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1. Rieken S, Gaiser T, Mohr A, Welzel T, Witt O, Kulozik AE, Wick W, Debus J, Combs SE: Outcome and prognostic factors of desmoplastic medulloblastoma treated within a multidisciplinary treatment concept. BMC Cancer; 2010;10:450
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Outcome and prognostic factors of desmoplastic medulloblastoma treated within a multidisciplinary treatment concept.
  • BACKGROUND: Desmoplasia in medulloblastoma is often diagnosed in adult patients and was repeatedly associated with improved results.
  • Today, all medulloblastoma patients receive intensive multimodal treatment including surgery, radiotherapy and chemotherapy.
  • This study was set up to investigate treatment outcome and prognostic factors after radiation therapy in patients with desmoplastic medulloblastomas.
  • METHODS: Twenty patients treated for desmoplastic medulloblastoma in the Department of Radiation Oncology at the University of Heidelberg between 1984 and 2007 were included.
  • All patients underwent postsurgical radiotherapy (RT).
  • The median dose to the whole brain and the craniospinal axis was 35.2 Gray (Gy), and 54.4 Gy to the posterior fossa.
  • Fourteen patients received chemotherapy, including seven who were treated with combined radiochemotherapy and twelve who received adjuvant chemotherapy.
  • Five patients died from recurrent medulloblastoma.
  • Treatment-associated toxicity was acceptable.
  • CONCLUSIONS: Multimodal approaches with surgical resection followed by chemoirradiation achieved high response rates with long OS in desmoplastic medulloblastoma patients.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cerebellar Neoplasms / therapy. Cranial Irradiation. Medulloblastoma / therapy. Neoplasm Recurrence, Local / therapy. Neurosurgical Procedures
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Male. Middle Aged. Neoplasm Staging. Radiotherapy Dosage. Retrospective Studies. Survival Rate. Treatment Outcome. Young Adult

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  • (PMID = 20731859.001).
  • [ISSN] 1471-2407
  • [Journal-full-title] BMC cancer
  • [ISO-abbreviation] BMC Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2939548
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2. Reis Filho JS, Gasparetto EL, Faoro LN, Araújo JC, Torres LF: [Medulloblastomas: clinical, epidemiological and pathological findings in 28 cases]. Arq Neuropsiquiatr; 2000 Mar;58(1):76-80

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Medulloblastomas: clinical, epidemiological and pathological findings in 28 cases].
  • We report the clinical, epidemiological and pathological findings of 28 patients with medulloblastoma: 22 were male; age ranged from 1 to 50 years, with a mean of 15 years.
  • Only one patient showed a desmoplastic medulloblastoma variant, the others showed classical medulloblastomas.
  • Regarding treatment, most patients were submitted to total resection (n=10) or partial tumorectomy (n=7).
  • Chemotherapy seemed to contribute to a lower recurrence rate amongst our patients.
  • Our findings are similar to those reported in literature, thus helping to understand the biological behavior of this type of tumor.
  • [MeSH-major] Cerebellar Neoplasms. Medulloblastoma

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  • (PMID = 10770870.001).
  • [ISSN] 0004-282X
  • [Journal-full-title] Arquivos de neuro-psiquiatria
  • [ISO-abbreviation] Arq Neuropsiquiatr
  • [Language] por
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] BRAZIL
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3. Rutkowski S, Bode U, Deinlein F, Ottensmeier H, Warmuth-Metz M, Soerensen N, Graf N, Emser A, Pietsch T, Wolff JE, Kortmann RD, Kuehl J: Treatment of early childhood medulloblastoma by postoperative chemotherapy alone. N Engl J Med; 2005 Mar 10;352(10):978-86
Hazardous Substances Data Bank. METHOTREXATE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment of early childhood medulloblastoma by postoperative chemotherapy alone.
  • BACKGROUND: The prognosis for young children with medulloblastoma is poor, and survivors are at high risk for cognitive deficits.
  • We conducted a trial of the treatment of this brain tumor by intensive postoperative chemotherapy alone.
  • METHODS: After surgery, children received three cycles of intravenous chemotherapy (cyclophosphamide, vincristine, methotrexate, carboplatin, and etoposide) and intraventricular methotrexate.
  • Treatment was terminated if a complete remission was achieved.
  • In children who had complete resection (17 patients), residual tumor (14), and macroscopic metastases (12), the five-year progression-free and overall survival rates (+/-SE) were 82+/-9 percent and 93+/-6 percent, 50+/-13 percent and 56+/-14 percent, and 33+/-14 percent and 38+/-15 percent, respectively.
  • Desmoplastic histology, metastatic disease, and an age younger than two years were independent prognostic factors for tumor relapse and survival.
  • Treatment strategies at relapse were successful in 8 of 16 patients.
  • After treatment, the mean IQ was significantly lower than that of healthy controls within the same age group but higher than that of patients in a previous trial who had received radiotherapy.
  • CONCLUSIONS: Postoperative chemotherapy alone is a promising treatment for medulloblastoma in young children without metastases.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cerebellar Neoplasms / drug therapy. Medulloblastoma / drug therapy
  • [MeSH-minor] Analysis of Variance. Carboplatin / administration & dosage. Chemotherapy, Adjuvant. Child, Preschool. Cyclophosphamide / administration & dosage. Etoposide / administration & dosage. Follow-Up Studies. Humans. Infant. Intelligence / drug effects. Methotrexate / administration & dosage. Neoplasm Recurrence, Local / drug therapy. Neuropsychological Tests. Postoperative Care. Proportional Hazards Models. Remission Induction. Survival Analysis. Vincristine / administration & dosage

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  • Hazardous Substances Data Bank. ETOPOSIDE .
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  • [Copyright] Copyright 2005 Massachusetts Medical Society.
  • [CommentIn] Nat Clin Pract Oncol. 2005 Aug;2(8):386-7 [16130930.001]
  • [CommentIn] N Engl J Med. 2005 Mar 10;352(10):1036-8 [15758016.001]
  • [CommentIn] N Engl J Med. 2005 Jun 2;352(22):2350-3; author reply 2350-3 [15930429.001]
  • [CommentIn] N Engl J Med. 2005 Jun 2;352(22):2350-3; author reply 2350-3 [15938011.001]
  • [CommentIn] N Engl J Med. 2005 Jun 2;352(22):2350-3; author reply 2350-3 [15938010.001]
  • (PMID = 15758008.001).
  • [ISSN] 1533-4406
  • [Journal-full-title] The New England journal of medicine
  • [ISO-abbreviation] N. Engl. J. Med.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; BG3F62OND5 / Carboplatin; YL5FZ2Y5U1 / Methotrexate
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4. Helton KJ, Fouladi M, Boop FA, Perry A, Dalton J, Kun L, Fuller C: Medullomyoblastoma: a radiographic and clinicopathologic analysis of six cases and review of the literature. Cancer; 2004 Sep 15;101(6):1445-54
The Weizmann Institute of Science GeneCards and MalaCards databases. gene/protein/disease-specific - MalaCards for medullomyoblastoma .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • BACKGROUND: Medullomyoblastoma (MMB) is a rare cerebellar embryonal neoplasm that occurs almost exclusively in children.
  • METHODS: The authors conducted a retrospective review of the radiographic and pathologic characteristics, treatment, and clinical outcomes of six children with MMB who were treated at St. Jude Children's Research Hospital (Memphis, TN) between 1984 and 2003.
  • Radiographically, all tumors were cerebellar and exhibited variable enhancement, and 50% of tumors had necrotic foci.
  • Three tumors contained discrete, magnetic resonance imaging (MRI) T2-weighted-hypointense/computed tomography (CT)-hyperdense enhancing regions and separate hyperintense/hypodense nonenhancing regions, which correlated microscopically with geographic islands of primitive neuroectodermal and rhabdomyoblastic cells.
  • Large cell/anaplastic (five tumors), nodular/desmoplastic (two tumors), and classic (two tumors) medulloblastoma histologies were encountered either alone (five tumors) or in combination with each other (two tumors).
  • All patients underwent macroscopic total resection and subsequently received chemotherapy and craniospinal (five patients) or local conformal (one patient) radiotherapy.
  • CONCLUSIONS: The results of the current study demonstrated the frequent correlation of biphasic nodularity (as determined by MRI or CT) with discrete rhabdomyoblastic and primitive neuroectodermal islands (as revealed by microscopy) in MMB.
  • These results also support the view that MMB and medulloblastoma may have common tumorigenic origins, given their similar histologic and molecular features.
  • [MeSH-major] Cerebellar Neoplasms / pathology. Cerebellar Neoplasms / radiography. Medulloblastoma / pathology. Medulloblastoma / radiography
  • [MeSH-minor] Child. Child, Preschool. Chromosomes, Human, Pair 17. Female. Genes, myc. Humans. In Situ Hybridization. Infant. Magnetic Resonance Imaging. Male. Retrospective Studies. Tomography, X-Ray Computed

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  • [Copyright] Copyright 2004 American Cancer Society.
  • (PMID = 15368333.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Number-of-references] 45
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5. Gulino A, Arcella A, Giangaspero F: Pathological and molecular heterogeneity of medulloblastoma. Curr Opin Oncol; 2008 Nov;20(6):668-75
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pathological and molecular heterogeneity of medulloblastoma.
  • PURPOSE OF REVIEW: The outcome of medulloblastoma patients and the quality of life of survivors can be improved by both novel therapies and a better tumor classification.
  • This review will focus on the pathology and molecular biology of medulloblastoma and its potential to influence risk assignment and future therapy.
  • RECENT FINDINGS: A risk stratification system of pediatric medulloblastoma based on a combination of histopathological evaluation and targeted molecular analysis can be outlined.
  • Among the four variants recognized by the 2007 WHO classification, the desmoplastic medulloblastoma and the medulloblastoma with extensive nodularity have significantly better survival with respect to classic medulloblastoma, whereas the large-cell and anaplastic have a worse prognosis.
  • Moreover, 17p loss, MYC amplification/expression, and 1q gain are associated with poor prognosis; in contrast, monosomy 6, mutation of CTNNB1, and trkC expression identify tumors with a favorable outcome.
  • Emerging evidence indicates that the different precursor cell populations that form the cerebellum are susceptible to mutations in signal pathways that regulate their functions; these mutations alter normal development programmes and may result in the formation of distinct variants of medulloblastoma.
  • SUMMARY: Better understanding of the growth control mechanisms involved in the development and progression of medulloblastoma will allow improved therapeutic stratification of patients using existing adjuvant therapy as well as the development of new therapeutic approaches.
  • [MeSH-major] Brain Neoplasms / drug therapy. Gene Expression Regulation, Neoplastic. Medulloblastoma / drug therapy
  • [MeSH-minor] Adolescent. Cerebellum / pathology. Child. Child, Preschool. Hedgehog Proteins / metabolism. Humans. Infant. Infant, Newborn. Quality of Life. Receptors, Notch / metabolism. Risk. Treatment Outcome. Wnt Proteins / metabolism

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  • (PMID = 18841049.001).
  • [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 / Hedgehog Proteins; 0 / Receptors, Notch; 0 / Wnt Proteins
  • [Number-of-references] 31
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6. Duplan SM, Théorêt Y, Kenigsberg RL: Antitumor activity of fibroblast growth factors (FGFs) for medulloblastoma may correlate with FGF receptor expression and tumor variant. Clin Cancer Res; 2002 Jan;8(1):246-57
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Antitumor activity of fibroblast growth factors (FGFs) for medulloblastoma may correlate with FGF receptor expression and tumor variant.
  • Members of the fibroblast growth factor (FGF) family, which normally control cerebellar neuronal maturation, may represent more natural and less toxic tools with which to target medulloblastoma (MB), an embryonal brain tumor thought to arise from cerebellar neuronal precursors.
  • In support of this, we found previously basic FGF/FGF-2 can inhibit MB progression by inducing neuronal-like differentiation, slowing the growth, and triggering apoptosis of a MB cell line we established from a histopathologically classic tumor (R. L.
  • This activity is only noted for cell lines that originate from classic (UM-MB1 and SYR) rather than desmoplastic (HSJ) tumors.
  • Whereas these FGFs inhibit proliferation of both classic cell lines, they only advance neuronal differentiation and induce apoptosis of one, UM-MB1.
  • Consistent with these responses, after FGF treatment, levels of neurofilaments and the proapoptotic modulator Bax only increase in UM-MB1, whereas the cyclin-dependent kinase inhibitor p27/Kip1 (p27), which accumulates in cerebellar neuronal precursors before they exit the cell cycle, goes up in both UM-MB1 and SYR.
  • Finally, although the histological variant of MB may help predict the sensitivity of MB to the FGFs, the selectivity, specificity, and type of response elicited may be dictated by, as evident by immunoprecipitation and Western blot analyses, the expression of certain FGF receptor types.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Brain Neoplasms / drug therapy. Fibroblast Growth Factor 2 / therapeutic use. Fibroblast Growth Factors / therapeutic use. Medulloblastoma / drug therapy. Receptors, Fibroblast Growth Factor / metabolism
  • [MeSH-minor] Animals. Blotting, Western. Cell Differentiation / physiology. Cell Survival. Child, Preschool. Fibroblast Growth Factor 9. Humans. Male. Neurites / drug effects. Precipitin Tests. Proto-Oncogene Proteins / metabolism. Proto-Oncogene Proteins c-bcl-2 / metabolism. Tumor Cells, Cultured / drug effects. Tumor Cells, Cultured / metabolism. bcl-2-Associated X Protein. bcl-X Protein


7. Rutkowski S, Gerber NU, von Hoff K, Gnekow A, Bode U, Graf N, Berthold F, Henze G, Wolff JE, Warmuth-Metz M, Soerensen N, Emser A, Ottensmeier H, Deinlein F, Schlegel PG, Kortmann RD, Pietsch T, Kuehl J, German Pediatric Brain Tumor Study Group: Treatment of early childhood medulloblastoma by postoperative chemotherapy and deferred radiotherapy. Neuro Oncol; 2009 Apr;11(2):201-10
MedlinePlus Health Information. consumer health - Cancer Chemotherapy.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment of early childhood medulloblastoma by postoperative chemotherapy and deferred radiotherapy.
  • To investigate the utility of postoperative chemotherapy in delaying radiotherapy and to identify prognostic factors in early childhood medulloblastoma, we studied children younger than 3 years of age registered to the HIT-SKK'87 (Therapieprotokoll für Säuglinge und Kleinkinder mit Hirntumoren [Brain Tumor Radiotherapy for Infants and Toddlers with Medulloblastoma] 1987) trial who received systemic interval chemotherapy until craniospinal radiotherapy was applied at 3 years of age or at relapse, from 1987 to 1993.
  • Children with postoperative residual tumor or metastatic disease received systemic induction chemotherapy prior to interval chemotherapy.
  • Survival was superior in nine children with desmoplastic or extensive nodular histology compared with 20 children with classic medulloblastoma (10-year PFS, 88.9% +/- 10.5% and 30.0% +/- 10.3%, p = 0.003; OS, 88.9% +/- 10.5% and 40.0% +/- 11.0%, p = 0.006).
  • Eleven of 12 children with tumor progression during chemotherapy had classic medulloblastoma.
  • After treatment, IQ scores were inferior compared with nonirradiated children from the subsequent study, HIT-SKK'92.
  • In terms of survival, craniospinal radiotherapy was successfully delayed especially in young children with medulloblastoma of desmoplastic/extensive nodular histology, which was a strong independent favorable prognostic factor.
  • Because of the neurocognitive deficits of survivors, the emerging concepts to avoid craniospinal radiotherapy should rely on the histological medulloblastoma subtype.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Cerebellar Neoplasms / drug therapy. Cerebellar Neoplasms / radiotherapy. Cranial Irradiation. Medulloblastoma / drug therapy. Medulloblastoma / radiotherapy. Neoplasm Recurrence, Local / therapy
  • [MeSH-minor] Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Humans. Infant. Male. Pilot Projects. Prognosis. Survival Rate. Treatment Outcome

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  • (PMID = 18818397.001).
  • [ISSN] 1522-8517
  • [Journal-full-title] Neuro-oncology
  • [ISO-abbreviation] Neuro-oncology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Other-IDs] NLM/ PMC2718992
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8. Lafay-Cousin L, Bouffet E, Hawkins C, Amid A, Huang A, Mabbott DJ: Impact of radiation avoidance on survival and neurocognitive outcome in infant medulloblastoma. Curr Oncol; 2009 Dec;16(6):21-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Impact of radiation avoidance on survival and neurocognitive outcome in infant medulloblastoma.
  • PURPOSE: Concerns about radiotherapy-related neurocognitive sequelae in young children have led to deferral or avoidance of radiation in contemporary treatment for this fragile group of patients.
  • We compared survival and neurocognitive outcome in two groups of infants with medulloblastoma who received adjuvant conventional craniospinal irradiation (CSI) or reduced or no radiotherapy during an era of change in the philosophy of infant medulloblastoma treatment.
  • Children treated before 1994 received adjuvant radiation with chemotherapy; subsequently, radiation was prescribed essentially for disease progression or relapse.
  • As part of initial treatment, 8 children received adjuvant radiotherapy with chemotherapy, and 21 children received postoperative chemotherapy only.
  • Five children treated with chemotherapy alone are in prolonged remission.
  • Extent of resection, metastatic status, and desmoplastic histology were not found to be significant prognostic factors.
  • On serial neurocognitive evaluations, patients treated with chemotherapy with or without reduced radiotherapy demonstrated improvement of intellectual function over time.
  • CONCLUSIONS: Avoidance of conventional CSI in treatment of very young children with medulloblastoma appears to be associated with a preserved neurocognitive profile.

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  • (PMID = 20016743.001).
  • [ISSN] 1718-7729
  • [Journal-full-title] Current oncology (Toronto, Ont.)
  • [ISO-abbreviation] Curr Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Canada
  • [Other-IDs] NLM/ PMC2794676
  • [Keywords] NOTNLM ; Infants / medulloblastoma / neurocognitive outcome
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9. Nishio S, Morioka T, Inamura T, Takeshita I, Ishihara S, Fukui M: [Medulloblastoma with neuronal differentiation: a report of five cases]. No To Shinkei; 2000 May;52(5):391-7
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  • [Title] [Medulloblastoma with neuronal differentiation: a report of five cases].
  • This report presents a retrospective analysis of 5 patients who were treated for cerebellar medulloblastoma with neuronal differentiation.
  • Four males and 1 female ranged in age from 6 months to 9 years at the time of diagnosis.
  • While these tumors were composed of small cells and had regions resembling desmoplastic medulloblastoma, they in part showed neuronal characteristics which included parallel row or linear array arrangements of tumor cells in an eosinophilic fibrillary matrix.
  • Postoperatively, 3 patients received craniospinal radiation therapy, one received local radiation to the primary site, and the remaining one received only systemic chemotherapy.
  • The clinical and anatomic pathological features of medulloblastomas with neuronal differentiation are reviewed while the therapeutic problems associated with these tumors are also discussed.
  • [MeSH-major] Cerebellar Neoplasms / pathology. Medulloblastoma / pathology

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  • (PMID = 10845206.001).
  • [ISSN] 0006-8969
  • [Journal-full-title] Nō to shinkei = Brain and nerve
  • [ISO-abbreviation] No To Shinkei
  • [Language] jpn
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] JAPAN
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10. Mazza E, Spreafico F, Cefalo G, Scaramuzza D, Massimino M: Case report: Pseudomonas aeruginosa-related intervertebral discitis in a young boy with medulloblastoma. J Neurooncol; 2004 Jul;68(3):245-8
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  • [Title] Case report: Pseudomonas aeruginosa-related intervertebral discitis in a young boy with medulloblastoma.
  • We report a case of a 15-year-old boy with desmoplastic medulloblastoma of the posterior fossa (T3M3, according to Chang classification) incompletely resected, with leptomeningeal and nodular spread in the posterior fossa and in the cervical and thoracic tracts of the spine, treated with sequential high dose iv chemotherapy and with hyperfractionated cranio-spinal radiotherapy.
  • While on maintenance chemotherapy, the boy developed fever and septic status caused by Pseudomonas aeruginosa, and 1 week later also low back pain.
  • Magnetic resonance imaging (MRI) demonstrated abnormal signal in the fourth ventricle and in the dorso-lumbar tract suggesting medulloblastoma recurrence, so he started with a chemotherapy program.
  • So patient was treated with intensive antibiotic therapy with resolution of the infection.
  • [MeSH-major] Cerebellar Neoplasms / complications. Discitis / etiology. Lumbar Vertebrae. Medulloblastoma / complications. Pseudomonas Infections / etiology. Thoracic Vertebrae
  • [MeSH-minor] Adolescent. Anti-Bacterial Agents / therapeutic use. Antineoplastic Agents / therapeutic use. Back Pain / diagnosis. Back Pain / etiology. Biopsy, Fine-Needle. Bone Neoplasms / diagnosis. Bone Neoplasms / secondary. Cephalosporins / therapeutic use. Cerebral Ventricle Neoplasms / complications. Cerebral Ventricle Neoplasms / drug therapy. Cerebral Ventricle Neoplasms / pathology. Cerebral Ventricle Neoplasms / radiotherapy. Diagnosis, Differential. Drug Therapy, Combination. Humans. Imipenem / therapeutic use. Magnetic Resonance Imaging. Male. Treatment Outcome

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  • (PMID = 15332328.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Anti-Bacterial Agents; 0 / Antineoplastic Agents; 0 / Cephalosporins; 71OTZ9ZE0A / Imipenem; 807PW4VQE3 / cefepime
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11. Radosavljević D, Jelić S, Tomasević Z, Matković S, Stamatović L, Nikolić-Tomasević Z, Popov I: High-dose anthracyclines in the treatment of advanced primitive neuroectodermal tumors in adults--a single institution experience. Med Sci Monit; 2000 May-Jun;6(3):512-8
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  • [Title] High-dose anthracyclines in the treatment of advanced primitive neuroectodermal tumors in adults--a single institution experience.
  • Primitive neuroectodermal tumors (PNET) are rare malignancies of presumed neural crest origin, most often presenting as bone or soft tissue masses in the trunk or axial skeleton, in children and young adults.
  • Treatment of advanced PNET in adults is not clearly defined in the literature.
  • Data concerning dose-intensive chemotherapy regimens for poor-risk patients with those tumors are sparse, due to rarity of PNET in adults, their diverse presentation, the variable treatment procedures applied and the absence of direct comparisons.
  • On the other hand, the role of anthracyclines in the treatment of advanced soft tissue sarcomas is well known and substantial.
  • Six advanced PNET patients were treated at the Institute for Oncology and Radiology of Serbia, during last five years, with high-doses of doxorubicin or epidoxorubicin combined with cisplatin.
  • The paper reviews each of our patients, discussing how does chemotherapy influence the outcome in these patients, in context of the feasibility of high-doses of anthracyclines in advanced settings.
  • High dose anthracyclines (epidoxorubicin 150 mg/m2) in combination with cisplatin 120 mg/m2 induced a complete response lasting for 63+ months in a patient with desmoplastic medulloblastoma of the cerebellum metastatic to bones and bone marrow.
  • The same treatment but with the epidoxorubicin dose of 180 mg/m2 induced a complete response in a patient with olfactory neuroblastoma.
  • Administration of high dose Doxorubicin (75 mg/m2) seems feasible in association with irradiation treatment in patients with extraosseal Ewing sarcoma/PNET but the place of high dose chemotherapy within this setting remains to be determined.
  • [MeSH-major] Antibiotics, Antineoplastic / therapeutic use. Doxorubicin / therapeutic use. Epirubicin / therapeutic use. Neuroectodermal Tumors, Primitive / drug therapy
  • [MeSH-minor] Adult. Cerebellar Neoplasms / drug therapy. Cerebellar Neoplasms / radiography. Disease-Free Survival. Female. Humans. Male. Medulloblastoma / drug therapy. Medulloblastoma / radiography. Neuroblastoma / drug therapy. Neuroblastoma / radiography. Treatment Outcome

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  • (PMID = 11208363.001).
  • [ISSN] 1234-1010
  • [Journal-full-title] Medical science monitor : international medical journal of experimental and clinical research
  • [ISO-abbreviation] Med. Sci. Monit.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Poland
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 3Z8479ZZ5X / Epirubicin; 80168379AG / Doxorubicin
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12. Cai DX, Mafra M, Schmidt RE, Scheithauer BW, Park TS, Perry A: Medulloblastomas with extensive posttherapy neuronal maturation. Report of two cases. J Neurosurg; 2000 Aug;93(2):330-4
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  • [Title] Medulloblastomas with extensive posttherapy neuronal maturation. Report of two cases.
  • The authors report on two patients with classic medulloblastoma, each of whom underwent extensive therapy-associated neuronal maturation.
  • The first patient presented at 3 months of age with hydrocephalus caused by a 5-cm tumor in the cerebellar vermis.
  • He underwent a gross-total resection of a desmoplastic medulloblastoma.
  • Despite adjuvant chemotherapy, a 1.5-cm recurrent tumor developed 6 months later.
  • Sections from the subtotally resected tumor demonstrated exclusively mature neuronal elements, ranging from neurocytes to ganglion cells.
  • The specimen collected this time demonstrated classic medulloblastoma morphological characteristics.
  • The patient was subsequently treated with radiation therapy, which seemed to have an effect; however, the tumor eventually progressed and the patient died.
  • The second patient presented at 3 years of age with a midline medulloblastoma and was treated with subtotal resection, radiation therapy, and chemotherapy.
  • Histological examination revealed predominantly small mature neurons with scattered ganglion cells and extensive calcification.
  • No adjuvant therapy was given and the patient is alive and well as of his last follow-up examination.
  • The mature neuronal neoplasms resected in both patients demonstrated negligible proliferative indices and stained appropriately with neuronal immunohistochemical markers.
  • The smaller neuronal population resembled those of a central neurocytoma and medullocytoma/cerebellar neurocytoma.
  • Analogous to neuroblastoma, our cases suggest that adjuvant therapy can induce extensive or complete neuronal maturation in medulloblastoma.
  • [MeSH-major] Cell Differentiation. Cerebellar Neoplasms / physiopathology. Medulloblastoma / physiopathology
  • [MeSH-minor] Adolescent. Chemotherapy, Adjuvant. Child, Preschool. Humans. Male. Neurons / cytology. Prognosis. Radiotherapy, Adjuvant

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  • (PMID = 10930022.001).
  • [ISSN] 0022-3085
  • [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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13. Kalifa C, Grill J: The therapy of infantile malignant brain tumors: current status? J Neurooncol; 2005 Dec;75(3):279-85
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  • [Title] The therapy of infantile malignant brain tumors: current status?
  • Genetic predisposition to infantile malignant brain tumors are known in Gorlin syndrome for example who present with desmoplastic medulloblastoma in about 5% of the affected patients.
  • In addition, sequelae from tumor and its treatment are more severe at this age [2].
  • Thus, malignant brain tumors represent a true therapeutic challenge in neuro-oncology.
  • Consequently, the pediatric oncologists are more often confronted with very young children who need a complementary treatment.
  • Before the development of specific approaches for this age group, these children received the same kind of treatment than the older children did, but their survival and quality of life were significantly worse.
  • The reasons of these poor results were probably due in part to the fear of late effects induced by radiation therapy, leading to decrease the necessary doses of irradiation which increased treatment failures without avoiding treatment related complications [3].
  • At the end of the 80s, pilot studies were performed using postoperative chemotherapy in young medulloblastoma patients.
  • Van Eys treated 12 selected children with medulloblastoma with MOPP regimen and without irradiation; 8 of them were reported to be long term survivors [4].
  • Subsequently, the pediatric oncology cooperative groups studies have designed therapeutic trials for very young children with malignant brain tumors.
  • Different approaches have been explored: * Prolonged postoperative chemotherapy and delayed irradiation as designed in the POG (Pediatric Oncology Group).
  • * Postoperative chemotherapy without irradiation in the SFOP (Société Française d'Oncologie Pédiatrique) and in the GPO (German Pediatric Oncology) studies.
  • * The role of high-dose chemotherapy with autologous stem cells transplantation was explored in different ways: High-dose chemotherapy given in all patients as proposed in the Head Start protocol.
  • High-dose chemotherapy given in relapsing patients as salvage treatment in the French strategy.
  • In the earliest trials, the same therapy was applied to all histological types of malignant brain tumors and whatever the initial extension of the disease.
  • All these collected data have brought to an increased knowledge of infantile malignant brain tumors in terms of diagnosis, prognostic factors and response to chemotherapy.
  • Prospective study of sequelae can bring information on the impact of the different factors as hydrocephalus, location of the tumor, surgical complications, chemotherapy toxicity and irradiation modalities.
  • With these informations it is now possible to design therapeutic trials devoted to each histological types, adapted to pronostic factors and more accurate treatment to decrease long term sequelae.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Brain Neoplasms / surgery
  • [MeSH-minor] Cerebellar Neoplasms / drug therapy. Cerebellar Neoplasms / radiotherapy. Cerebellar Neoplasms / surgery. Combined Modality Therapy. Glioma / drug therapy. Glioma / radiotherapy. Glioma / surgery. Humans. Infant. Infant, Newborn. Infant, Newborn, Diseases. Medulloblastoma / drug therapy. Medulloblastoma / radiotherapy. Medulloblastoma / surgery. Neuroectodermal Tumors, Primitive / drug therapy. Neuroectodermal Tumors, Primitive / radiotherapy. Neuroectodermal Tumors, Primitive / surgery. Prognosis. Stem Cell Transplantation

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  • (PMID = 16195802.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 40
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14. Grill J, Bhangoo R: Recent development in chemotherapy of paediatric brain tumours. Curr Opin Oncol; 2007 Nov;19(6):612-5
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  • [Title] Recent development in chemotherapy of paediatric brain tumours.
  • PURPOSE OF REVIEW: Chemotherapy has gained a larger importance in the management of brain tumours, especially in children.
  • RECENT FINDINGS: Converging results were presented in 2005 by the German, French and North-American cooperative groups indicating that a subgroup of young children with medulloblastoma (i.e. those with desmoplastic histology) could be cured with chemotherapy only strategies.
  • The usefulness of high-dose chemotherapy followed by stem-cell transplant was shown not only as salvage strategy but also upfront in high-risk patients with medulloblastoma.
  • Targeted therapies have entered the paediatric neuro-oncology field as well.
  • SUMMARY: In the most frequent paediatric brain tumors (medulloblastoma and low grade gliomas), the improvements have been impressive in recent years.
  • These patients still await new targeted therapies to lower the burden of treatments and their related side-effects.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Brain Neoplasms / drug therapy. Glioma / drug therapy. Medulloblastoma / drug therapy
  • [MeSH-minor] Child. Clinical Trials as Topic. Humans. Medical Oncology / methods. Pediatrics / methods. Treatment Outcome

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  • (PMID = 17906461.001).
  • [ISSN] 1040-8746
  • [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 / Antineoplastic Agents
  • [Number-of-references] 36
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15. Pizem J, Cör A, Zadravec Zaletel L, Popovic M: Prognostic significance of apoptosis in medulloblastoma. Neurosci Lett; 2005 Jun 10-17;381(1-2):69-73
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  • [Title] Prognostic significance of apoptosis in medulloblastoma.
  • Since apoptosis is a major contributor to cell loss in medulloblastoma, either spontaneous or induced by radiation and chemotherapy, the apoptotic rate in resection specimens could have prognostic significance.
  • We analysed the apoptotic rate in 58 medulloblastoma resection specimens using an antibody against cleaved caspase 3, a specific marker of apoptotic cell death, and tested its possible prognostic significance.
  • The apoptotic rate varied considerably among medulloblastomas (0.1-25.9%, median 1.1%).
  • The apoptotic rate was higher in medulloblastomas with CSF dissemination, tended to be higher in desmoplastic medulloblastomas, but there was no association with age group and sex.
  • The variation in apoptotic rate among medulloblastomas is very likely predominantly associated with variations in tumour microenvironment, as supported by apoptotic cell clustering and rimming around necrotic areas.
  • The apoptotic rate in medulloblastoma resection specimens does not seem to be of prognostic value.
  • [MeSH-major] Apoptosis. Cerebellar Neoplasms / classification. Cerebellar Neoplasms / pathology. Medulloblastoma / classification. Medulloblastoma / pathology. Risk Assessment / methods

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  • (PMID = 15882792.001).
  • [ISSN] 0304-3940
  • [Journal-full-title] Neuroscience letters
  • [ISO-abbreviation] Neurosci. Lett.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Ireland
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16. Gajjar A, Chintagumpala M, Ashley D, Kellie S, Kun LE, Merchant TE, Woo S, Wheeler G, Ahern V, Krasin MJ, Fouladi M, Broniscer A, Krance R, Hale GA, Stewart CF, Dauser R, Sanford RA, Fuller C, Lau C, Boyett JM, Wallace D, Gilbertson RJ: Risk-adapted craniospinal radiotherapy followed by high-dose chemotherapy and stem-cell rescue in children with newly diagnosed medulloblastoma (St Jude Medulloblastoma-96): long-term results from a prospective, multicentre trial. Lancet Oncol; 2006 Oct;7(10):813-20
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  • [Title] Risk-adapted craniospinal radiotherapy followed by high-dose chemotherapy and stem-cell rescue in children with newly diagnosed medulloblastoma (St Jude Medulloblastoma-96): long-term results from a prospective, multicentre trial.
  • BACKGROUND: Current treatment for medulloblastoma, which includes postoperative radiotherapy and 1 year of chemotherapy, does not cure many children with high-risk disease.
  • We aimed to investigate the effectiveness of risk-adapted radiotherapy followed by a shortened period of dose-intense chemotherapy in children with medulloblastoma.
  • METHODS: After resection, patients were classified as having average-risk medulloblastoma (< or = 1.5 cm2 residual tumour and no metastatic disease) or high-risk medulloblastoma (> 1.5 cm2 residual disease or metastatic disease localised to neuraxis) medulloblastoma.
  • All patients received risk-adapted craniospinal radiotherapy (23.4 Gy for average-risk disease and 36.0-39.6 Gy for high-risk disease) followed by four cycles of cyclophosphamide-based, dose-intensive chemotherapy.
  • Patients were assessed regularly for disease status and treatment side-effects.
  • FINDINGS: Of 134 children with medulloblastoma who underwent treatment (86 average-risk, 48 high-risk), 119 (89%) completed the planned protocol.
  • No treatment-related deaths occurred.
  • For the 116 patients whose histology was reviewed centrally, histological subtype correlated with 5-year event-free survival (p=0.04): 84% (74-95) for classic histology, 77% (49-100) for desmoplastic tumours, and 57% (33-80) for large-cell anaplastic tumours.
  • INTERPRETATION: Risk-adapted radiotherapy followed by a shortened schedule of dose-intensive chemotherapy can be used to improve the outcome of patients with high-risk medulloblastoma.
  • [MeSH-major] Cerebellar Neoplasms / therapy. Cranial Irradiation / methods. Cyclophosphamide / therapeutic use. Hematopoietic Stem Cell Transplantation / methods. Medulloblastoma / therapy
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Agents, Alkylating / therapeutic use. Child. Child, Preschool. Combined Modality Therapy. Disease Progression. Dose-Response Relationship, Drug. Female. Follow-Up Studies. Humans. Male. Prospective Studies. Risk Factors. Survival Analysis. Survival Rate. Transplantation, Autologous. Treatment Outcome

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  • [CommentIn] Curr Neurol Neurosci Rep. 2007 Mar;7(2):130, 132 [17324362.001]
  • [CommentIn] Lancet Oncol. 2006 Oct;7(10):787-9 [17012036.001]
  • [ErratumIn] Lancet Oncol. 2006 Oct;7(10):797
  • (PMID = 17012043.001).
  • [ISSN] 1470-2045
  • [Journal-full-title] The Lancet. Oncology
  • [ISO-abbreviation] Lancet Oncol.
  • [Language] eng
  • [Databank-accession-numbers] ClinicalTrials.gov/ NCT00003211
  • [Grant] United States / NCI NIH HHS / CA / CA 21765
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 8N3DW7272P / Cyclophosphamide
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17. Dhall G, Grodman H, Ji L, Sands S, Gardner S, Dunkel IJ, McCowage GB, Diez B, Allen JC, Gopalan A, Cornelius AS, Termuhlen A, Abromowitch M, Sposto R, Finlay JL: Outcome of children less than three years old at diagnosis with non-metastatic medulloblastoma treated with chemotherapy on the "Head Start" I and II protocols. Pediatr Blood Cancer; 2008 Jun;50(6):1169-75
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Outcome of children less than three years old at diagnosis with non-metastatic medulloblastoma treated with chemotherapy on the "Head Start" I and II protocols.
  • PURPOSE: To determine the survival of infants and young children with non-metastatic medulloblastoma using intensive myeloablative chemotherapy and autologous hematopoietic progenitor cell rescue (AuHCR).
  • METHODS: Twenty-one children less than 3 years old at diagnosis with non-metastatic medulloblastoma were enrolled on two identical serial studies, "Head Start" I and "Head Start" II.
  • After surgery, patients received five cycles of induction chemotherapy consisting of vincristine, cisplatin, cyclophosphamide and etoposide.
  • Following induction, all patients underwent myeloablative chemotherapy using carboplatin, thiotepa and etoposide with AuHCR.
  • RESULTS: The 5-year event-free (EFS) and overall survival (OS) rates (+/-SE) for all patients, patients with gross total resection, and patients with residual tumor were 52 +/- 11% and 70 +/- 10%, 64 +/- 13% and 79 +/- 11%, and 29 +/- 17% and 57 +/- 19%, respectively.
  • The 5-year EFS and OS ( +/- SE) for patients with desmoplastic and classical medulloblastoma were 67 +/- 16% and 78 +/- 14%, and 42 +/- 14 and 67 +/- 14%, respectively.
  • There were four treatment related deaths.
  • CONCLUSION: This strategy of brief intensive chemotherapy for young children with non-metastatic medulloblastoma eliminated the need for craniospinal irradiation 52% of the patients, and may preserve QoL and intellectual functioning.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cerebellar Neoplasms / drug therapy. Medulloblastoma / drug therapy
  • [MeSH-minor] Child Behavior. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Humans. Infant. Intelligence Tests. Male. Neuropsychological Tests. Quality of Life. Survival Rate

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 18293379.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study; Randomized Controlled Trial; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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18. Grundy RG, Wilne SH, Robinson KJ, Ironside JW, Cox T, Chong WK, Michalski A, Campbell RH, Bailey CC, Thorp N, Pizer B, Punt J, Walker DA, Ellison DW, Machin D, Children's Cancer and Leukaemia Group (formerly UKCCSG) Brain Tumour Committee: Primary postoperative chemotherapy without radiotherapy for treatment of brain tumours other than ependymoma in children under 3 years: results of the first UKCCSG/SIOP CNS 9204 trial. Eur J Cancer; 2010 Jan;46(1):120-33
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  • [Title] Primary postoperative chemotherapy without radiotherapy for treatment of brain tumours other than ependymoma in children under 3 years: results of the first UKCCSG/SIOP CNS 9204 trial.
  • BACKGROUND: Radiotherapy is an effective adjuvant treatment for brain tumours arising in very young children, but it has the potential to damage the child's developing nervous system at a crucial time - with a resultant reduction in IQ leading to cognitive impairment, associated endocrinopathy and risk of second malignancy.
  • We aimed to assess the role of a primary chemotherapy strategy in avoiding or delaying radiotherapy in children younger than 3 years with malignant brain tumours other than ependymoma, the results of which have already been published.
  • METHODS: Ninety-seven children were enrolled between March 1993 and July 2003 and, following diagnostic review, comprised: medulloblastoma (n=31), astrocytoma (26), choroid plexus carcinoma [CPC] (15), CNS PNET (11), atypical teratoid/rhabdoid tumours [AT/RT] (6) and ineligible (6).
  • Following maximal surgical resection, chemotherapy was delivered every 14 d for 1 year or until disease progression.
  • FINDINGS: Over all diagnostic groups the cumulative progression rate was 80.9% at 5 years while the corresponding need-for-radiotherapy rate for progression was 54.6%, but both rates varied by tumour type.
  • There was no clear relationship between chemotherapy dose intensity and outcome.
  • Patients with medulloblastoma presented as a high-risk group, 83.9% having residual disease and/or metastases at diagnosis.
  • The 5-year OS for desmoplastic/nodular medulloblastoma was 52.9% (95% confidence interval (CI): 27.6-73.0) and for classic medulloblastoma 33.3% (CI: 4.6-67.6); the 5-year EFS were 35.3% (CI: 14.5-57.0) and 33.3% (CI: 4.6-67.6), respectively.
  • All children with large cell or anaplastic variants of medulloblastoma died within 2 years of diagnosis.
  • For CPC the 5-year OS was 26.67% (CI: 8.3-49.6) without RT.
  • This treatment strategy was less effective for AT/RT with 3-year OS of 16.7% (CI: 0.8-51.7) and CNS PNET with 1-year OS of 9.1% (CI: 0.5-33.3).
  • INTERPRETATION: The outcome for very young children with brain tumours is dictated by degree of surgical resection and histological tumour type and underlying biology as an indicator of treatment sensitivity.
  • Desmoplastic/nodular sub-type of medulloblastoma has a better prognosis than classic histology, despite traditional adverse clinical features of metastatic disease and incomplete surgical resection.
  • A subgroup with HGG and CPC are long-term survivors without RT.
  • This study highlights the differing therapeutic challenges presented by the malignant brain tumours of early childhood, the importance of surgical approaches and the need to explore individualised brain sparing approaches to the range of malignant brain tumours that present in early childhood.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy
  • [MeSH-minor] Astrocytoma / drug therapy. Astrocytoma / radiotherapy. Astrocytoma / surgery. Child, Preschool. Choroid Plexus Neoplasms / drug therapy. Choroid Plexus Neoplasms / radiotherapy. Choroid Plexus Neoplasms / surgery. Disease Progression. Drug Administration Schedule. Female. Follow-Up Studies. Humans. Infant. Male. Medulloblastoma / drug therapy. Medulloblastoma / radiotherapy. Medulloblastoma / surgery. Neuroectodermal Tumors, Primitive / drug therapy. Neuroectodermal Tumors, Primitive / radiotherapy. Neuroectodermal Tumors, Primitive / surgery. Radiotherapy, Adjuvant / methods. Survival Analysis. Teratoma / drug therapy. Teratoma / radiotherapy. Teratoma / surgery. Treatment Outcome

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  • (PMID = 19818598.001).
  • [ISSN] 1879-0852
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Grant] United Kingdom / Cancer Research UK / /
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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19. Rutkowski S, Cohen B, Finlay J, Luksch R, Ridola V, Valteau-Couanet D, Hara J, Garre ML, Grill J: Medulloblastoma in young children. Pediatr Blood Cancer; 2010 Apr;54(4):635-7
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  • [Title] Medulloblastoma in young children.
  • In early childhood medulloblastoma, three distinct treatment strategies are currently used by different national groups to improve survival rates and to delay or avoid craniospinal radiotherapy:.
  • (1) systemic chemotherapy and high-dose chemotherapy, followed by radiotherapy at relapse;.
  • (2) systemic and intraventricular chemotherapy;.
  • (3) systemic chemotherapy and local conformal radiotherapy.
  • A role for high-dose chemotherapy to delay or avoid craniospinal radiotherapy as a part of multimodal treatment strategies, especially in young children with metastatic or postoperative residual disease, has been recognized by different co-operative groups.
  • Clinical and histological factors such as nodular-desmoplastic variants are considered as important prognostic factors for risk-adapted treatment recommendations.
  • [MeSH-major] Cerebellar Neoplasms / therapy. Clinical Trials as Topic. Medulloblastoma / therapy
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Child, Preschool. Combined Modality Therapy. Humans. Infant. Radiotherapy

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  • (PMID = 20146217.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 14
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20. Kortmann RD, Brandes AA: Current and future strategies in the management of medulloblastoma in adults. Forum (Genova); 2003;13(1):99-110

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Current and future strategies in the management of medulloblastoma in adults.
  • Medulloblastoma in adults are rare and differ from their childhood counterparts in terms of tumor site (hemispheric location), histological subtype (desmoplastic variant), lower incidence of metastatic spread and frequent late relapses.
  • The therapeutic strategies are essentially based on experiences in childhood medulloblastomas.
  • Surgery followed by irradiation of the entire central nervous system followed by a boost to the posterior fossa is the cornerstones of treatment.
  • A sufficient dose prescription and a high quality of treatment are a prerequisite for an optimal tumor control.
  • Unlike in children the role of chemotherapy is an open question and requires prospective investigations.

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  • (PMID = 14732891.001).
  • [ISSN] 1970-0008
  • [Journal-full-title] Forum (Genoa, Italy)
  • [ISO-abbreviation] Forum (Genova)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
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21. Menon G, Krishnakumar K, Nair S: Adult medulloblastoma: clinical profile and treatment results of 18 patients. J Clin Neurosci; 2008 Feb;15(2):122-6
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  • [Title] Adult medulloblastoma: clinical profile and treatment results of 18 patients.
  • The objective of this article is to examine the clinicoradiological features and surgical outcomes of adult patients (>16 years) with medulloblastoma.
  • An attempt was made to identify the predictors of poor outcome and assess patterns of relapse and to compare these with pediatric medulloblastoma.
  • Statistical analysis was performed using chi-square test, Fischer's exact test and Student's t-test.
  • The tumor was located in the vermis in 12 patients (66.6%) and in the cerebellar hemisphere in six (16.6%).
  • All patients underwent adjuvant radiotherapy in the form of craniospinal irradiation with a posterior fossa boost.
  • All 11 subsequently underwent chemotherapy.
  • In spite of recent advances in management, patients with medulloblastoma still have a poor prognosis.
  • Desmoplastic variant histology was not observed to be a significant prognostic factor in the adult group while brain stem invasion carried a poor prognosis.
  • [MeSH-major] Cerebellar Neoplasms / therapy. Clinical Trials as Topic. Medulloblastoma / therapy
  • [MeSH-minor] Adolescent. Adult. Age Factors. Female. Humans. Male. Middle Aged. Prognosis. Retrospective Studies. Statistics, Nonparametric. Treatment Outcome

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  • (PMID = 18078755.001).
  • [ISSN] 0967-5868
  • [Journal-full-title] Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
  • [ISO-abbreviation] J Clin Neurosci
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Scotland
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22. Chan AW, Tarbell NJ, Black PM, Louis DN, Frosch MP, Ancukiewicz M, Chapman P, Loeffler JS: Adult medulloblastoma: prognostic factors and patterns of relapse. Neurosurgery; 2000 Sep;47(3):623-31; discussion 631-2
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  • [Title] Adult medulloblastoma: prognostic factors and patterns of relapse.
  • OBJECTIVE: To determine the patterns of relapse and the prognostic factors for adult medulloblastomas treated in the magnetic resonance imaging era.
  • METHODS: Between 1986 and 1996, 32 adult patients (age, > or =16 yr) with medulloblastomas confined to the craniospinal axis were treated in our institutions.
  • Twenty cases involved classic histological features and 12 involved the desmoplastic variant.
  • All patients received postoperative radiotherapy, with median doses of 36 Gy to the entire craniospinal axis and 55 Gy to the posterior fossa.
  • Twenty-four patients received chemotherapy (20 before radiotherapy, 3 after radiotherapy, and 1 before and after radiotherapy).
  • RESULTS: With a median follow-up period of 5.4 years, 17 patients experienced recurrences.
  • Twenty-nine percent of all relapses occurred more than 5 years after treatment.
  • In univariate analyses, factors adversely affecting posterior fossa control were less than complete resection (P<0.001), the presence of brainstem invasion (P = 0.02), and the use of chemotherapy (P = 0.03).
  • CONCLUSION: Late relapse is common among adult patients with medulloblastomas, and long-term follow-up monitoring is important.
  • Because of the high risk of systemic failure among the low-risk patients treated with radiotherapy alone, the role of chemotherapy for this group of patients needs to be further investigated.
  • [MeSH-major] Cerebellar Neoplasms / surgery. Medulloblastoma / surgery
  • [MeSH-minor] Adolescent. Adult. Cerebellum / pathology. Cerebellum / surgery. Combined Modality Therapy. Female. Humans. Male. Middle Aged. Neoadjuvant Therapy. Neoplasm Recurrence, Local / mortality. Neoplasm Recurrence, Local / pathology. Neoplasm Recurrence, Local / surgery. Neoplasm Staging. Prognosis. Salvage Therapy. Survival Rate

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  • (PMID = 10981749.001).
  • [ISSN] 0148-396X
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] UNITED STATES
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23. Abacioglu U, Uzel O, Sengoz M, Turkan S, Ober A: Medulloblastoma in adults: treatment results and prognostic factors. Int J Radiat Oncol Biol Phys; 2002 Nov 1;54(3):855-60
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  • [Title] Medulloblastoma in adults: treatment results and prognostic factors.
  • PURPOSE: To investigate the treatment outcome and prognostic factors of adult medulloblastoma patients who received postoperative craniospinal irradiation (RT).
  • METHODS AND MATERIALS: Between 1983 and 2000, 30 adult patients (17 men and 13 women, age >or=16 years, median 27, range 16-45) underwent postoperative RT.
  • A desmoplastic variant was seen in 12 (40%).
  • All patients received craniospinal RT.
  • The median dose to the whole brain was 40 Gy (range 36-51), to the posterior fossa 54 Gy (range 49-56), and to the spinal axis 36 Gy (range 24-40).
  • The median interval between surgery and the start of RT was 31 days (range 12-69), and the median duration of RT was 45 days (range 34-89).
  • Ten patients (33%) received adjuvant chemotherapy.
  • Twelve patients (40%) developed relapse, with a median follow-up of 51 months.
  • The median time to relapse was 26 months (range 4-78).
  • In univariate analysis, M stage and the interval between surgery and the start of RT were significant prognostic factors for disease-free survival.
  • The 5-year disease-free survival rate for the patients whose interval between surgery and the start of RT was <3 weeks, between 3 and 6 weeks, and >6 weeks was 0%, 85%, and 75%, respectively (p = 0.002).
  • The 5-year posterior fossa control rate for patients who received >or=54 Gy or <54 Gy to the posterior fossa was 91% and 33%, respectively (p = 0.05).
  • CONCLUSION: The survival results for medulloblastomas in adults compare favorably with those in children.
  • However, late relapses, lateral tumor location, and desmoplastic histologic features are more frequent in adults.
  • A minimal dose of 54 Gy to the posterior fossa is essential for adequate tumor control.
  • The interval between surgery and the start of RT, which was found to be a significant prognostic factor, is an interesting issue that requires further study.
  • [MeSH-major] Cerebellar Neoplasms / radiotherapy. Cranial Irradiation. Medulloblastoma / radiotherapy. Spine
  • [MeSH-minor] Adolescent. Adult. Analysis of Variance. Female. Humans. Male. Middle Aged. Postoperative Care. Prognosis. Radiotherapy Dosage. Recurrence. Treatment Outcome

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  • (PMID = 12377339.001).
  • [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
  • [Publication-country] United States
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24. Boiardi A, Silvani A, Eoli M, Fariselli L, Zappacosta B, Salmaggi A: Embryonal tumors in the adult population: implications in therapeutic planning. Neurol Sci; 2000 Feb;21(1):23-30
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  • [Title] Embryonal tumors in the adult population: implications in therapeutic planning.
  • The natural history of neuroectodermal tumors is still debated as far as prognostic factors are concerned; the same uncertainty applies to the optimal radiotherapy schedule and even more to the presumptive additive effect of chemotherapy.
  • We evaluated clinical course in a cohort of 39 patients, including 31 with medulloblastoma (MB) and 8 with primitive neuroectodermal tumors (PNET).
  • All patients were treated with radiotherapy, a standardized chemotherapy protocol including PCV scheme, and a second-line chemotherapy with cisplatin and etoposide (VP16) at recurrence.
  • In 27 patients, intrathecal chemotherapy was also delivered.
  • Overall, PNET had a worse outcome as compared to MB: median survival times were 42.8 vs. 92.6 months, respectively (p = 0.05).
  • No significant difference in disease-free period was found between patients of different age, desmoplastic variant, tumor localization, or extent of surgery.
  • Systemic or intrathecal chemotherapy did not influence progression-free survival (PFS).
  • However, in the majority of chemotherapy-treated patients, a low-dose craniospinal radiotherapy was also delivered.
  • This combination of treatments may have avoided the expected increased percentage of failure.
  • Moreover, more than half of recurrent patients had a partial response to chemotherapy that extended survival for approximately 3 years.
  • Repeated surgery and chemotherapy at recurrence favorably influenced survival time.
  • [MeSH-major] Brain Neoplasms / drug therapy. Cerebellar Neoplasms / drug therapy. Medulloblastoma / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy. Patient Care Planning
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cohort Studies. Combined Modality Therapy. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local / drug therapy. Retreatment. Survival Analysis

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  • (PMID = 10938199.001).
  • [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] Journal Article
  • [Publication-country] ITALY
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25. Riffaud L, Saikali S, Leray E, Hamlat A, Haegelen C, Vauleon E, Lesimple T: Survival and prognostic factors in a series of adults with medulloblastomas. J Neurosurg; 2009 Sep;111(3):478-87
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  • [Title] Survival and prognostic factors in a series of adults with medulloblastomas.
  • OBJECT: In this article, the authors report their experience in the management of adult patients with medulloblastoma at their institution to identify prognostic factors important for survival and disease control.
  • METHODS: Between 1977 and 2005, 27 patients who were >or=16 years old and had medulloblastoma were treated consecutively.
  • There were 16 women and 11 men with a median age of 21 years (range 16-54 years).
  • Six patients had the desmoplastic variant, and 21 patients presented with classic medulloblastoma.
  • Seven patients received chemotherapy before radiotherapy.
  • The median overall survival time was 17.7 years.
  • The median event-free survival time was 17.9 years.
  • Univariate analysis showed that survival was significantly correlated with sex (women had a better prognosis than men) and M stage (patients without metastases had a better outcome).
  • Patient age, duration of symptoms, Karnofsky Performance Scale score at presentation, hydrocephalus, tumor location, brainstem invasion, extent of resection, histological subtype, preradiotherapy chemotherapy, risk group, and period of presentation were not significant variables.
  • Multivariate analysis identified sex and M stage as well as the period of presentation as independent prognostic factors for overall and event-free survival times.
  • Eleven patients suffered tumor recurrence within a median time of 4.2 years.
  • All patients in whom the tumor recurred have died despite aggressive treatments.
  • The median survival time after diagnosis of recurrence was 2.5 years.
  • Questionnaires on quality of life and cognition showed high scores in favor of limited negative effects in the perception of mental and physical health after treatment.
  • CONCLUSIONS: Long-term survival is possible in adults treated for medulloblastoma.
  • Tumor recurrences should be treated with aggressive therapies as some patients may have sustained response.
  • Adjuvant chemotherapy should be given to high-risk patients, but its role in reducing recurrences, particularly distant ones, remains unclear in the standard-risk group.
  • [MeSH-major] Cerebellar Neoplasms / mortality. Medulloblastoma / mortality

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  • (PMID = 19231932.001).
  • [ISSN] 0022-3085
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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26. Krampulz T, Hans VH, Oppel F, Dietrich U, Puchner MJ: Long-term relapse-free survival with supratentorial primitive neuroectodermal tumor in an adult: a case report. J Neurooncol; 2006 May;77(3):291-4
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  • Prognosis is worse in comparison to infratentorial medulloblastoma.
  • The original histological diagnosis was that of an undifferentiated sarcoma, malignant hemangioendothelioma, grade III.
  • The patient was treated by CyVADIC chemotherapy and conventional radiation therapy (60 Gy).
  • Microscopy revealed a malignant, highly cellular, poorly differentiated tumor with a desmoplastic component.
  • Almost all cells were stained for neuron specific enolase and NGF-Rp75, with neuronal and glial markers being present to a variable extent.
  • CONCLUSIONS: Although the prognosis of sPNET is reported to be poor, a small fraction with a rather benign biological and clinical behavior exists.
  • The value of chemotherapy is an issue of continuous investigation.
  • [MeSH-major] Biomarkers, Tumor / metabolism. Neuroectodermal Tumors, Primitive / pathology. Sarcoma / pathology. Supratentorial Neoplasms / pathology
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Combined Modality Therapy. Diagnosis, Differential. Disease-Free Survival. Humans. Ki-67 Antigen / metabolism. Male. Receptor, Nerve Growth Factor / metabolism. Treatment Outcome

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  • (PMID = 16528456.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Ki-67 Antigen; 0 / Receptor, Nerve Growth Factor
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27. Pession A, Tonelli R: The MYCN oncogene as a specific and selective drug target for peripheral and central nervous system tumors. Curr Cancer Drug Targets; 2005 Jun;5(4):273-83
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The MYCN oncogene as a specific and selective drug target for peripheral and central nervous system tumors.
  • Identification of selective inhibitors of MYCN and its mRNA and protein could be important for the development of more specific, effective and less toxic therapeutic agents for tumors of the PNS and CNS.
  • In children, the most common tumors of the PNS and CNS are neuroblastomas and medulloblastomas, respectively.
  • MYCN amplification and over-expression has been reported in medulloblastoma, and especially in the desmoplastic type.
  • Peptide nucleic acids (PNA), which belong to the most recent (third) generation of nucleic acid therapeutics, form highly stable duplexes with DNA and RNA, and are resistant to degradation by nucleases and proteases.
  • [MeSH-major] Central Nervous System Neoplasms / drug therapy. Genes, myc / drug effects. Peripheral Nervous System Neoplasms / drug therapy

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  • (PMID = 15975048.001).
  • [ISSN] 1568-0096
  • [Journal-full-title] Current cancer drug targets
  • [ISO-abbreviation] Curr Cancer Drug Targets
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Netherlands
  • [Number-of-references] 119
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