post-induction and prior to each course of maintenance therapy for the quantification of angiogenesis markers.
  • Median time to progression was 5.13 months (95% CI = 6.4, 10.8) and median overall survival (OS) was 9.8 months (95% CI = 6.4, 10.8).
  • Serum levels of both VEGF and endoglin tended to decrease during the first two cycles of therapy.
  • [MeSH-major] Antineoplastic Agents, Alkylating / administration & dosage. Brain Stem Neoplasms / drug therapy. Brain Stem Neoplasms / radiotherapy. Dacarbazine / analogs & derivatives. Glioma / drug therapy. Glioma / radiotherapy
  • [MeSH-minor] Adolescent. Canada. Child. Child, Preschool. Combined Modality Therapy / methods. Female. Humans. Infant. Male. Maximum Tolerated Dose. Pilot Projects. Thrombocytopenia / etiology. Treatment Outcome

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  • [Copyright] Copyright © 2010 Elsevier Ltd. All rights reserved.
  • (PMID = 20656474.001).
  • [ISSN] 1879-0852
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide
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21. Wolff JE, Mohiuddin K, Jorch N, Graf N, Wagner S, Vats T, Gnekow A: Measuring performance status in pediatric patients with brain tumors--experience of the HIT-GBM-C protocol. Pediatr Blood Cancer; 2010 Sep;55(3):520-4
MedlinePlus Health Information. consumer health - Childhood Brain Tumors.

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  • [Title] Measuring performance status in pediatric patients with brain tumors--experience of the HIT-GBM-C protocol.
  • Here, we report in a treatment protocol for pediatric patients with high-grade glioma and diffuse intrinsic pontine glioma.
  • PROCEDURE: The Fertigkeitenskala Münster-Heidelberg (FMH) is a 56-item quantitative measure of health status.
  • At the beginning of the treatment, only 5 patients scored over 40 FMH%, and 4 of these survived.
  • The physician's judgment was documented at diagnosis and during treatment (n = 50).
  • Per physician, 22% of the patients were normal before chemotherapy, decreasing to 16% in the middle of the protocol.
  • At diagnosis only 16% of patients had severely reduced activity, which increased to 30.6% in the middle of the protocol.
  • Patients with poor performance at diagnosis had a poorer prognosis.
  • [MeSH-major] Activities of Daily Living. Brain Neoplasms / physiopathology. Glioma / physiopathology. Health Status. Quality of Life. Surveys and Questionnaires
  • [MeSH-minor] Adolescent. Brain Stem Neoplasms / physiopathology. Child. Child, Preschool. Female. Humans. Male

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  • [Copyright] 2010 Wiley-Liss, Inc.
  • (PMID = 20658624.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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22. Massimino M, Simonetti F, Balestrini MR, Spreafico F, La Spina M, Terenziani M, Gandola L: Transitory, spontaneously recovering, peripheral facial nerve palsy after vinorelbine administration. Neurol Sci; 2006 Jun;27(2):110-3
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  • Childhood intrinsic brain-stem gliomas have a dismal prognosis.
  • Different treatment strategies have been adopted over the years without changing the final outcome of this ominous disease.
  • Due to this grim prognosis, experimental therapeutic designs are worthwhile.
  • By adopting vinorelbine during and after focal radiotherapy in the last two years, we have tried to evocate its known synergistic effect in brain-stem tumour control.
  • All the consecutive patients whose clinical and radiological features corresponded to the diagnosis of an intrinsic brain-stem tumour, i.e., diffuse pontine glioma, have been accrued to this treatment protocol since July 2002.
  • Two of the thirteen patients so far treated have developed multiple subsequent, and transitory, episodes of monolateral peripheral facial nerve palsy during vinorelbine administration.
  • The palsy always completely and spontaneously resolved at a short interval-around 30 min-after the end of the drug infusion.
  • Obvious tumour progression was excluded by means of MRI; therefore the drug was administered as scheduled until the end of the treatment.
  • We describe possible neurological and oncological implications of this unusual side effect, until now not reported in any other series dealing with vinorelbine as adjuvant treatment.
  • [MeSH-major] Bell Palsy / chemically induced. Brain Stem Neoplasms / drug therapy. Glioma / drug therapy. Radiation-Sensitizing Agents / adverse effects. Vinblastine / analogs & derivatives
  • [MeSH-minor] Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Male. Remission, Spontaneous

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  • (PMID = 16816907.001).
  • [ISSN] 1590-1874
  • [Journal-full-title] Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
  • [ISO-abbreviation] Neurol. Sci.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Radiation-Sensitizing Agents; 5V9KLZ54CY / Vinblastine; Q6C979R91Y / vinorelbine
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23. Laigle-Donadey F, Doz F, Delattre JY: Brainstem gliomas in children and adults. Curr Opin Oncol; 2008 Nov;20(6):662-7
MedlinePlus Health Information. consumer health - Childhood Brain Tumors.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • PURPOSE OF REVIEW: The purpose of this review is to determine if recent advances in diagnostic and treatment modalities result in improvement in the pattern of care of brainstem gliomas.
  • RECENT FINDINGS: New MRI techniques may contribute to differential diagnosis and aid neurosurgeons in removing resectable brainstem tumors.
  • However, biopsy remains indicated in many contrast enhancing brainstem masses in adults because of the great variety of differential diagnosis.
  • SUMMARY: Diffuse brainstem glioma is the most common subtype of brainstem tumor and remains a devastating malignancy in children.
  • Conventional radiotherapy is the standard of care and chemotherapy has been disappointing to date.
  • Given the lack of efficacy of conventional drugs, a better understanding of the biology of this tumor is the key to more targeted therapy.
  • [MeSH-major] Brain Neoplasms / drug therapy. Brain Stem / pathology. Glioma / drug therapy
  • [MeSH-minor] Adult. Angiogenesis Inhibitors / therapeutic use. Antineoplastic Agents / therapeutic use. Biopsy. Child. Drug Delivery Systems. Humans. Magnetic Resonance Imaging / methods. Medical Oncology / methods. Neoplasm Metastasis. Neurofibromatosis 1 / drug therapy. Neurofibromatosis 1 / pathology. Signal Transduction

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  • (PMID = 18841048.001).
  • [ISSN] 1531-703X
  • [Journal-full-title] Current opinion in oncology
  • [ISO-abbreviation] Curr Opin Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Antineoplastic Agents
  • [Number-of-references] 57
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24. Guillamo JS, Monjour A, Taillandier L, Devaux B, Varlet P, Haie-Meder C, Defer GL, Maison P, Mazeron JJ, Cornu P, Delattre JY, Association des Neuro-Oncologues d'Expression Française (ANOCEF): Brainstem gliomas in adults: prognostic factors and classification. Brain; 2001 Dec;124(Pt 12):2528-39

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • The charts of 48 adults suffering from brainstem glioma were reviewed in order to determine prognostic factors, evaluate the effect of treatment and propose a classification of these tumours.
  • Treatment consisted of partial resection (8%), radiotherapy (94%) and chemotherapy (56%).
  • On univariate analysis, the following favourable prognostic factors were identified (P< 0.01): age of onset <40 years, duration of symptoms before diagnosis >3 months, Karnofski performance status >70, low-grade histology, absence of contrast enhancement and 'necrosis' on MRI.
  • Eighty-five percent of the tumours could be classified into one of the following three groups on the basis of clinical, radiological and histological features. (i) Diffuse intrinsic low-grade gliomas (46%) usually occurred in young adults with a long clinical history before diagnosis and a diffusely enlarged brainstem on MRI that did not show contrast enhancement.
  • These patients were improved by radiotherapy in 62% of cases and had a long survival time (median 7.3 years).
  • These tumours were highly resistant to treatment and the patients had a median survival time of 11.2 months. (iii) Focal tectal gliomas (8%) occurred in young patients and were often revealed by isolated hydrocephalus.
  • The optimum timing of treatment for supratentorial low-grade tumours remains unclear.
  • In high-grade gliomas, the prognosis remains extremely poor despite aggressive treatment with radiotherapy and chemotherapy.
  • [MeSH-major] Brain Stem Neoplasms / classification. Brain Stem Neoplasms / mortality. Glioma / classification. Glioma / mortality
  • [MeSH-minor] Adult. Age Factors. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Stem / pathology. Disease Progression. Female. Humans. Hydrocephalus / etiology. Hydrocephalus / pathology. Magnetic Resonance Imaging. Male. Multivariate Analysis. Prognosis. Radiotherapy. Survival Rate. Tectum Mesencephali / pathology

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  • (PMID = 11701605.001).
  • [ISSN] 0006-8950
  • [Journal-full-title] Brain : a journal of neurology
  • [ISO-abbreviation] Brain
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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25. Sievert AJ, Fisher MJ: Pediatric low-grade gliomas. J Child Neurol; 2009 Nov;24(11):1397-408
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  • Cerebellar pilocytic astrocytomas occur most frequently followed by supratentorial diffuse fibrillary astrocytomas.
  • 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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26. Packer RJ, Krailo M, Mehta M, Warren K, Allen J, Jakacki R, Villablanca JG, Chiba A, Reaman G: A Phase I study of concurrent RMP-7 and carboplatin with radiation therapy for children with newly diagnosed brainstem gliomas. Cancer; 2005 Nov 1;104(9):1968-74
Hazardous Substances Data Bank. CARBOPLATIN .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A Phase I study of concurrent RMP-7 and carboplatin with radiation therapy for children with newly diagnosed brainstem gliomas.
  • BACKGROUND: Ninety percent of children with diffuse, intrinsic brainstem tumors will die within 18 months of diagnosis.
  • RMP-7 is a bradykinin analog that causes selective permeability of the blood-brain-tumor interface.
  • The objective of this Phase I study was to determine the toxicity and feasibility of delivering RMP-7 and carboplatin for 5 successive days during radiotherapy to children with newly diagnosed, diffuse, intrinsic brainstem gliomas.
  • Local radiotherapy, in dose fractions of 180 centigrays (cGy) per day (to a total dose of 5940 cGy), was given within 4 hours of completion of drug delivery.
  • Duration of treatment was escalated in a stepwise, weekly fashion in cohorts of 3 patients, until there was treatment-limiting toxicity or until radiotherapy was completed.
  • RESULTS: One child died early during treatment of progressive disease and was not assessable for toxicity.
  • Treatment for 3 weeks, 4 weeks, and 5 weeks was tolerated well, with mild flushing, tachycardia, nausea, emesis, dizziness, and abdominal pain.
  • One of 3 children treated at the full duration of therapy (33 doses over 7 weeks) developed dose-limiting hepatotoxicity and neutropenia.
  • The estimated median survival was 328 days, and 1 patient remained free of disease progression for > 400 days after the initiation of treatment.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bradykinin / analogs & derivatives. Brain Stem Neoplasms / drug therapy. Brain Stem Neoplasms / radiotherapy. Carboplatin / therapeutic use. Glioma / drug therapy. Glioma / radiotherapy
  • [MeSH-minor] Child. Child, Preschool. Combined Modality Therapy. Drug Administration Schedule. Feasibility Studies. Female. Humans. Male. Radiotherapy / adverse effects. Radiotherapy Dosage. Survival Rate. Treatment Outcome

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  • [Copyright] (c) 2005 American Cancer Society.
  • (PMID = 16177987.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase I; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 159768-75-9 / RMP 7; BG3F62OND5 / Carboplatin; S8TIM42R2W / Bradykinin
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