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1. Bokemeyer C, Schleucher N, Metzner B, Thomas M, Rick O, Schmoll HJ, Kollmannsberger C, Boehlke I, Kanz L, Hartmann JT: First-line sequential high-dose VIP chemotherapy with autologous transplantation for patients with primary mediastinal nonseminomatous germ cell tumours: a prospective trial. Br J Cancer; 2003 Jul 7;89(1):29-35
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  • [Title] First-line sequential high-dose VIP chemotherapy with autologous transplantation for patients with primary mediastinal nonseminomatous germ cell tumours: a prospective trial.
  • To determine the efficacy of first-line sequential high-dose VIP chemotherapy (HD-VIP) in patients with primary mediastinal nonseminomatous germ cell tumours (GCT), 28 patients were enrolled on a German multicentre trial.
  • High-Dose VIP chemotherapy consisted of 3-4 cycles of dose-intensive etoposide and ifosfamide plus cisplatin, q22days, each cycle followed by autologous peripheral blood stem cell transplantation plus granulocyte-colony stimulating factor (G-CSF) support.
  • Ten patients had mediastinal involvement as the only manifestation (36 %), 18 of 28 patients had additional metastatic sites, such as lung (n=17; 61%), liver (n=7; 25%), bone (n=5; 18%), lymph nodes (n=3; 11%) and CNS (n=3; 11%).
  • Two patients developed recurrence of GCT or teratoma.
  • Compared to data of an international database analysis including 253 patients with mediastinal nonseminoma treated with conventional chemotherapy, the results may indicate that HD-VIP results in an approximately 15% survival improvement.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Germinoma / drug therapy. Mediastinal Neoplasms / drug therapy. Peripheral Blood Stem Cell Transplantation
  • [MeSH-minor] Adult. Cisplatin / administration & dosage. Combined Modality Therapy. Disease-Free Survival. Etoposide / administration & dosage. Female. Granulocyte Colony-Stimulating Factor / administration & dosage. Humans. Ifosfamide / administration & dosage. Male. Middle Aged. Neoplasm Recurrence, Local. Prognosis. Prospective Studies. Treatment Outcome

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  • (PMID = 12838296.001).
  • [ISSN] 0007-0920
  • [Journal-full-title] British journal of cancer
  • [ISO-abbreviation] Br. J. Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study
  • [Publication-country] England
  • [Chemical-registry-number] 143011-72-7 / Granulocyte Colony-Stimulating Factor; 6PLQ3CP4P3 / Etoposide; Q20Q21Q62J / Cisplatin; UM20QQM95Y / Ifosfamide; ICE protocol 1
  • [Other-IDs] NLM/ PMC2394224
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2. Chi SN, Zimmerman MA, Yao X, Cohen KJ, Burger P, Biegel JA, Rorke-Adams LB, Fisher MJ, Janss A, Mazewski C, Goldman S, Manley PE, Bowers DC, Bendel A, Rubin J, Turner CD, Marcus KJ, Goumnerova L, Ullrich NJ, Kieran MW: Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor. J Clin Oncol; 2009 Jan 20;27(3):385-9
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  • [Title] Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor.
  • PURPOSE: Atypical teratoid rhabdoid tumor (ATRT) of the CNS is a highly malignant neoplasm primarily affecting young children, with a historic median survival ranging from 6 to 11 months.
  • Based on a previous pilot series, a prospective multi-institutional trial was conducted for patients with newly diagnosed CNS ATRT.
  • PATIENTS AND METHODS: Treatment was divided into five phases: preirradiation, chemoradiation, consolidation, maintenance, and continuation therapy.
  • Intrathecal chemotherapy was administered, alternating intralumbar and intraventricular routes.
  • Radiation therapy (RT) was prescribed, either focal (54 Gy) or craniospinal (36 Gy, plus primary boost), depending on age and extent of disease at diagnosis.
  • Fifteen patients received radiation therapy: 11 focal and four craniospinal.
  • CONCLUSION: This intensive multimodality regimen has resulted in a significant improvement in time to progression and overall survival for patients with this previously poor-prognosis tumor.
  • [MeSH-major] Brain Neoplasms / therapy. Rhabdoid Tumor / therapy. Teratoma / therapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Combined Modality Therapy. Humans. Infratentorial Neoplasms. Prognosis. Prospective Studies. Supratentorial Neoplasms. Young Adult

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  • (PMID = 19064966.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / R01 CA046274; United States / NCI NIH HHS / CA / R01 CA046274-17A2; United States / NCI NIH HHS / CA / CA46274
  • [Publication-type] Journal Article; Multicenter Study; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC2645855
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3. Lee YK, Choi CG, Lee JH: Atypical teratoid/rhabdoid tumor of the cerebellum: report of two infantile cases. AJNR Am J Neuroradiol; 2004 Mar;25(3):481-3
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  • Atypical teratoid/rhabdoid tumor of the CNS is an aggressive infantile neoplasm of uncertain origin.
  • Despite intensive chemotherapy and radiation therapy, both of our two patients died within 8 months of pathologic diagnosis.
  • [MeSH-major] Cerebellar Neoplasms / congenital. Magnetic Resonance Imaging. Rhabdoid Tumor / congenital. Teratoma / congenital. Tomography, X-Ray Computed
  • [MeSH-minor] Biomarkers, Tumor / analysis. Cerebellum / pathology. Combined Modality Therapy. Dominance, Cerebral / physiology. Fatal Outcome. Female. Follow-Up Studies. Fourth Ventricle / pathology. Humans. Infant. Kidney Neoplasms / congenital. Kidney Neoplasms / diagnosis. Kidney Neoplasms / pathology. Kidney Neoplasms / therapy. Male. Medulla Oblongata / pathology. Neoplasms, Multiple Primary / congenital. Neoplasms, Multiple Primary / diagnosis. Neoplasms, Multiple Primary / pathology. Neoplasms, Multiple Primary / therapy

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  • (PMID = 15037476.001).
  • [ISSN] 0195-6108
  • [Journal-full-title] AJNR. American journal of neuroradiology
  • [ISO-abbreviation] AJNR Am J Neuroradiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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4. Fouladi M, Blaney SM, Poussaint TY, Freeman BB 3rd, McLendon R, Fuller C, Adesina AM, Hancock ML, Danks MK, Stewart C, Boyett JM, Gajjar A: Phase II study of oxaliplatin in children with recurrent or refractory medulloblastoma, supratentorial primitive neuroectodermal tumors, and atypical teratoid rhabdoid tumors: a pediatric brain tumor consortium study. Cancer; 2006 Nov 1;107(9):2291-7
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  • CONCLUSIONS: Oxaliplatin was well tolerated in children but has limited activity in children with recurrent CNS embryonal tumors previously treated with platinum compounds.
  • [MeSH-major] Medulloblastoma / drug therapy. Neoplasm Recurrence, Local / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy. Organoplatinum Compounds / therapeutic use. Rhabdoid Tumor / drug therapy. Supratentorial Neoplasms / drug therapy. Teratoma / drug therapy
  • [MeSH-minor] Adolescent. Antineoplastic Agents / administration & dosage. Antineoplastic Agents / pharmacokinetics. Antineoplastic Agents / therapeutic use. Child. Child, Preschool. Female. Humans. Infant. Male. Treatment Outcome


5. 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.
  • 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.
  • 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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6. Garrè ML, Tekautz T: Role of high-dose chemotherapy (HDCT) in treatment of atypical teratoid/rhabdoid tumors (AT/RTs). Pediatr Blood Cancer; 2010 Apr;54(4):647-8
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  • [Title] Role of high-dose chemotherapy (HDCT) in treatment of atypical teratoid/rhabdoid tumors (AT/RTs).
  • Atypical teratoid/rhabdoid tumors (AT/RTs) of the CNS have been recently characterized as a distinct clinicopathologic entity with an unusually poor prognosis and with the highest incidence in the first 2 years of life.
  • AT/RT of the CNS is a usually fatal disease virtually unresponsive to chemotherapy (CT) and radiotherapy (RT).
  • Rapid progression and CNS dissemination are commonly reported.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Central Nervous System Neoplasms / drug therapy. Rhabdoid Tumor / drug therapy. Teratoma / drug therapy

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  • (PMID = 20146222.001).
  • [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] 18
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7. Khatua S, Dhall G, O'Neil S, Jubran R, Villablanca JG, Marachelian A, Nastia A, Lavey R, Olch AJ, Gonzalez I, Gilles F, Nelson M, Panigrahy A, McComb G, Krieger M, Fan J, Sposto R, Finlay JL: Treatment of primary CNS germinomatous germ cell tumors with chemotherapy prior to reduced dose whole ventricular and local boost irradiation. Pediatr Blood Cancer; 2010 Jul 15;55(1):42-6
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  • [Title] Treatment of primary CNS germinomatous germ cell tumors with chemotherapy prior to reduced dose whole ventricular and local boost irradiation.
  • BACKGROUND: The purpose of this study was to evaluate a reduced irradiation dose strategy for newly diagnosed primary central nervous system (CNS) germinomas.
  • METHODS: Twenty patients with histologically diagnosed localized pure germinoma (n = 19) or germinoma with a mature teratoma component (n = 1) received four cycles of carboplatin and etoposide at 3-week intervals.
  • In 18 patients, chemotherapy was followed by whole ventricular irradiation to 21.6-25.5 Gy with a simultaneous integrated or sequential primary site boost to 30-30.6 Gy.
  • Neurocognitive function was evaluated periodically following treatment.
  • CONCLUSION: Chemotherapy followed by reduced dose whole ventricular and local boost irradiation appears to be effective in patients with localized pure CNS germinoma with evidence of preservation of neurocognitive function.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / therapy. Central Nervous System Neoplasms / therapy. Germinoma / therapy. Neoplasms, Germ Cell and Embryonal / therapy
  • [MeSH-minor] Adolescent. Adult. Carboplatin / adverse effects. Carboplatin / therapeutic use. Child. Child, Preschool. Chorionic Gonadotropin, beta Subunit, Human / blood. Combined Modality Therapy. Dose-Response Relationship, Radiation. Etoposide / adverse effects. Etoposide / therapeutic use. Female. Humans. Infant. Magnetic Resonance Imaging. Male. Retrospective Studies. Young Adult. alpha-Fetoproteins / analysis

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  • (PMID = 20222020.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Chorionic Gonadotropin, beta Subunit, Human; 0 / alpha-Fetoproteins; 6PLQ3CP4P3 / Etoposide; BG3F62OND5 / Carboplatin
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8. Matsutani M, Japanese Pediatric Brain Tumor Study Group: Combined chemotherapy and radiation therapy for CNS germ cell tumors--the Japanese experience. J Neurooncol; 2001 Sep;54(3):311-6
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  • [Title] Combined chemotherapy and radiation therapy for CNS germ cell tumors--the Japanese experience.
  • Among intracranial germ cell tumors, nongerminomatous tumors have proved refractory to conventional treatment with surgery and irradiation.
  • Since 1983, chemotherapy has been delivered in Japan as an adjuvant therapy in patients with intracranial nongerminomatous germ cell tumors.
  • Based on our clinical experience, we undertook a multi-institutional phase II study to establish post-surgical combined chemotherapy and radiation therapy for primary germ cell tumors in the brain.
  • We adopted carboplatin-etoposide (CARB-VP) or cisplatin-etoposide (PE) combination chemotherapy for patients with germinomas and those with tumors that placed them in the intermediate prognosis group, and ifosphamide-cisplatin-etoposide (ICE) for patients with tumors that placed them in the poor prognosis group.
  • Among patients with germinoma (n = 75), the rate or complete remission after combination therapy was 92.0%; it was 67.8% for patients in the intermediate prognosis group (n = 28).
  • Of 9 patients with a poor prognosis, 4 experienced disease progression during treatment and died within 10 months.
  • There were no serious complications attributable to the combination therapy.
  • Our treatment protocols are effective for patients with germinomas and those with an intermediate prognosis.
  • [MeSH-major] Central Nervous System Neoplasms / drug therapy. Central Nervous System Neoplasms / radiotherapy. Germinoma / drug therapy. Germinoma / radiotherapy. Postoperative Care. Teratoma / drug therapy. Teratoma / radiotherapy
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / adverse effects. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Combined Modality Therapy. Humans. Neoplasm Recurrence, Local. Prognosis. Remission Induction

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  • (PMID = 11767296.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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9. Gardner SL, Asgharzadeh S, Green A, Horn B, McCowage G, Finlay J: Intensive induction chemotherapy followed by high dose chemotherapy with autologous hematopoietic progenitor cell rescue in young children newly diagnosed with central nervous system atypical teratoid rhabdoid tumors. Pediatr Blood Cancer; 2008 Aug;51(2):235-40
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  • [Title] Intensive induction chemotherapy followed by high dose chemotherapy with autologous hematopoietic progenitor cell rescue in young children newly diagnosed with central nervous system atypical teratoid rhabdoid tumors.
  • BACKGROUND: Central nervous system (CNS) atypical teratoid rhabdoid tumors (AT/RT) are rare tumors of childhood with a dismal prognosis.
  • Historically, surgery and standard dose chemotherapy have resulted in a median survival of 8.5 months from diagnosis.
  • METHODS: Thirteen children newly diagnosed with CNS AT/RT were treated with either the "Head Start I" (HS I) or "Head Start II" (HS II) regimens.
  • Therapy included resection followed by five cycles of cisplatin, vincristine, cyclophosphamide, and etoposide.
  • Eight patients died of disease (six on HS I); one patient died from infection; one patient died from secondary malignancy following treatment for recurrent AT/RT.
  • CONCLUSION: Three of seven children with CNS AT/RT treated on HS II have experienced long term remissions.
  • Long term survival can be achieved in a subset of young children with CNS AT/RT following resection with the use of multi-drug chemotherapy including high dose methotrexate and myeloablative chemotherapy without radiation therapy (RT).
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / therapy. Hematopoietic Stem Cell Transplantation. Rhabdoid Tumor / therapy. Teratoma / therapy
  • [MeSH-minor] Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Transplantation, Autologous

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  • (PMID = 18381756.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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10. Drabko K, Wiśniewska-Slusarz H, Wójcik B, Choma M, Zaucha-Prazmo A, Kowalczyk JR: [Megachemotherapy followed by autologous haematopoietic stem cell rescue in children with high risk CNS tumours]. Med Wieku Rozwoj; 2005 Jul-Sep;9(3 Pt 2):439-47
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  • [Title] [Megachemotherapy followed by autologous haematopoietic stem cell rescue in children with high risk CNS tumours].
  • AIM: the Lublin bone marrow transplantation unit experience in megachemotherapy followed by autologus haematopoietic stem cell transplantation in children with high-risk CNS tumours is described.
  • The group consisted of 5 cases of medulloblastoma, and single cases of ependynmoma, teratoma, astrocytoma anaplasticum and glioblastoma.
  • Stem cell apheresis from peripheral blood was performed during chemotherapy after surgery.
  • Seven children are alive and well with the median observation time 23 months.
  • CONCLUSION: megachemotherapy followed by stem cell rescue is a safe and feasible procedure in children with malignant brain tumours and may improve results of the treatment in high-risk patients, but these results should be con firmed in larger series of patients.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Brain Neoplasms / surgery. Hematopoietic Stem Cell Transplantation
  • [MeSH-minor] Adolescent. Astrocytoma / drug therapy. Astrocytoma / surgery. Child. Dose-Response Relationship, Drug. Ependymoma / drug therapy. Ependymoma / surgery. Female. Glioblastoma / drug therapy. Glioblastoma / surgery. Glioma / drug therapy. Glioma / surgery. Humans. Male. Medulloblastoma / drug therapy. Medulloblastoma / surgery. Teratoma / drug therapy. Teratoma / surgery. Transplantation, Autologous. Treatment Outcome

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  • (PMID = 16719156.001).
  • [Journal-full-title] Medycyna wieku rozwojowego
  • [ISO-abbreviation] Med Wieku Rozwoj
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
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11. Chen YW, Wong TT, Ho DM, Huang PI, Chang KP, Shiau CY, Yen SH: Impact of radiotherapy for pediatric CNS atypical teratoid/rhabdoid tumor (single institute experience). Int J Radiat Oncol Biol Phys; 2006 Mar 15;64(4):1038-43
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  • [Title] Impact of radiotherapy for pediatric CNS atypical teratoid/rhabdoid tumor (single institute experience).
  • PURPOSE: To assess outcomes and prognostic factors in radiotherapy of pediatric central nervous system atypical teratoid/rhabdoid tumor (AT/RT).
  • METHODS AND MATERIALS: Seventeen patients with central nervous system AT/RT were retrospectively reviewed after curative radiotherapy as primary or adjuvant therapy between January 1990 and December 2003.
  • The log-rank method was used to compare the effects of dosage (>50 Gy or < or =50 Gy) and treatment duration (>45 days or < or =45 days).
  • Multivariate analysis revealed a significant relationship between the following: overall survival and performance status (p = 0.019), failure-free survival and total irradiation dose (p = 0.037), time interval between surgery and radiotherapy initiation (p = 0.031), and time interval between surgery and radiotherapy end point (p = 0.047).
  • CONCLUSION: Radiotherapy is crucial in the treatment of AT/RT.
  • We recommend initiating radiotherapy immediately postoperatively and before systemic chemotherapy in pediatric patients > or =3 years of age.
  • [MeSH-major] Brain Neoplasms / radiotherapy. Rhabdoid Tumor / radiotherapy. Teratoma / radiotherapy

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  • [CommentIn] Int J Radiat Oncol Biol Phys. 2006 Jul 15;65(4):1273; author reply 1273-4 [16798419.001]
  • (PMID = 16406394.001).
  • [ISSN] 0360-3016
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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12. Serowka K, Chiu Y, Gonzalez I, Gilles F, McComb G, Krieger M, Dhall G, Britt B, Ji L, Sposto R, Finlay JL: Central nervous system (CNS) tumors in the first six months of life: the Children's Hospital Los Angeles experience, 1979-2005. Pediatr Hematol Oncol; 2010 Mar;27(2):90-102
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Central nervous system (CNS) tumors in the first six months of life: the Children's Hospital Los Angeles experience, 1979-2005.
  • BACKGROUND: The authors report the experience at the Children's Hospital Los Angeles with brain tumors diagnosed before 6 months of age, describing the characteristics of the patients, their tumors, treatment strategies, and prognostic factors.
  • There were 11 gliomas, 9 choroid plexus tumors, 8 medulloblastomas and supratentorial primitive neuroectodermal tumors (PNET), 2 atypical teratoid/rhabdoid tumors (ATRT), and 1 each of ependymoma, craniopharyngioma, and immature teratoma.
  • The treatment strategies included 5 patients with biopsy only, 18 less than gross total resections (<GTRx), and 9 GTRx.
  • Fourteen children (42%) received chemotherapy.
  • [MeSH-minor] Female. Hospitals, Pediatric. Humans. Infant. Infant, Newborn. Los Angeles / epidemiology. Male. Prognosis. Survival Rate. Treatment Outcome

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  • (PMID = 20201690.001).
  • [ISSN] 1521-0669
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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13. Brandes AA, Pasetto LM, Monfardini S: The treatment of cranial germ cell tumours. Cancer Treat Rev; 2000 Aug;26(4):233-42
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  • [Title] The treatment of cranial germ cell tumours.
  • Germ cell tumours of the central nervous system (CNS) include many subtypes whose response to treatment varies, even though the symptoms and radiological appearances are similar.
  • Patients with choriocarcinoma, embryonal carcinoma, or yolk sac tumour have the lowest survival rates; patients with germinoma or mature teratoma have longer survival rates.
  • Beside the delayed injury induced by radiotherapy, the late injury induced by chemotherapy is becoming increasingly evident.
  • Cisplatin is considered an indispensable drug, but it may cause renal damage, ototoxicity, peripheral neuropathy and sterility, while etoposide is associated with an excess frequency of second neoplasms.
  • Taking into account all of the published literature, the following therapeutic options are suggested: in pure germinoma tumours (GT) radiotherapy alone will usually ensure adequate control of the disease, and the long-term sequelae may be limited by reducing the dose delivered, as was proposed for germ cell testicular tumours, to 30 Gy to limited fields plus 25-30 Gy to the spinal axis if there is disseminated disease.
  • In cases of recurrence, which should be uncommon, patients may be rescued with both radiotherapy and chemotherapy.
  • In NGGC tumours, the prognosis is more unfavourable and there is often dissemination to the spine at diagnosis; however, the tumour's high chemosensitivity suggests neoadjuvant treatment chemotherapy with cisplatin and etoposide for three cycles followed by consolidation radiotherapy with 40 Gy to the limited fields plus 30 Gy to the spinal axis if disseminated.
  • In our opinion, a higher dose of radiotherapy in cases in which chemotherapy does not achieve a radiological complete remission is not advisable, because very often the residual radiological abnormality does not represent biologically active tumour but differentiated forms such as mature teratoma.
  • The challenge for 2000 is to both cure these patients, and avoid the late and permanent sequelae of radiation and/or chemotherapy that may subsequently impair quality of life.
  • [MeSH-major] Brain Neoplasms / therapy. Neoplasms, Germ Cell and Embryonal / therapy
  • [MeSH-minor] Combined Modality Therapy. Cranial Irradiation. Drug Therapy. Humans. Neurosurgical Procedures. Prognosis. Radiotherapy Dosage. Survival Rate

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  • [Copyright] Copyright 2000 Harcourt Publishers Ltd.
  • (PMID = 10913379.001).
  • [ISSN] 0305-7372
  • [Journal-full-title] Cancer treatment reviews
  • [ISO-abbreviation] Cancer Treat. Rev.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] ENGLAND
  • [Number-of-references] 59
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14. Halperin EC: Neonatal neoplasms. Int J Radiat Oncol Biol Phys; 2000 Apr 1;47(1):171-8
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  • PURPOSE: To describe neoplasms diagnosed in children </= 28 days of age along with their treatment, associated congenital anomalies, and the long-term consequences of the diagnoses and treatments.
  • The 20 patients identified via the computerized registry system for 1980-1998 constitute 2% (20/925) of all neoplasms seen in patients </= 16 years of age over this same time period at DUMC.
  • The histologic diagnoses were teratoma/germ cell tumor (n = 8, 35%), neuroblastoma (n = 5, 22%), retinoblastoma (n = 4, 17%), primary central nervous system (CNS) tumor (n = 3, 13%), and one case each of rhabdomyosarcoma, glossal glial choristoma, and hemangioma in the setting of Kasabach-Merritt Syndrome.
  • Of the eight teratoma/germ cell tumor patients, 6 were female (75%) and 2 male (25%).
  • Two of the seven patients with immature teratomas or teratoma were long-term survivors following surgery.
  • The one patient with malignant germ cell tumor, treated with surgery and chemotherapy, died.
  • Two were treated with surgery + chemotherapy + radiotherapy; two with surgery + chemotherapy; and one with surgery alone.
  • A child with a dumbbell neuroblastoma, treated with surgery and chemotherapy, is paraplegic.
  • The two children with trilateral retinoblastoma died after therapy with surgery, craniospinal and orbital irradiation, and chemotherapy.
  • Two children with bilateral disease are long-term survivors: one treated with radiotherapy + chemotherapy and one with radiotherapy alone.
  • The three patients with CNS tumors were female.
  • Two of the patients are long-term survivors after surgery + chemotherapy.
  • Six children received eight courses of radiation therapy: 2 for Stage 4S neuroblastoma with respiratory compromise from an enlarging liver and 4 for retinoblastoma.
  • The two infants with trilateral retinoblastoma received two courses of irradiation each: one of the treatment of intraocular tumor and a second, at an older age, for the pineal tumor.
  • The most serious complication of anesthesia was a case of enterobacter cloacae sepsis in the central venous access line used for repetitively administering the anesthetic.
  • CONCLUSION: The most common neonatal neoplasm histologic diagnoses are teratoma/germ cell tumor, neuroblastoma, and retinoblastoma.
  • Radiation therapy is administered infrequently in a population highly susceptible to late ill effects.
  • When radiotherapy is required, anesthesia may be repetitively administered to aid in reproducible treatment.
  • [MeSH-minor] Anesthesia. Brain Neoplasms / epidemiology. Brain Neoplasms / pathology. Brain Neoplasms / therapy. Female. Follow-Up Studies. Hemangioma / epidemiology. Hemangioma / pathology. Hemangioma / therapy. Humans. Infant, Newborn. Male. Neuroblastoma / epidemiology. Neuroblastoma / pathology. Neuroblastoma / therapy. Registries. Retinoblastoma / epidemiology. Retinoblastoma / pathology. Retinoblastoma / therapy. Survivors. Teratoma / epidemiology. Teratoma / pathology. Teratoma / therapy

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  • (PMID = 10758320.001).
  • [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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15. Athale UH, Duckworth J, Odame I, Barr R: Childhood atypical teratoid rhabdoid tumor of the central nervous system: a meta-analysis of observational studies. J Pediatr Hematol Oncol; 2009 Sep;31(9):651-63
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  • [Title] Childhood atypical teratoid rhabdoid tumor of the central nervous system: a meta-analysis of observational studies.
  • PURPOSE: Therapy for central nervous system (CNS) atypical teratoid rhabdoid tumor (ATRT) is controversial.
  • We describe 4 children treated with sarcoma-like therapy and review the literature to evaluate outcome in relation to treatment modalities.
  • PROCEDURE: Reports from 1995 to 2007, describing clinical features of children (< or =18 years) were reviewed for details of demography, therapy, and outcome.
  • Kaplan-Meier survival analyses were used to study the impact of clinical features, demography, and therapy on overall survival (OS).
  • RESULTS: The median OS for patients treated with multiagent chemotherapy (n=79) was 17.3 months (range, 1.5-93 mo); unrelated to age at diagnosis, sex, tumor site, and extent of resection.
  • Patients (n=30) treated with intrathecal (IT) chemotherapy had significantly higher 2-year OS [64% (95% confidence interval, 46.5-82.0) vs. 17.3% (95% confidence interval, 5.4-29.3); P<0.0001] and lower prevalence of distant CNS metastasis compared with those without IT therapy (n=49) (20% vs. 59.2%; P=0.001).
  • CONCLUSIONS: Despite dismal OS, multimodal therapy can induce remission even in metastatic CNS ATRT with partial resection.
  • IT chemotherapy results in higher OS and, because of an overall high rate of distant relapse, should be considered in future trials.
  • [MeSH-major] Brain Neoplasms / epidemiology. Rhabdoid Tumor / epidemiology. Teratoma / epidemiology
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Cisplatin / administration & dosage. Combined Modality Therapy. Craniotomy. Cyclophosphamide / administration & dosage. Dactinomycin / administration & dosage. Doxorubicin / administration & dosage. Etoposide / administration & dosage. Fatal Outcome. Female. Humans. Infant. Infant, Newborn. Injections, Spinal. Kaplan-Meier Estimate. Male. Prognosis. Prospective Studies. Spinal Neoplasms / diagnosis. Spinal Neoplasms / drug therapy. Spinal Neoplasms / epidemiology. Spinal Neoplasms / radiotherapy. Spinal Neoplasms / surgery. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 19707161.001).
  • [ISSN] 1536-3678
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Meta-Analysis
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; Q20Q21Q62J / Cisplatin
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16. Zimmerman MA, Goumnerova LC, Proctor M, Scott RM, Marcus K, Pomeroy SL, Turner CD, Chi SN, Chordas C, Kieran MW: Continuous remission of newly diagnosed and relapsed central nervous system atypical teratoid/rhabdoid tumor. J Neurooncol; 2005 Mar;72(1):77-84
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  • [Title] Continuous remission of newly diagnosed and relapsed central nervous system atypical teratoid/rhabdoid tumor.
  • AT/RT can occur in the central nervous system (CNS AT/RT) and disease in this location carries an even worse prognosis with a median survival of 7 months.
  • In spite of multiple treatment regimens consisting of maximal surgical resection (including second look surgery), radiation therapy (focal and craniospinal), and multi-agent intravenous, oral and intrathecal chemotherapy, with or without high-dose therapy and stem cell rescue, only seven long-term survivors of CNS AT/RT have been reported, all in patients with newly diagnosed disease.
  • For this reason, many centers now direct such patients, particularly those under 5 years of age, or those with recurrent disease, towards comfort care rather than attempt curative therapy.
  • We now report on four children, two with newly diagnosed CNS AT/RT and two with progressive disease after multi-agent chemotherapy who are long term survivors (median follow-up of 37 months) using a combination of surgery, radiation therapy, and intensive chemotherapy.
  • The chemotherapy component was modified from the Intergroup Rhabdomyosarcoma Study Group (IRS III) parameningeal protocol as three of the seven reported survivors in the literature were treated using this type of therapy.
  • Our four patients, when added to the three reported survivors in the literature using this approach, suggest that patients provided this aggressive therapy can significantly alter the course of their disease.
  • More importantly, we report on the first two survivors after relapse with multi-agent intravenous and intrathecal chemotherapy treated with this modified regimen.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / therapy. Neoplasm Recurrence, Local / therapy. Rhabdoid Tumor / therapy. Teratoma / therapy
  • [MeSH-minor] Chemotherapy, Adjuvant / methods. Child. Child, Preschool. Female. Humans. Infant. Male. Radiotherapy, Adjuvant / methods. Remission Induction. Survival Analysis

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  • (PMID = 15803379.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Number-of-references] 37
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17. D'cunja J, Shalaby T, Rivera P, von Büren A, Patti R, Heppner FL, Arcaro A, Rorke-Adams LB, Phillips PC, Grotzer MA: Antisense treatment of IGF-IR induces apoptosis and enhances chemosensitivity in central nervous system atypical teratoid/rhabdoid tumours cells. Eur J Cancer; 2007 Jul;43(10):1581-9
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  • [Title] Antisense treatment of IGF-IR induces apoptosis and enhances chemosensitivity in central nervous system atypical teratoid/rhabdoid tumours cells.
  • Central nervous system (CNS) atypical teratoid/rhabdoid tumours (AT/RT) are among the paediatric malignant tumours with the worst prognosis and fatal outcome.
  • Insulin-like growth factor I receptor (IGF-IR) protects cancer cells from apoptosis induced by a variety of anticancer drugs and radiation.
  • Moreover, we found IGF-I and IGF-II mRNA in BT-16 CNS AT/RT cells and IGF-II mRNA in BT-12 CNS AT/RT cells, and autophosphorylated IGF-IR in both cell lines, indicating the potential presence of an autocrine/paracrine IGF-I/II/IGF-IR loop in CNS AT/RT.
  • IGF-IR antisense oligonucleotide treatment of human CNS AT/RT cells resulted in significant down-regulation of IGF-IR mRNA and protein expression, induction of apoptosis, and chemosensitisation to doxorubicin and cisplatin.
  • These studies provide evidence for the influence of IGF-IR on cellular responses to chemotherapy and raise the possibility that curability of selected CNS AT/RT may be improved by pharmaceutical strategies directed towards the IGF-IR.
  • [MeSH-major] Apoptosis / drug effects. Central Nervous System Neoplasms / drug therapy. Oligoribonucleotides, Antisense / therapeutic use. Receptor, IGF Type 1 / drug effects. Rhabdoid Tumor / drug therapy. Teratoma / drug therapy
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Child. Child, Preschool. Cisplatin / therapeutic use. Down-Regulation. Doxorubicin / therapeutic use. Female. Humans. Infant. Insulin-Like Growth Factor I / metabolism. Male


18. Phi JH, Kim SK, Park SH, Hong SH, Wang KC, Cho BK: Immature teratomas of the central nervous system: is adjuvant therapy mandatory? J Neurosurg; 2005 Dec;103(6 Suppl):524-30

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  • [Title] Immature teratomas of the central nervous system: is adjuvant therapy mandatory?
  • OBJECT: Immature teratomas of the central nervous system (CNS) are rare neoplasms.
  • Although adjuvant therapy is generally recommended after resection, the exact role of each therapeutic modality is not yet established.
  • The purpose of this study was to analyze the clinicopathological correlation and the role of resection to define the optimal treatment modalities for immature teratomas of the CNS.
  • METHODS: Between 1987 and 2002, eight patients underwent radical surgery for a lesion diagnosed as a CNS immature teratoma at the authors' institution.
  • Two patients received postoperative adjuvant therapies and two patients did not, against medical advice.
  • Another four patients, all of whom underwent GTR of the tumor, did not receive adjuvant therapy as part of a prospective treatment scheme.
  • CONCLUSIONS: Aggressive resection seems to be of utmost importance in the treatment of immature teratomas of the CNS.
  • Adjuvant chemotherapy and radiotherapy can be deferred if GTR is achieved in low-grade, immature teratomas, but adjuvant therapies may be warranted for high-grade ones.
  • [MeSH-major] Central Nervous System Neoplasms / surgery. Chemotherapy, Adjuvant. Neurosurgical Procedures. Radiotherapy, Adjuvant. Teratoma / surgery
  • [MeSH-minor] Child. Child, Preschool. Female. Follow-Up Studies. Humans. Infant. Infant, Newborn. Magnetic Resonance Imaging. Male. Neoplasm Recurrence, Local. Retrospective Studies. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 16383251.001).
  • [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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19. Nurzyńska-Flak J, Zawitkowska-Klaczyńska J, Katski K, Kowalczyk JR: [Central nervous system metastases in children with solid tumours]. Med Wieku Rozwoj; 2004 Apr-Jun;8(2 Pt 1):175-82
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  • [Title] [Central nervous system metastases in children with solid tumours].
  • BACKGROUND: Central nervous system (CNS) metastases occur in 20-30% of adult patients with systemic cancers. but they rarely occur in children with solid tumours.
  • AIM: clinical and prognostic characteristics of CNS recurrence in children treated for solid tumours were analysed.
  • The diagnosis in this group was as follow: soft tissue sarcomas -- 51 patients, bone tumours -- 50.
  • Wilms' tumour -- 48, neuroblastoma (NBL) -- 36, germ cell tumours -- 33: Children with primary CNS tumours, retinoblastoma, lymphoma and rare tumours were not analysed.
  • RESULTS: CNS metastases were diagnosed in five children (2.3%) - (2 boys.
  • Two of them were treated due to Wilms' tumour, one -- NBL, one -- teratoma malignum, one -- leiomyosarcoma.
  • None of the children with bone tumours had CNS metastases.
  • Diagnosis of CNS metastases was confirmed by imaging studies (CT, MRI).
  • The median time from initial diagnosis to the detection of CNS metastases was 14 months.
  • One of them was also irradiated and received chemotherapy and only this child is alive and achieved complete remission.
  • CONCLUSIONS: CNS metastases may occur in children with the recurrence of primary neoplastic disease.
  • [MeSH-major] Central Nervous System Neoplasms / secondary. Central Nervous System Neoplasms / therapy. Leiomyosarcoma / secondary. Neuroblastoma / secondary. Teratoma / secondary. Wilms Tumor / secondary

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  • (PMID = 15738591.001).
  • [Journal-full-title] Medycyna wieku rozwojowego
  • [ISO-abbreviation] Med Wieku Rozwoj
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
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20. Albert A, Cruz O, Montaner A, Vela A, Badosa J, Castañón M, Morales L: [Congenital solid tumors. A thirteen-year review]. Cir Pediatr; 2004 Jul;17(3):133-6
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  • This is an interesting group of tumors because their type, relative incidence, natural history and response to treatment differ from those seen in older children.
  • There were 8 teratomas (3 sacrocoxigeal, 1 retroperitoneal, 1 in the CNS, 1 orbitary and two oronasal), two hepatic tumors (1 hepatoblastoma, 1 hemangioendothelioma, two CNS tumors, two giant nevus (one on a hamartoma), and one each Wilms tumor, infantile fibrosarcoma and myofibroblastic tumor.
  • Treatment was surgical resection alone in 17 cases (68%) and surgery + chemotherapy in 8 (32%) (5 neuroblastomas, one CNS tumor, one Wilms tumor and one presacral teratoma who developed a yolk sac tumor); 3 patients died (11%): one at surgery, one of tumoural airway obstruction at birth and one with craniopharyngioma.
  • Among the 14 tumors that were initially not malignant, two can be locally agressive, one was an immature teratoma, the giant nevus with hamartoma developed in situ melanoma, the other nevus had meningeal melanosis with hydrocephalus, and one mature presacral teratoma developed a yolk sac tumor.
  • Their natural history is more benign than in other age groups, except for CNS tumors and very large or obstructing tumors.
  • Complete surgical excision is the treatment of choice, most cases not need adjuvant chemotherapy.
  • [MeSH-major] Central Nervous System Neoplasms / congenital. Kidney Neoplasms / congenital. Liver Neoplasms / congenital. Neuroblastoma / congenital. Skin Neoplasms / congenital. Soft Tissue Neoplasms / congenital. Teratoma / congenital. Wilms Tumor / congenital
  • [MeSH-minor] Female. Follow-Up Studies. Humans. Infant, Newborn. Male. Neoplasm Recurrence, Local. Postoperative Complications. Pregnancy. Prenatal Diagnosis. Time Factors

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  • (PMID = 15503950.001).
  • [ISSN] 0214-1221
  • [Journal-full-title] Cirugía pediátrica : organo oficial de la Sociedad Española de Cirugía Pediátrica
  • [ISO-abbreviation] Cir Pediatr
  • [Language] spa
  • [Publication-type] Comparative Study; English Abstract; Journal Article
  • [Publication-country] Spain
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21. Morgenstern DA, Gibson S, Brown T, Sebire NJ, Anderson J: Clinical and pathological features of paediatric malignant rhabdoid tumours. Pediatr Blood Cancer; 2010 Jan;54(1):29-34
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  • BACKGROUND: Malignant rhabdoid tumours (MRT) and their central nervous system (CNS) counterparts atypical teratoid/rhabdoid tumours (ATRT) are rare, highly aggressive malignant neoplasms of childhood.
  • Although there are isolated reports of long-term survival with intensive, multimodal therapy, outcomes are generally poor.
  • PROCEDURE: We conducted a retrospective review of all patients diagnosed with MRT/ATRT at Great Ormond Street Hospital over the 20 years from 1989 to 2009.
  • There were four long-term survivors (>30 months), all of whom received chemotherapy with or without surgical resection or radiotherapy.
  • CONCLUSIONS: In view of poor outcomes, there is a clear need for new treatment strategies and the identification of novel molecular targets for MRT/ATRT.
  • [MeSH-major] Rhabdoid Tumor / pathology. Teratoma / pathology
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biomarkers, Tumor / analysis. Child. Combined Modality Therapy. Female. Humans. Immunoenzyme Techniques. Infant. Male. Neoplasm Staging. Prognosis. Radiotherapy. Retrospective Studies. Survival Rate. Treatment Outcome

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  • [Copyright] Copyright 2009 Wiley-Liss, Inc.
  • (PMID = 19653294.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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22. Bambakidis NC, Robinson S, Cohen M, Cohen AR: Atypical teratoid/rhabdoid tumors of the central nervous system: clinical, radiographic and pathologic features. Pediatr Neurosurg; 2002 Aug;37(2):64-70
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  • [Title] Atypical teratoid/rhabdoid tumors of the central nervous system: clinical, radiographic and pathologic features.
  • INTRODUCTION: Atypical teratoid/rhabdoid tumors (ATT/RT) of the central nervous system (CNS) are uncommon malignancies of childhood with an aggressive course and a uniformly fatal outcome.
  • RESULTS: Eight children underwent surgery for CNS ATT/RT at our institution since 1996.
  • Four tumors had multifocal disease at the time of diagnosis.
  • Six patients received multiagent chemotherapy including 3 patients with autologous bone marrow transplantation, and 6 patients received radiation therapy.
  • Median survival was 9 months from the time of diagnosis.
  • CONCLUSIONS: In spite of aggressive therapy, the prognosis for ATT/RT remains dismal.
  • The search for effective treatment strategies will require a better understanding of the biology and molecular genetics of this tumor.
  • [MeSH-major] Central Nervous System Neoplasms / diagnostic imaging. Central Nervous System Neoplasms / pathology. Rhabdoid Tumor / diagnostic imaging. Rhabdoid Tumor / pathology. Teratoma / diagnostic imaging. Teratoma / pathology
  • [MeSH-minor] Adolescent. Child, Preschool. Female. Follow-Up Studies. Humans. Infant. Magnetic Resonance Imaging. Male. Neoplasm Staging. Retrospective Studies. Tomography, X-Ray Computed

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  • [Copyright] Copyright 2002 S. Karger AG, Basel
  • (PMID = 12145514.001).
  • [ISSN] 1016-2291
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
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23. Squire SE, Chan MD, Marcus KJ: Atypical teratoid/rhabdoid tumor: the controversy behind radiation therapy. J Neurooncol; 2007 Jan;81(1):97-111
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  • [Title] Atypical teratoid/rhabdoid tumor: the controversy behind radiation therapy.
  • To date, approximately 200 cases of atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system have been described in the literature.
  • This CNS tumor tends to present at an age of less than 3 years, and most patients succumb to their disease within 1 year of diagnosis.
  • However, lessons learned from regimens based upon medulloblastoma have revealed that AT/RT requires more aggressive treatment.
  • A significant portion of patients die of local recurrence in spite of aggressive surgery and chemotherapy.
  • As most patients with AT/RT present as infants or young children, radiation therapy has been a less than standard treatment option.
  • However, recent evidence suggests that long-term survival can occur with use of more aggressive treatment approaches including dose-intense chemotherapy as well as adjuvant radiation therapy.
  • A standardized and effective approach to treating this usually fatal tumor remains elusive, and the role of radiation therapy presents a particular dilemma as young patients with this disease may experience devastating late effects of therapy if they achieve a long-term survival.
  • Review of the literature reveals an association between initial radiation therapy and the ability to achieve a prolonged survival.
  • [MeSH-major] Central Nervous System Neoplasms / radiotherapy. Radiotherapy / methods. Rhabdoid Tumor / radiotherapy. Teratoma / radiotherapy
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Humans

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  • (PMID = 16855864.001).
  • [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
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 92
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24. Yang QY, Sun XF, Huang HQ, Zhen ZJ, Xia Y, Cai QQ, Ling JY: [Treatment outcome of primary central nervous system germ cell tumors after combined therapy: a report of 23 cases]. Ai Zheng; 2008 Apr;27(4):438-41
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  • [Title] [Treatment outcome of primary central nervous system germ cell tumors after combined therapy: a report of 23 cases].
  • BACKGROUND & OBJECTIVE: Primary central nervous system (CNS) germ cell tumors (GCTs) are rare malignant neoplasms with various histological types.
  • Excluding pure germinoma and mature teratoma, other types carry a poor prognosis.
  • Previous investigations focused on combined modality treatment including chemotherapy to improve survival.
  • This study was to analyze the efficacy and toxicity of chemotherapy combined with surgery and/or radiotherapy on CNS GCTs.
  • METHODS: A total of 23 patients with CNS GCTs were treated in Cancer Center of Sun Yat-sen University from May 2002 to Jun. 2006.
  • All patients were treated with chemotherapy of PEB regimen combined with surgery and/or radiotherapy.
  • RESULTS: Of the 23 patients, 17 newly diagnosed patients received induction chemotherapy followed by radiotherapy or surgery/radiotherapy followed by adjuvant chemotherapy; 6 recurrent patients received salvage chemotherapy, of which 3 patients with disseminated tumor received salvage chemotherapy followed by craniospinal irradiation.
  • Chemotherapy alone gained a response rate of 87.0% and a complete remission rate of 30.4%.
  • Fourteen patients (60.9%) were alive without evidence of diseases after combined modality treatment.
  • CONCLUSIONS: The combined modality treatment including PEB regimen is highly effective in treating CNS GCTs patients, especially in the patients with elevated tumor markers.
  • [MeSH-major] Central Nervous System Neoplasms / therapy. Neoplasms, Germ Cell and Embryonal / therapy
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bleomycin / therapeutic use. Cisplatin / therapeutic use. Combined Modality Therapy. Etoposide / therapeutic use. Female. Humans. Male. Survival Rate

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  • (PMID = 18423134.001).
  • [Journal-full-title] Ai zheng = Aizheng = Chinese journal of cancer
  • [ISO-abbreviation] Ai Zheng
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 11056-06-7 / Bleomycin; 6PLQ3CP4P3 / Etoposide; Q20Q21Q62J / Cisplatin; BEP protocol
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25. de León-Bojorge B, Rueda-Franco F, Anaya-Jara M: Central nervous system atypical teratoid rhabdoid tumor: experience at the National Institute of Pediatrics, Mexico City. Childs Nerv Syst; 2008 Mar;24(3):307-12
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Central nervous system atypical teratoid rhabdoid tumor: experience at the National Institute of Pediatrics, Mexico City.
  • OBJECTIVE: The purpose of this study is to present our experience with ten cases of Central nervous system atypical teratoid rhabdoid tumor (CNS/ATRT).
  • PATIENTS AND METHODS: A series of ten patients with CNS/ATRT, were diagnosed and treated between 1990 and 2005, at the National Institute of Pediatrics, in Mexico City.
  • The gender, age of presentation, clinical features, tumor localization, imaging studies, grade of tumor resection, complications, adjuvant therapy, and survival are presented.
  • There were two cases with longer survival (9 and 16 months), and their tumors were resected in total or subtotal manner and received adjuvant therapy (radiotherapy and chemotherapy).
  • CONCLUSIONS: Preliminary results, show that in older children, we can improve their survival with the subtotal or total resection of the tumor and the addition of chemotherapy and radiotherapy.
  • [MeSH-major] Central Nervous System Neoplasms / pathology. Infratentorial Neoplasms / pathology. Rhabdoid Tumor / pathology. Supratentorial Neoplasms / pathology. Teratoma / pathology
  • [MeSH-minor] Child. Child, Preschool. Female. Humans. Hydrocephalus / etiology. Hydrocephalus / pathology. Infant. Male. Retrospective Studies. Survival Analysis. Treatment Outcome

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  • (PMID = 17876589.001).
  • [ISSN] 0256-7040
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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26. Sakurada K, Saino M, Mouri W, Sato A, Kitanaka C, Kayama T: Nestin expression in central nervous system germ cell tumors. Neurosurg Rev; 2008 Apr;31(2):173-6; discussion 176-7

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Nestin expression in central nervous system germ cell tumors.
  • Central nervous system (CNS) germ cell tumors constitute a unique class of rare tumors that mainly affect children and adolescents.
  • Recently, results of treatment of germ cell tumors have improved with use of radiotherapy and combination chemotherapy.
  • However, some tumors have proven refractory to intensive treatment with surgery, radiation, and combination chemotherapy.
  • Nestin is an intermediate filament protein expressed in undifferentiated cells during CNS development and in CNS tumors and is used as a marker of immature elements of tumors, including brain tumor stem cells.
  • In this study, we examined for the first time nestin expression in 19 CNS germ cell tumors (nine pure germinomas, five germinomas with syncytiotrophoblastic giant cells, one yolk sac tumor, one choriocarcinoma, one embryonal carcinoma, and two mature teratomas).
  • These findings suggest that the detection of nestin expression could be useful in the management of CNS germ cell tumors, as an auxiliary predictor of dissemination and/or progression.
  • [MeSH-major] Biomarkers, Tumor / biosynthesis. Biomarkers, Tumor / genetics. Central Nervous System Neoplasms / genetics. Intermediate Filament Proteins / biosynthesis. Intermediate Filament Proteins / genetics. Neoplasms, Germ Cell and Embryonal / genetics. Nerve Tissue Proteins / biosynthesis. Nerve Tissue Proteins / genetics
  • [MeSH-minor] Adolescent. Adult. Child. Choriocarcinoma / genetics. Choriocarcinoma / metabolism. Choriocarcinoma / pathology. Endodermal Sinus Tumor / genetics. Endodermal Sinus Tumor / metabolism. Endodermal Sinus Tumor / pathology. Female. Germinoma / genetics. Germinoma / metabolism. Germinoma / pathology. Giant Cell Tumors / genetics. Giant Cell Tumors / metabolism. Giant Cell Tumors / pathology. Humans. Hypopituitarism / etiology. Immunoenzyme Techniques. Magnetic Resonance Imaging. Male. Nestin. Teratoma / genetics. Teratoma / metabolism. Teratoma / pathology. Vision Disorders / etiology

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  • (PMID = 18092184.001).
  • [ISSN] 0344-5607
  • [Journal-full-title] Neurosurgical review
  • [ISO-abbreviation] Neurosurg Rev
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Intermediate Filament Proteins; 0 / NES protein, human; 0 / Nerve Tissue Proteins; 0 / Nestin
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27. Packer RJ, Biegel JA, Blaney S, Finlay J, Geyer JR, Heideman R, Hilden J, Janss AJ, Kun L, Vezina G, Rorke LB, Smith M: Atypical teratoid/rhabdoid tumor of the central nervous system: report on workshop. J Pediatr Hematol Oncol; 2002 Jun-Jul;24(5):337-42
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Atypical teratoid/rhabdoid tumor of the central nervous system: report on workshop.
  • Childhood atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system (CNS) is a recently described entity.
  • The tumor's incidence is still undefined, but it may comprise as high as 1 in 4 primitive CNS tumors in infants.
  • Treatment is far from optimal, but there are occasional long-term survivors, especially among older children.
  • Therapeutic approached have included surgery, chemotherapy, and radiotherapy.
  • [MeSH-major] Brain Neoplasms / pathology. Rhabdoid Tumor / pathology. Teratoma / pathology
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Child, Preschool. Chromosomal Proteins, Non-Histone. Chromosome Deletion. Chromosomes, Human, Pair 22 / genetics. DNA-Binding Proteins / genetics. General Surgery. Humans. Infant. Monosomy. Neoplasm Proteins / genetics. Radiotherapy. Transcription Factors

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  • (PMID = 12142780.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Congresses; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / Neoplasm Proteins; 0 / SMARCB1 protein, human; 0 / Transcription Factors
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28. Hilden JM, Meerbaum S, Burger P, Finlay J, Janss A, Scheithauer BW, Walter AW, Rorke LB, Biegel JA: Central nervous system atypical teratoid/rhabdoid tumor: results of therapy in children enrolled in a registry. J Clin Oncol; 2004 Jul 15;22(14):2877-84
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Central nervous system atypical teratoid/rhabdoid tumor: results of therapy in children enrolled in a registry.
  • PURPOSE: Atypical teratoid/rhabdoid tumor (AT/RT) of the CNS is an extremely rare and aggressive tumor of early childhood.
  • The poor outcome with conventional infant brain tumor therapy has resulted in a lack of clear treatment guidelines.
  • Primary therapy included chemotherapy in all patients, radiotherapy in 13 patients (31%), stem-cell rescue in 13 patients (31%), and intrathecal chemotherapy in 16 patients (38%).
  • CONCLUSION: Aggressive therapy has prolonged the natural history in a subset of children.
  • [MeSH-major] Central Nervous System Neoplasms / therapy. Registries. Rhabdoid Tumor / therapy. Teratoma / therapy
  • [MeSH-minor] Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Survival Analysis. Treatment Outcome

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  • (PMID = 15254056.001).
  • [ISSN] 0732-183X
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 46274
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
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29. Perek D, Brozyna A, Dembowska-Baginska B, Stypinska M, Sojka M, Bacewicz L, Polnik D, Kalicinski P: [Tumours in newborns and infants up to three months of life. One institution experience]. Med Wieku Rozwoj; 2006 Jul-Sep;10(3 Pt 1):711-23
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  • INTRODUCTION: Newborns and infants up to three months of life are a specific group of population in paediatric oncology due to immaturity of tissues and organs and rarity of neoplastic diseases in this group of patients (pts).
  • There are no strict therapeutic procedures established for these children.
  • THE AIM of our study was to examine distribution of tumours in newborns and infants up to 3 months of age treated in our institution and to present our own experience in the treatment of these patients.
  • Distribution of tumour types in newborns and babies from 1 to 3 months of age was analyzed separately.
  • Due to similar growth pattern, response to treatment and it's side effects in newborns and small infants, treatment results were evaluated for the whole group.
  • There were also 3 cases of soft tissue sarcomas (STS), 2 central nervous system tumours (CNS), 2 retinoblastoma (RB), 2 hepatoblastoma (HB).
  • In the group of 21 babies aged 1-3 months NBL was the commonest (37%) followed by RB, CNS tumours (14% of each) HB and MT (10% of each) and Wilms tumour (WT) and immature teratoma (IT) each 5%.
  • It concerned pts with MT--28, IT--3 pts, yolk sac tumour (YST)--1 pt and malignant tumours (stage I and II): 8-NBL, 2-CNS tumours, 2 STS, 3-HB, 1-WT.
  • Eleven pts underwent combined treatment of chemotherapy and surgery: 5 with stage III and IV NBL, 6 with other tumours.
  • Chemotherapy alone was administered to 7 pts in whom local advancement of disease enabled surgery and to pts with RBL.
  • One pt, critically ill, died before any treatment.
  • 2. Newborns and small infants with advanced neoplastic disease, similarly to older children can be cured with chemotherapy.
  • 3. Individual approach is warranted in newborns and small infants and treatment should be carried out in specialized centres.
  • 4. All patients who completed treatment of any tumour type should be followed up by a pediatric oncologist.
  • [MeSH-major] Infant Welfare / statistics & numerical data. Neoplasms / epidemiology. Neoplasms / therapy

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  • (PMID = 17317902.001).
  • [Journal-full-title] Medycyna wieku rozwojowego
  • [ISO-abbreviation] Med Wieku Rozwoj
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
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30. Uzzaman M, Benveniste RJ, Keller G, Germano IM: Embryonic stem cell-derived astrocytes: a novel gene therapy vector for brain tumors. Neurosurg Focus; 2005 Sep 15;19(3):E6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Embryonic stem cell-derived astrocytes: a novel gene therapy vector for brain tumors.
  • OBJECT: For gene therapy strategies currently in clinical trials, viral vectors are used to deliver transgenes directly to normal and tumor cells within the central nervous system (CNS).
  • The aim of this study was to assess whether embryonic stem cell (ESC)-derived astrocytes expressing a doxycyclineinducible transgene can be used as a vector for gene therapy.
  • METHODS: The authors generated a pure population of ESC-derived astrocytes carrying a transgene, tumor necrosis factor-related apoptosis-inducing ligand (TRAIL), inserted in the chromosome under the control of a highly regulated doxycycline-inducible expression system.
  • RESULTS: The ESC-derived astrocytes started to migrate from the transplant site 48 hours after the procedure.
  • They were found to have migrated throughout the brain tissue by 6 weeks.
  • Teratoma formation was not observed in long-term experiments (12 weeks).
  • CONCLUSIONS: These data show that ESC-derived astrocytes can be used as delivery vectors for CNS tumors.
  • This technique might have a major impact on the treatment of patients with malignant gliomas and a wide spectrum of other neurological diseases.
  • [MeSH-major] Astrocytes / physiology. Brain Neoplasms / therapy. Genetic Therapy / methods. Stem Cells / physiology
  • [MeSH-minor] Animals. Apoptosis Regulatory Proteins / metabolism. Blotting, Northern / methods. Brain / metabolism. Brain / pathology. Cell Line. Cell Movement / physiology. Doxycycline / pharmacology. Embryo, Mammalian. Female. Gene Expression / drug effects. Gene Expression / physiology. Gene Transfer Techniques. Genetic Vectors / physiology. Immunohistochemistry / methods. Membrane Glycoproteins / metabolism. Mice. RNA, Messenger / biosynthesis. Reverse Transcriptase Polymerase Chain Reaction / methods. TNF-Related Apoptosis-Inducing Ligand. Time Factors. Tumor Necrosis Factor-alpha / metabolism

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  • (PMID = 16190605.001).
  • [ISSN] 1092-0684
  • [Journal-full-title] Neurosurgical focus
  • [ISO-abbreviation] Neurosurg Focus
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Apoptosis Regulatory Proteins; 0 / Membrane Glycoproteins; 0 / RNA, Messenger; 0 / TNF-Related Apoptosis-Inducing Ligand; 0 / Tnfsf10 protein, mouse; 0 / Tumor Necrosis Factor-alpha; N12000U13O / Doxycycline
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