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1. Bisson E, Florman J, Wald S, Tranmer B: Primitive neuroectodermal tumor arising in long-standing cerebellar atrophy. Pediatr Neurosurg; 2003 Feb;38(2):76-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primitive neuroectodermal tumor arising in long-standing cerebellar atrophy.
  • Primitive neuroectodermal tumors (PNETs) account for one fifth of childhood brain tumors.
  • Although little is known of the pathobiology of this tumor type, there are associations with both genetic syndromes and exposures to specific environmental agents.
  • Progressive cerebellar atrophy predating the presentation of a primary brain tumor, in the absence of a genetic syndrome, has not been reported with PNETs.
  • We report a case of a posterior fossa PNET occurring in association with long-standing cerebellar atrophy without evidence of a genetic syndrome.
  • This case may represent an unrecognized paraneoplastic syndrome or a unique subtype of PNET.
  • [MeSH-minor] Adolescent. Atrophy. Cerebellar Ataxia / diagnosis. Cerebellar Ataxia / pathology. Cerebellar Ataxia / surgery. Child. Child, Preschool. Combined Modality Therapy. Cranial Irradiation. Dominance, Cerebral / physiology. Female. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Neurologic Examination. Paraneoplastic Syndromes / diagnosis. Paraneoplastic Syndromes / pathology. Paraneoplastic Syndromes / radiotherapy. Paraneoplastic Syndromes / surgery. Radiotherapy, Adjuvant

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  • [Copyright] Copyright 2003 S. Karger AG, Basel
  • (PMID = 12566839.001).
  • [ISSN] 1016-2291
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
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2. Vassal G, Tranchand B, Valteau-Couanet D, Mahé C, Couanet D, Schoeppfer C, Grill J, Kalifa C, Hill C, Ardiet C, Hartmann O: Pharmacodynamics of tandem high-dose melphalan with peripheral blood stem cell transplantation in children with neuroblastoma and medulloblastoma. Bone Marrow Transplant; 2001 Mar;27(5):471-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • The aim of this study was to evaluate the tolerance, pharmacokinetics (PK) and pharmacodynamics (PD) of HD single-agent melphalan administered over two consecutive courses (C1 and C2) in children.
  • Five had metastatic neuroblastoma (NB) and 16 a cerebral primitive neuroectodermal tumour (PNET).
  • Partial remission was observed in 11/14 patients with measurable cerebral PNET.
  • In conclusion, tandem HD melphalan is feasible and safe in children, and achieved a high response rate in cerebral PNET.
  • [MeSH-major] Antineoplastic Agents, Alkylating / administration & dosage. Cerebellar Neoplasms / therapy. Hematopoietic Stem Cell Transplantation / methods. Medulloblastoma / therapy. Melphalan / administration & dosage. Melphalan / pharmacology. Neuroblastoma / therapy. Transplantation Conditioning
  • [MeSH-minor] Adolescent. Area Under Curve. Child. Child, Preschool. Dose-Response Relationship, Drug. Drug Administration Schedule. Female. Humans. Infant. Male. Metabolic Clearance Rate


3. Reddy AT, Janss AJ, Phillips PC, Weiss HL, Packer RJ: Outcome for children with supratentorial primitive neuroectodermal tumors treated with surgery, radiation, and chemotherapy. Cancer; 2000 May 1;88(9):2189-93
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Outcome for children with supratentorial primitive neuroectodermal tumors treated with surgery, radiation, and chemotherapy.
  • BACKGROUND: The outcome of a child with a primitive neuroectodermal tumors arising supratentorially (SPNET) is not well characterized and may differ from the outcome of a patient with a histologically similar cerebellar tumor (medulloblastoma [MB]).
  • Tumor location included was 13 pineal, 6 cortical, and 3 thalamic or suprasellar.
  • There was no statistical association between tumor location and survival.
  • This suggests that there may be biologic differences between supratentorial and infratentorial primitive neuroectodermal tumors, thus requiring refinements in treatment.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cranial Irradiation. Neuroectodermal Tumors, Primitive / surgery. Supratentorial Neoplasms / surgery
  • [MeSH-minor] Adolescent. Antineoplastic Agents / administration & dosage. Antineoplastic Agents, Alkylating / administration & dosage. Antineoplastic Agents, Phytogenic / administration & dosage. Cerebral Cortex / drug effects. Cerebral Cortex / radiation effects. Cerebral Cortex / surgery. Child. Child, Preschool. Cisplatin / administration & dosage. Disease Progression. Disease-Free Survival. Follow-Up Studies. Humans. Linear Models. Lomustine / administration & dosage. Neoplasm Staging. Pinealoma / drug therapy. Pinealoma / radiotherapy. Pinealoma / surgery. Retrospective Studies. Survival Rate. Thalamic Diseases / drug therapy. Thalamic Diseases / radiotherapy. Thalamic Diseases / surgery. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 10813733.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Antineoplastic Agents, Alkylating; 0 / Antineoplastic Agents, Phytogenic; 5J49Q6B70F / Vincristine; 7BRF0Z81KG / Lomustine; Q20Q21Q62J / Cisplatin
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4. De Sio L, Milano GM, Castellano A, Jenkner A, Fidani P, Dominici C, Donfrancesco A: Temozolomide in resistant or relapsed pediatric solid tumors. Pediatr Blood Cancer; 2006 Jul;47(1):30-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • PURPOSE: We report the off-label study aimed at investigating the use of temozolomide (TMZ) as single agent in relapsed or resistant pediatric solid tumors.
  • The drug was administered at the dose of 215 mg/m2/day x 5 days or 180 mg/m2/day x 5 days in patients with prior craniospinal irradiation (CSI) or autologous bone marrow transplantation (ABMT).
  • Tumor types were: neuroblastoma (NB; n = 17), medulloblastoma (MB; 8), brain stem glioma (BSG; 8), extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor (EOES; 4), Ewing's sarcoma (ES; 4), anaplastic astrocytoma (AA; 3), rhabdomyosarcoma (RMS; 2), ependymoma (EP; 2), cerebral primitive neuroectodermal tumor (cPNET; 2), hepatocarcinoma (HC; 1), and osteosarcoma (OS; 1).
  • RESULTS: Objective response-rate (CR + PR + MR) in our series was 13.4% (1.9% CR, 3.8% PR, and 7.7% MR), SD occurred in 38.4% of patients and 48% had PD.
  • [MeSH-major] Antineoplastic Agents, Alkylating / therapeutic use. Dacarbazine / analogs & derivatives. Drug Resistance, Neoplasm. Neoplasm Recurrence, Local / drug therapy. Neoplasms / drug therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Disease-Free Survival. Dose-Response Relationship, Drug. Female. Humans. Male. Survival Analysis

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  • [Copyright] Copyright 2006 Wiley-Liss, Inc.
  • [ErratumIn] Pediatr Blood Cancer. 2006 Oct 15;47(5):647-8
  • (PMID = 16047361.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide
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5. Chang Q, Ng HK: [Different hypermethylation status of RASSF1A in medulloblastoma and supratentorial primitive neuroectodermal tumor]. Zhonghua Bing Li Xue Za Zhi; 2007 Jan;36(1):24-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Different hypermethylation status of RASSF1A in medulloblastoma and supratentorial primitive neuroectodermal tumor].
  • OBJECTIVE: To investigate the epigenetic involvement of RASSF1A in intracranial primitive neuroectodermal tumors (PNETs) and compare the methylation patterns between medulloblastoma (MBs) and supratentorial PNETs (SPNETs).
  • RASSF1A-deficient PNET cell lines were treated with 5-aza-2'deoxycytidine, a demethylating agent, to explore the relationship between hypermethylation and the gene expression.
  • RESULTS: The results revealed no promoter hypermethylation of RASSF1A in 2 normal cerebellar and 5 normal cerebral tissue specimens examined.
  • In contrast, promoter hypermethylation of RASSF1A was detected in 100% (25/25) of primary MBs, 6/9 of primary SPNETs, and all PNET cell lines.
  • These results demonstrated that such epigenetic alteration was tumor-specific.
  • Treatment of RASSF1A-deficient PNET cell lines with 5-aza-2'deoxycytidine restored RASSF1A expression, providing evidence that promoter hypermethylation contributes to transcriptional silencing.
  • Different hypermethylation status of RASSF1A are found in PNET subtypes suggesting that MBs and SPNETs are epigenetical distinct tumors.
  • [MeSH-major] Brain Neoplasms / genetics. Cerebellar Neoplasms / genetics. Medulloblastoma / genetics. Neuroectodermal Tumors, Primitive / genetics. Tumor Suppressor Proteins / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Cell Line, Tumor. Child. Child, Preschool. DNA Methylation. Female. Gene Expression Regulation, Neoplastic. Gene Silencing. HeLa Cells. Humans. Infant. Male. Promoter Regions, Genetic / genetics. Reverse Transcriptase Polymerase Chain Reaction. Young Adult


6. Spreafico F, Gandola L, Marchianò A, Simonetti F, Poggi G, Adduci A, Clerici CA, Luksch R, Biassoni V, Meazza C, Catania S, Terenziani M, Musumeci R, Fossati-Bellani F, Massimino M: Brain magnetic resonance imaging after high-dose chemotherapy and radiotherapy for childhood brain tumors. Int J Radiat Oncol Biol Phys; 2008 Mar 15;70(4):1011-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • RESULTS: Of the 49 children, 18 (6 of 25 with high-grade gliomas and 12 of 24 with primitive neuroectodermal tumors) had abnormal brain MRI findings occurring a median of 8 months (range, 2-39 months) after RT and beginning to regress a median of 13 months (range, 2-26 months) after onset.
  • Four patients with primitive neuroectodermal tumors also had subdural fluid leaks, with meningeal enhancement over the effusion.
  • CONCLUSION: Multiple enhancing cerebral lesions were frequently seen on MRI scans soon after high-dose chemotherapy and RT.
  • Such findings pose a major diagnostic challenge in terms of their differential diagnosis vis-à-vis recurrent tumor.
  • [MeSH-major] Antineoplastic Agents, Alkylating / adverse effects. Brain. Brain Neoplasms. Glioma. Neuroectodermal Tumors, Primitive. Thiotepa / adverse effects
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Cognition / drug effects. Cognition / radiation effects. Combined Modality Therapy / adverse effects. Combined Modality Therapy / methods. Disease-Free Survival. Female. Humans. Infant. Intelligence / drug effects. Intelligence / radiation effects. Magnetic Resonance Imaging. Male. Medulloblastoma / drug therapy. Medulloblastoma / radiotherapy

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  • (PMID = 17904307.001).
  • [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
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 905Z5W3GKH / Thiotepa
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7. Oruckaptan HH, Ozisik P, Akalan N: Prolonged cerebral salt wasting syndrome associated with the intraventricular dissemination of brain tumors. Report of two cases and review of the literature. Pediatr Neurosurg; 2000 Jul;33(1):16-20
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Prolonged cerebral salt wasting syndrome associated with the intraventricular dissemination of brain tumors. Report of two cases and review of the literature.
  • The two common clinical manifestations are the inappropriate secretion of antidiuretic hormone (SIADH) and the cerebral salt wasting syndrome (CSWS), which were usually attributed to each other due to identical clinical presentation.
  • In this article, we report on 2 cases of a primitive neuroectodermal tumor with prolonged CSWS manifested during the intraventricular dissemination of primary disease and the high catabolic stage.
  • [MeSH-major] Brain Diseases, Metabolic / diagnosis. Brain Diseases, Metabolic / metabolism. Brain Neoplasms / diagnosis. Inappropriate ADH Syndrome / diagnosis. Inappropriate ADH Syndrome / metabolism. Neuroectodermal Tumors, Primitive / diagnosis. Sodium Chloride / metabolism
  • [MeSH-minor] Child, Preschool. Disease Progression. Humans. Hydrocephalus / complications. Hydrocephalus / diagnosis. Hyponatremia / drug therapy. Hyponatremia / etiology. Hyponatremia / metabolism. Infant. Magnetic Resonance Imaging. Male. Neoplasm Invasiveness. Time Factors

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  • [Copyright] Copyright 2000 S. Karger AG, Basel.
  • (PMID = 11025417.001).
  • [ISSN] 1016-2291
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 451W47IQ8X / Sodium Chloride
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8. Chang Q, Pang JC, Li KK, Poon WS, Zhou L, Ng HK: Promoter hypermethylation profile of RASSF1A, FHIT, and sFRP1 in intracranial primitive neuroectodermal tumors. Hum Pathol; 2005 Dec;36(12):1265-72
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Promoter hypermethylation profile of RASSF1A, FHIT, and sFRP1 in intracranial primitive neuroectodermal tumors.
  • Medulloblastomas (MBs) and supratentorial primitive neuroectodermal tumors (SPNETs) are histologically alike intracranial PNETs found in different anatomical locations of the brain.
  • The aim of this study was to investigate whether promoter hypermethylation of putative tumor suppressor genes was involved in both types of intracranial PNETs.
  • Our results revealed no promoter hypermethylation of RASSF1A, FHIT, and sFRP1 in 2 normal cerebellar and 5 normal cerebral tissue specimens examined.
  • In contrast, promoter hypermethylation of RASSF1A was detected in 100% of primary MBs, 67% (6/9) of primary SPNETs, and all PNET cell lines.
  • Treatment of RASSF1A-deficient PNET cell lines with 5-aza-2'deoxycytidine, a demethylating agent, restored RASSF1A expression, providing evidence that promoter hypermethylation contributes to transcriptional silencing.
  • [MeSH-major] Acid Anhydride Hydrolases / genetics. Brain Neoplasms / genetics. Cell Cycle Proteins / genetics. DNA Methylation. Neoplasm Proteins / genetics. Neuroectodermal Tumors, Primitive / genetics. Protein-Serine-Threonine Kinases / genetics. Tumor Suppressor Proteins / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Ataxia Telangiectasia Mutated Proteins. Cell Line, Tumor. Child. Child, Preschool. DNA Primers / chemistry. DNA, Neoplasm / analysis. Female. Humans. Infant. Male. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 16311119.001).
  • [ISSN] 0046-8177
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Cell Cycle Proteins; 0 / DNA Primers; 0 / DNA, Neoplasm; 0 / Neoplasm Proteins; 0 / RASSF1 protein, human; 0 / Tumor Suppressor Proteins; 0 / fragile histidine triad protein; EC 2.7.11.1 / ATR protein, human; EC 2.7.11.1 / Ataxia Telangiectasia Mutated Proteins; EC 2.7.11.1 / Protein-Serine-Threonine Kinases; EC 3.6.- / Acid Anhydride Hydrolases
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