Search Results - (Author, Cooperation:E. Bouffet)

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  1. 1
    A. S. Morrissy ; L. Garzia ; D. J. Shih ; S. Zuyderduyn ; X. Huang ; P. Skowron ; M. Remke ; F. M. Cavalli ; V. Ramaswamy ; P. E. Lindsay ; S. Jelveh ; L. K. Donovan ; X. Wang ; B. Luu ; K. Zayne ; Y. Li ; C. Mayoh ; N. Thiessen ; E. Mercier ; K. L. Mungall ; Y. Ma ; K. Tse ; T. Zeng ; K. Shumansky ; A. J. Roth ; S. Shah ; H. Farooq ; N. Kijima ; B. L. Holgado ; J. J. Lee ; S. Matan-Lithwick ; J. Liu ; S. C. Mack ; A. Manno ; K. A. Michealraj ; C. Nor ; J. Peacock ; L. Qin ; J. Reimand ; A. Rolider ; Y. Y. Thompson ; X. Wu ; T. Pugh ; A. Ally ; M. Bilenky ; Y. S. Butterfield ; R. Carlsen ; Y. Cheng ; E. Chuah ; R. D. Corbett ; N. Dhalla ; A. He ; D. Lee ; H. I. Li ; W. Long ; M. Mayo ; P. Plettner ; J. Q. Qian ; J. E. Schein ; A. Tam ; T. Wong ; I. Birol ; Y. Zhao ; C. C. Faria ; J. Pimentel ; S. Nunes ; T. Shalaby ; M. Grotzer ; I. F. Pollack ; R. L. Hamilton ; X. N. Li ; A. E. Bendel ; D. W. Fults ; A. W. Walter ; T. Kumabe ; T. Tominaga ; V. P. Collins ; Y. J. Cho ; C. Hoffman ; D. Lyden ; J. H. Wisoff ; J. H. Garvin, Jr. ; D. S. Stearns ; L. Massimi ; U. Schuller ; J. Sterba ; K. Zitterbart ; S. Puget ; O. Ayrault ; S. E. Dunn ; D. P. Tirapelli ; C. G. Carlotti ; H. Wheeler ; A. R. Hallahan ; W. Ingram ; T. J. MacDonald ; J. J. Olson ; E. G. Van Meir ; J. Y. Lee ; K. C. Wang ; S. K. Kim ; B. K. Cho ; T. Pietsch ; G. Fleischhack ; S. Tippelt ; Y. S. Ra ; S. Bailey ; J. C. Lindsey ; S. C. Clifford ; C. G. Eberhart ; M. K. Cooper ; R. J. Packer ; M. Massimino ; M. L. Garre ; U. Bartels ; U. Tabori ; C. E. Hawkins ; P. Dirks ; E. Bouffet ; J. T. Rutka ; R. J. Wechsler-Reya ; W. A. Weiss ; L. S. Collier ; A. J. Dupuy ; A. Korshunov ; D. T. Jones ; M. Kool ; P. A. Northcott ; S. M. Pfister ; D. A. Largaespada ; A. J. Mungall ; R. A. Moore ; N. Jabado ; G. D. Bader ; S. J. Jones ; D. Malkin ; M. A. Marra ; M. D. Taylor
    Nature Publishing Group (NPG)
    Published 2016
    Staff View
    Publication Date:
    2016-01-14
    Publisher:
    Nature Publishing Group (NPG)
    Print ISSN:
    0028-0836
    Electronic ISSN:
    1476-4687
    Topics:
    Biology
    Chemistry and Pharmacology
    Medicine
    Natural Sciences in General
    Physics
    Published by:
    http://www.nature.com/

    Latest Papers from Table of Contents or Articles in Press
  2. 2
    Staff View
    Publication Date:
    2012-06-23
    Publisher:
    Nature Publishing Group (NPG)
    Print ISSN:
    0028-0836
    Electronic ISSN:
    1476-4687
    Topics:
    Biology
    Chemistry and Pharmacology
    Medicine
    Natural Sciences in General
    Physics
    Keywords:
    Animals ; CREB-Binding Protein/genetics ; Cadherins/genetics ; Cdh1 Proteins ; Cell Cycle Proteins/deficiency/genetics ; Cell Lineage ; Cerebellar Neoplasms/*classification/*genetics/pathology ; Child ; DEAD-box RNA Helicases/genetics ; DNA Copy Number Variations ; DNA Helicases/genetics ; DNA Mutational Analysis ; Disease Models, Animal ; Genome, Human/genetics ; Genomics ; Hedgehog Proteins/metabolism ; Histone Demethylases/genetics ; Histones/metabolism ; Humans ; Medulloblastoma/*classification/*genetics/pathology ; Methylation ; Mice ; Mutation/*genetics ; Nuclear Proteins/genetics ; Phosphatidylinositol 3-Kinases/genetics ; Transcription Factors/genetics ; Wnt Proteins/metabolism ; beta Catenin/genetics
    Published by:
    http://www.nature.com/

    Latest Papers from Table of Contents or Articles in Press
  3. 3
    Staff View
    Publication Date:
    2012-02-22
    Publisher:
    Nature Publishing Group (NPG)
    Print ISSN:
    0028-0836
    Electronic ISSN:
    1476-4687
    Topics:
    Biology
    Chemistry and Pharmacology
    Medicine
    Natural Sciences in General
    Physics
    Keywords:
    Animals ; Clonal Evolution/*genetics ; CpG Islands/genetics ; DNA Methylation ; DNA Transposable Elements/genetics ; Disease Models, Animal ; Genes, p53/genetics ; Germ-Line Mutation/genetics ; Humans ; Li-Fraumeni Syndrome/complications/genetics ; Medulloblastoma/complications/*genetics/*pathology ; Mice ; Mutagenesis, Insertional ; Neoplasm Metastasis/*genetics/*pathology ; Survival Rate
    Published by:
    http://www.nature.com/

    Latest Papers from Table of Contents or Articles in Press
  4. 4
    P. A. Northcott ; D. J. Shih ; J. Peacock ; L. Garzia ; A. S. Morrissy ; T. Zichner ; A. M. Stutz ; A. Korshunov ; J. Reimand ; S. E. Schumacher ; R. Beroukhim ; D. W. Ellison ; C. R. Marshall ; A. C. Lionel ; S. Mack ; A. Dubuc ; Y. Yao ; V. Ramaswamy ; B. Luu ; A. Rolider ; F. M. Cavalli ; X. Wang ; M. Remke ; X. Wu ; R. Y. Chiu ; A. Chu ; E. Chuah ; R. D. Corbett ; G. R. Hoad ; S. D. Jackman ; Y. Li ; A. Lo ; K. L. Mungall ; K. M. Nip ; J. Q. Qian ; A. G. Raymond ; N. T. Thiessen ; R. J. Varhol ; I. Birol ; R. A. Moore ; A. J. Mungall ; R. Holt ; D. Kawauchi ; M. F. Roussel ; M. Kool ; D. T. Jones ; H. Witt ; L. A. Fernandez ; A. M. Kenney ; R. J. Wechsler-Reya ; P. Dirks ; T. Aviv ; W. A. Grajkowska ; M. Perek-Polnik ; C. C. Haberler ; O. Delattre ; S. S. Reynaud ; F. F. Doz ; S. S. Pernet-Fattet ; B. K. Cho ; S. K. Kim ; K. C. Wang ; W. Scheurlen ; C. G. Eberhart ; M. Fevre-Montange ; A. Jouvet ; I. F. Pollack ; X. Fan ; K. M. Muraszko ; G. Y. Gillespie ; C. Di Rocco ; L. Massimi ; E. M. Michiels ; N. K. Kloosterhof ; P. J. French ; J. M. Kros ; J. M. Olson ; R. G. Ellenbogen ; K. Zitterbart ; L. Kren ; R. C. Thompson ; M. K. Cooper ; B. Lach ; R. E. McLendon ; D. D. Bigner ; A. Fontebasso ; S. Albrecht ; N. Jabado ; J. C. Lindsey ; S. Bailey ; N. Gupta ; W. A. Weiss ; L. Bognar ; A. Klekner ; T. E. Van Meter ; T. Kumabe ; T. Tominaga ; S. K. Elbabaa ; J. R. Leonard ; J. B. Rubin ; L. M. Liau ; E. G. Van Meir ; M. Fouladi ; H. Nakamura ; G. Cinalli ; M. Garami ; P. Hauser ; A. G. Saad ; A. Iolascon ; S. Jung ; C. G. Carlotti ; R. Vibhakar ; Y. S. Ra ; S. Robinson ; M. Zollo ; C. C. Faria ; J. A. Chan ; M. L. Levy ; P. H. Sorensen ; M. Meyerson ; S. L. Pomeroy ; Y. J. Cho ; G. D. Bader ; U. Tabori ; C. E. Hawkins ; E. Bouffet ; S. W. Scherer ; J. T. Rutka ; D. Malkin ; S. C. Clifford ; S. J. Jones ; J. O. Korbel ; S. M. Pfister ; M. A. Marra ; M. D. Taylor
    Nature Publishing Group (NPG)
    Published 2012
    Staff View
    Publication Date:
    2012-07-27
    Publisher:
    Nature Publishing Group (NPG)
    Print ISSN:
    0028-0836
    Electronic ISSN:
    1476-4687
    Topics:
    Biology
    Chemistry and Pharmacology
    Medicine
    Natural Sciences in General
    Physics
    Keywords:
    Carrier Proteins/genetics ; Cerebellar Neoplasms/*classification/*genetics/metabolism ; Child ; DNA Copy Number Variations/genetics ; Gene Duplication/genetics ; Genes, myc/genetics ; Genome, Human/*genetics ; Genomic Structural Variation/*genetics ; Genomics ; Hedgehog Proteins/metabolism ; Humans ; Medulloblastoma/*classification/*genetics/metabolism ; NF-kappa B/metabolism ; Nerve Tissue Proteins/genetics ; Oncogene Proteins, Fusion/genetics ; Proteins/genetics ; RNA, Long Noncoding ; Signal Transduction ; Transforming Growth Factor beta/metabolism ; Translocation, Genetic/genetics
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    http://www.nature.com/

    Latest Papers from Table of Contents or Articles in Press
  5. 5
    S. C. Mack ; H. Witt ; R. M. Piro ; L. Gu ; S. Zuyderduyn ; A. M. Stutz ; X. Wang ; M. Gallo ; L. Garzia ; K. Zayne ; X. Zhang ; V. Ramaswamy ; N. Jager ; D. T. Jones ; M. Sill ; T. J. Pugh ; M. Ryzhova ; K. M. Wani ; D. J. Shih ; R. Head ; M. Remke ; S. D. Bailey ; T. Zichner ; C. C. Faria ; M. Barszczyk ; S. Stark ; H. Seker-Cin ; S. Hutter ; P. Johann ; S. Bender ; V. Hovestadt ; T. Tzaridis ; A. M. Dubuc ; P. A. Northcott ; J. Peacock ; K. C. Bertrand ; S. Agnihotri ; F. M. Cavalli ; I. Clarke ; K. Nethery-Brokx ; C. L. Creasy ; S. K. Verma ; J. Koster ; X. Wu ; Y. Yao ; T. Milde ; P. Sin-Chan ; J. Zuccaro ; L. Lau ; S. Pereira ; P. Castelo-Branco ; M. Hirst ; M. A. Marra ; S. S. Roberts ; D. Fults ; L. Massimi ; Y. J. Cho ; T. Van Meter ; W. Grajkowska ; B. Lach ; A. E. Kulozik ; A. von Deimling ; O. Witt ; S. W. Scherer ; X. Fan ; K. M. Muraszko ; M. Kool ; S. L. Pomeroy ; N. Gupta ; J. Phillips ; A. Huang ; U. Tabori ; C. Hawkins ; D. Malkin ; P. N. Kongkham ; W. A. Weiss ; N. Jabado ; J. T. Rutka ; E. Bouffet ; J. O. Korbel ; M. Lupien ; K. D. Aldape ; G. D. Bader ; R. Eils ; P. Lichter ; P. B. Dirks ; S. M. Pfister ; A. Korshunov ; M. D. Taylor
    Nature Publishing Group (NPG)
    Published 2014
    Staff View
    Publication Date:
    2014-02-21
    Publisher:
    Nature Publishing Group (NPG)
    Print ISSN:
    0028-0836
    Electronic ISSN:
    1476-4687
    Topics:
    Biology
    Chemistry and Pharmacology
    Medicine
    Natural Sciences in General
    Physics
    Keywords:
    Animals ; Brain Neoplasms/drug therapy/genetics ; CpG Islands/*genetics ; DNA Methylation/drug effects ; Embryonic Stem Cells/metabolism ; Ependymoma/drug therapy/*genetics ; Epigenesis, Genetic/*genetics ; Epigenomics ; Female ; Gene Expression Regulation, Neoplastic ; Gene Silencing/drug effects ; Histones/drug effects/metabolism ; Humans ; Infant ; Mice ; Mice, Inbred NOD ; Mice, SCID ; Mutation/genetics ; Phenotype ; Polycomb Repressive Complex 2/metabolism ; Prognosis ; Rhombencephalon/pathology ; Xenograft Model Antitumor Assays
    Published by:
    http://www.nature.com/

    Latest Papers from Table of Contents or Articles in Press
  6. 6
    Combaret, V. ; Favrot, M. C. ; Chauvin, F. ; Bouffet, E. ; Philip, I. ; Philip, T.

    Oxford, UK : Blackwell Publishing Ltd
    Published 1989
    Staff View
    ISSN:
    1744-313X
    Source:
    Blackwell Publishing Journal Backfiles 1879-2005
    Topics:
    Biology
    Medicine
    Notes:
    Using experimental models with normal allogeneic bone marrow (BM) contaminated with Burkitt or neuroblastoma cell lines, and a liquid culture assay, we demonstrated that, when used in optimal conditions, the immunomagnetic depletion technique permitted a reproducible elimination of 3–4 log malignant cells. Results were very similar to those obtained with the complement lysis procedure in Burkitt lymphoma. This immunomagnetic procedure was used in 123 cases of autologous bone marrow transplantation (ABMT) in children with neuroblastoma. The analysis of the cases demonstrated, first, that the procedure induced a significant loss of mononuclear cells but was not toxic for BM precursors. Delays to engraftment observed in a few patients were probably due to the combination of pejorative factors, especially the damage caused to the micro-environment by previous heavy and prolonged chemotherapy or the double ABMT programme. Second, patients presented with profound T-cell defect with undetectable IL2 secretion up to 1 year post-graft but they all had normal NK functions from the first month post-graft, these functions exceeding normal values on the second and third months post-graft. Finally, in 20 cases, dual-immunofluorescence staining permitted the demonstration that the autograft contained malignant cells before purging that were eliminated by the immunomagnetic depletion.
    Type of Medium:
    Electronic Resource
    URL:
    http://dx.doi.org/10.1111/j.1744-313X.1989.tb00454.x

    Articles: DFG German National Licenses
  7. 7
    Staff View
    ISSN:
    1432-0843
    Source:
    Springer Online Journal Archives 1860-2000
    Topics:
    Medicine
    Notes:
    Abstract. Nine children with soft tissue sarcomas, five of them rhabdomyosarcomas with initial metastatic disease, (one patient, partial response, one patient), refractory primary, (two patients, relapse, five patients) were treated with a combination of high-dose VP16 (100 mg/m2 daily for 5 days) and cisplatin (40 mg/m2 daily for 5 days). The response rate was five out of nine or 55% (±32%) (two complete and three partial, responses). Three of the five responders had rhabdomyosarcomas. The duration of response was 4 – 58 months (median 11 months). The toxicity was mainly hematological. Thus, the high-dose VP16 – cisplatin association warrants further evaluation in soft tissue sarcoma in children.
    Type of Medium:
    Electronic Resource
    URL:
    http://dx.doi.org/10.1007/BF00685912

    Articles: DFG German National Licenses
  8. 8
    Staff View
    ISSN:
    1432-0843
    Source:
    Springer Online Journal Archives 1860-2000
    Topics:
    Medicine
    Notes:
    Abstract Nine children with soft tissue sarcomas, five of them rhabdomyosarcomas with initial metastatic disease, (one patient, partial response, one patient), refractory primary, (two patients, relapse, five patients) were treated with a combination of high-dose VP 16 (100 mg/m2 daily for 5 days) and cisplatin (40 mg/m2 daily for 5 days). The response rate was five out of nine or 55% (±32%) (two complete and three partial, responses). Three of the five responders had rhabdomyosarcomas. The duration of response was 4–58 months (median 11 months). The toxicity was mainly hematological. Thus, the high-dose VP 16-cisplatin association warrants further evaluation in soft tissue sarcoma in children.
    Type of Medium:
    Electronic Resource
    URL:
    http://dx.doi.org/10.1007/BF00685912

    Articles: DFG German National Licenses
  9. 9
    Staff View
    ISSN:
    1432-1084
    Keywords:
    Key words: Rhabdoid tumor ; Kidney ; Brain tumor ; Percutaneous biopsy ; Child
    Source:
    Springer Online Journal Archives 1860-2000
    Topics:
    Medicine
    Notes:
    Abstract. We report a case of a malignant rhabdoid tumor of the kidney (MRTK) associated with a cerebellar tumor. Diagnosis was confirmed before neoadjuvant chemotherapy by a percutaneous fine-needle biopsy of the abdominal tumor. The clinical and radiologic features of this rare association of childhood neoplasms are reviewed.
    Type of Medium:
    Electronic Resource
    URL:
    http://dx.doi.org/10.1007/s003300050374

    Articles: DFG German National Licenses
  10. 10
    Staff View
    ISSN:
    1433-0350
    Keywords:
    Key words Spinal cord ; Astrocytoma ; Chemotherapy
    Source:
    Springer Online Journal Archives 1860-2000
    Topics:
    Medicine
    Notes:
    Abstract Standard treatment of spinal cord astrocytomas is based upon surgery, followed by radiotherapy when resection is incomplete or when histology is of high grade. Owing to the major consequences of radiotherapy on the spine in childhood, alternative therapies must be explored. The potential role of chemotherapy in the management of spinal cord astrocytoma remains to be defined. Two patients are described. The first was a 19-month-old child with an anaplastic astrocytoma of the cervical spinal cord that progressed rapidly after initial partial resection. Chemotherapy was begun according to the UKCCSG Baby Brain Protocol, with marked clinical improvement. Reassessment by MRI at 4 months showed improvement, and at the end of treatment no evaluable disease remained. The second was a 4-year-old child with a recurrent low-grade astrocytoma. Chemotherapy according to the SIOP Protocol for Low Grade Gliomas was administered for 3 months, after which marked tumour regression was seen, with neurological recovery. These patients demonstrate the potential value and low morbidity of chemotherapy in spinal cord astrocytoma. The management of this rare tumour is discussed.
    Type of Medium:
    Electronic Resource
    URL:
    http://dx.doi.org/10.1007/s003810050233

    Articles: DFG German National Licenses
  11. 11
    Bouffet, E. ; Foreman, N.
    Springer
    Published 1999
    Staff View
    ISSN:
    1433-0350
    Keywords:
    Key words Ependymoma ; Children ; Chemotherapy ; Phase II
    Source:
    Springer Online Journal Archives 1860-2000
    Topics:
    Medicine
    Notes:
    Abstract  Chemotherapy has been used extensively in the management of children with intracranial ependymoma. In this review, we discuss the results of phase II studies and clinical trials conducted in newly diagnosed and recurrent ependymomas. There is little evidence that chemotherapy is effective in this tumour. The response rate to single agents is 11%, with less than 5% complete responses, cisplatin being the most active agent in phase II studies. Combinations may be more effective, although the response rate with high-dose regimens is disappointing. Early results of protocols conducted in infants and young children do not suggest that chemotherapy is beneficial. A more rigorous assessment of chemotherapy is required in order to define its role in patients with intracranial ependymomas. Indeed, it is difficult to justify the use of chemotherapy outside such studies. More large studies, perhaps intergroup, limited to children with ependymomas would be of particular value.
    Type of Medium:
    Electronic Resource
    URL:
    http://dx.doi.org/10.1007/s003810050544

    Articles: DFG German National Licenses
  12. 12
    Staff View
    ISSN:
    1433-0350
    Keywords:
    Children ; Oligodendroglioma ; Radiation therapy ; Chemotherapy ; Outcome
    Source:
    Springer Online Journal Archives 1860-2000
    Topics:
    Medicine
    Notes:
    Abstract The cases of 15 children operated on for cerebral pure oligodendroglioma were studied. Two groups of children were distinguished. Group 1 children presented with epilepsy (7 cases); their tumor was histologically benign in all cases. These children are all alive after a median follow-up of 72 months; however, 2 of them presented with a local recurrence, which was operated on, and are actually disease-free. Group 2 children presented with intracranial hypertension (8 cases); the tumor was anaplastic in 7 cases. Despite postoperative radiotherapy and chemotherapy 6 children died; their median survival time was 17 months. The present series shows the existence of a clear correlation between clinical presentation, histological grading and survival in childhood cerebral oligodendrogliomas.
    Type of Medium:
    Electronic Resource
    URL:
    http://dx.doi.org/10.1007/BF00261605

    Articles: DFG German National Licenses
  13. 13
    Staff View
    ISSN:
    1433-0350
    Keywords:
    Key words Spinal cord compression ; Metastases ; Child ; Solid tumour
    Source:
    Springer Online Journal Archives 1860-2000
    Topics:
    Medicine
    Notes:
    Abstract Over a 9-year period, 35 out of 614 children with malignant tumours who were treated at the Centre Léon Bérard developed spinal metastases. Of these, 18 with known malignancies before the development of spinal cord compression are reviewed. The most common tumours causing spinal metastases were Ewing's sarcoma, neuroblastoma and renal tumours. Cord compression occurred 5–88 months after the diagnosis of systemic cancer. The median interval from first symptoms to the diagnosis of compression was 17 days. There were 16 patients with neurological deficit, including 5 with paraplegia. Specific imaging procedures were performed in 16 patients. Treatment included operation in 8 patients, followed by chemotherapy (6 patients) and/or radiotherapy (4 patients); 9 of the 10 non-operated patients received radiotherapy. Only 6 patients had a significant neurological improvement. All patients but 1 died within a median time of 2 months. Early diagnosis might prevent permanent disability in these children with a short survival expectancy.
    Type of Medium:
    Electronic Resource
    URL:
    http://dx.doi.org/10.1007/s003810050105

    Articles: DFG German National Licenses
  14. 14
    Staff View
    ISSN:
    1573-7373
    Keywords:
    brain neoplasm ; germ cell tumors ; children ; chemotherapy ; radiotherapy ; markers
    Source:
    Springer Online Journal Archives 1860-2000
    Topics:
    Medicine
    Notes:
    Abstract Purpose: Intracranial αFP and βHCG secreting germ cell tumors have a very poor prognosis with local treatment alone. Because of efficacy of chemotherapy in extracranial forms, we tried in the SFOP (Société Française d'Oncologie Pédiatrique) to treat them with chemotherapeutic treatment exclusively. Patients and methods: Eighteen patients (10 αFP, 2 βHCG, 6 αFP and βHCG secretion) were enrolled from January 1988 to December 1992. After biological diagnosis patients were to receive 6 cycles of chemotherapy (vinblastine – bleomycin – carboplatin or etoposide – carboplatin/ifosfamide – etoposide). After completion of chemotherapy, surgery was to be performed in case of residual tumor. Focal radiation was only to be delivered in case of viable residual tumor. Results: All patients had biological remission and tumor reduction. Fifteen patients were treated according to the protocol by chemotherapy alone (13) or chemotherapy and radiation of residue [2]. Twelve of the 13 non irradiated patients relapsed, 8 in local and/or regional area, 3 in cerebrospinal area and 1 in undeterminated area with mild elevation of markers except in one case. Six patients are alive in second complete remission after chemotherapy and/or surgery and then a consolidation treatment with radiation and/or high dose chemotherapy (5) or craniospinal radiation (1). Three patients received radiation after 2 or 3 cycles of chemotherapy as protocol violations and didn't relapse. Thus, 12 patients out of the 18 patients are alive with a median follow up of 68 months. All but 1 had focal radiation as part of treatment. No toxic death was observed. Conclusion: Although survival rate is noteworthy (66%), these tumors were not curable with this conventional chemotherapy alone and focal radiotherapy should be part of the treatment.
    Type of Medium:
    Electronic Resource
    URL:
    http://dx.doi.org/10.1023/A:1005863601481

    Articles: DFG German National Licenses
  15. 15
    Staff View
    ISSN:
    1573-7373
    Keywords:
    children ; medulloblastoma ; BMT ; Busulfan ; Thiotepa
    Source:
    Springer Online Journal Archives 1860-2000
    Topics:
    Medicine
    Notes:
    Abstract Cranio-spinal irradiation is the gold standard treatment used in non metastatic medulloblastoma as prophylaxis against central nervous system (CNS) metastases. However, given the severe late effects caused by this procedure in children under 3 years of age, most pediatric oncologists are currently treating these patients with conventional chemotherapy in order to postpone or even avoid irradiation. In the French Society of Pediatric Oncology ('FOP) this attitude has been adopted since 1987. Among the patients treated without radiotherapy, 20 relapsed while on conventional chemotherapy and were entered in a study of high-dose chemotherapy (HDC) followed by ABMT. Their median age at diagnosis was 23 months (R5-71) and the relapse occurred at a median time of 6.3 months after the initiation of chemotherapy. Complete surgical removal of the local relapse was the first treatment in 4/20 patients who were not evaluable for response. Sixteen of the twenty patients had measurable disease at the primary site (9 patients), or at metastatic sites (3 patients) or both (4 patients). The conditioning regimen consisted of combination Busulfan 600 mg/m2 over 4 days and Thiotepa 900 mg/m2 over three days. After recovery from aplasia, patients with a local relapse received local radiotherapy limited to posterior fossa. Results. Among the 16 patients with measurable disease, 6 CR, 6 PR, 3 NR, were observed following HDC (response rate 75 %). One patient was not evaluable. For the 20 patients, the EFS is 50%. Among the surviving patients, the median follow up is 31 months post BMT (R12-82). Ten patients who developped a local relapse or local progression are alive with NED without craniospinal irradiation. Among the 7 patients who developped metastases or progression of metastases, only one is alive. Toxicity was high but manageable: the median duration of granulocytopenia 〈 0.5 × 109/1 and thrombocytopenia 〈 50 × 109/1 was 13 and 41 days respectively. Bacteremia was documented in 4 cases. Grade 〉 2 mucositis and diarrhea were observed in 60% of patients. One complication-related death occurred 1 month post BMT. Conclusion. With a 75 % response rate, this HDC proved to be very efficient in relapsed medulloblastoma. A longer follow up is necessary to demonstrate whether, after a local relapse, HDC could replace craniospinal irradiation as prophylaxis against CNS metastases.
    Type of Medium:
    Electronic Resource
    URL:
    http://dx.doi.org/10.1007/BF00146088

    Articles: DFG German National Licenses
  16. 16
    Staff View
    ISSN:
    1573-7373
    Keywords:
    brain ; children ; Rendu-Osier disease ; chromosome 22 ; genetic ; neoplasm ; rhabdoid tumor
    Source:
    Springer Online Journal Archives 1860-2000
    Topics:
    Medicine
    Notes:
    Abstract The case of a boy with a familial history of Rendu-Osier disease, who successively developed a cerebellar pilocytic astrocytoma (at 3 years of age) and a metastatic supratentorial malignant rhabdoid tumor (at the age of 12 years) is reported. After a complete surgical removal, the CSF was cleared by 4 courses of chemotherapy, and the child received a craniospinal irradiation. He is currently alive and well 19 months after completion of the treatment. The authors discuss the ethiopathogeny of such intracranial tumors and argue for aggressive treatment.
    Type of Medium:
    Electronic Resource
    URL:
    http://dx.doi.org/10.1007/BF01054275

    Articles: DFG German National Licenses