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1. Frühwald MC, Rickert CH, O'Dorisio MS, Madsen M, Warmuth-Metz M, Khanna G, Paulus W, Kühl J, Jürgens H, Schneider P, Müller HL: Somatostatin receptor subtype 2 is expressed by supratentorial primitive neuroectodermal tumors of childhood and can be targeted for somatostatin receptor imaging. Clin Cancer Res; 2004 May 1;10(9):2997-3006
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  • [Title] Somatostatin receptor subtype 2 is expressed by supratentorial primitive neuroectodermal tumors of childhood and can be targeted for somatostatin receptor imaging.
  • PURPOSE: Although gliomas predominate among central nervous system (CNS) neoplasms in adulthood, embryonal tumors are the most common malignant brain tumors in children.
  • Despite novel treatment approaches, including improved radiotherapy and high-dose chemotherapy, survival rates remain unsatisfactory.
  • The timely diagnosis of residual or recurrent embryonal CNS tumors and thus the earliest possible time point for intervention is often hampered by inaccuracies of conventional imaging techniques.
  • Here, we evaluated somatostatin receptor type 2 (sst(2)) expression using an antibody in an array of CNS tumors of childhood.
  • Eight high-grade gliomas, 4 atypical teratoid/rhabdoid tumors, 7 supratentorial primitive neuroectodermal tumors (stPNET), 1 medulloepithelioma (ME), and 8 ependymomas were screened.
  • Tumors positive in vitro were additionally analyzed in vivo using SRI.
  • RESULTS: Abundant expression of somatostatin receptor type 2 in stPNET, a ME, and ependymomas warranted in vivo imaging of 7 stPNET, 1 rhabdomyosarcoma, 3 ependymomas, 1 ME, and 1 glioblastoma.
  • In selected cases SRI was more sensitive in the detection of relapse than conventional imaging by magnetic resonance imaging and computed tomography.
  • CONCLUSIONS: SRI should be considered in the evaluation of residual or recurrent embryonal CNS tumors, especially stPNET.
  • The strengths of SRI lie in the differentiation of reactive tissue changes versus residual or recurrent tumor, the detection of small lesions, and possibly in the distinction of stPNET from gliomas.
  • [MeSH-major] Neuroectodermal Tumors, Primitive / pathology. Receptors, Somatostatin / biosynthesis. Supratentorial Neoplasms / pathology
  • [MeSH-minor] Central Nervous System Neoplasms / metabolism. Central Nervous System Neoplasms / pathology. Central Nervous System Neoplasms / radionuclide imaging. Child. Child, Preschool. Female. Humans. Immunohistochemistry. Infant. Male. Tomography, Emission-Computed, Single-Photon / methods

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  • (PMID = 15131035.001).
  • [ISSN] 1078-0432
  • [Journal-full-title] Clinical cancer research : an official journal of the American Association for Cancer Research
  • [ISO-abbreviation] Clin. Cancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Receptors, Somatostatin; 0 / somatostatin receptor 2
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2. Fitzhugh CD, Wise B, Baird K, Tsokos M, Helman L, Mackall C, Savage SA, Warren KE: Secondary supratentorial primitive neuroectodermal tumor following treatment of childhood osteosarcoma. Pediatr Blood Cancer; 2009 Sep;53(3):496-8
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  • [Title] Secondary supratentorial primitive neuroectodermal tumor following treatment of childhood osteosarcoma.
  • A 16-year-old Caucasian male was diagnosed with a primitive neuroectodermal tumor (PNET) 5 years following the diagnosis of nonmetastatic osteosarcoma of the left proximal humerus.
  • The patient was initially treated with standard chemotherapy and limb salvage resection for osteosarcoma.
  • Nine months after the completion of therapy, he developed lung metastases for which he underwent surgical resection and received additional chemotherapy.
  • Almost 5 years after the osteosarcoma diagnosis, the patient was diagnosed with a supratentorial PNET, which represents the first known case reported in a patient with osteosarcoma.
  • [MeSH-major] Bone Neoplasms / drug therapy. Neoplasms, Second Primary / etiology. Neuroectodermal Tumors, Primitive / etiology. Osteosarcoma / drug therapy. Supratentorial Neoplasms / etiology

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  • [Copyright] Published 2009 Wiley-Liss, Inc.
  • [Cites] Neuropathology. 2001 Mar;21(1):40-4 [11304041.001]
  • [Cites] J Clin Oncol. 2001 Jul 1;19(13):3163-72 [11432882.001]
  • [Cites] Am J Med Genet. 2001 Jul 22;102(1):11-7 [11471165.001]
  • [Cites] Blood Cells Mol Dis. 2001 May-Jun;27(3):662-6 [11482881.001]
  • [Cites] Eur J Hum Genet. 2003 Aug;11(8):611-8 [12891382.001]
  • [Cites] Ann Intern Med. 1969 Oct;71(4):747-52 [5360287.001]
  • [Cites] Cancer Res. 1988 Sep 15;48(18):5358-62 [3409256.001]
  • [Cites] Br J Cancer. 1991 Jun;63(6):993-9 [2069856.001]
  • [Cites] Diagn Mol Pathol. 2007 Jun;16(2):108-11 [17525681.001]
  • [Cites] J Pediatr Hematol Oncol. 2006 Dec;28(12):774-80 [17164644.001]
  • [Cites] J Natl Cancer Inst. 2006 Nov 1;98(21):1528-37 [17077355.001]
  • [Cites] N Engl J Med. 2006 Oct 12;355(15):1572-82 [17035650.001]
  • [Cites] J Pediatr Hematol Oncol. 2006 Jun;28(6):374-8 [16794506.001]
  • [Cites] Pediatr Hematol Oncol. 2005 Mar;22(2):89-101 [15804994.001]
  • [Cites] Br J Cancer. 1997;76(1):1-14 [9218725.001]
  • [Cites] Am J Pathol. 1997 Jan;150(1):1-13 [9006316.001]
  • [Cites] Cancer Epidemiol Biomarkers Prev. 1996 Apr;5(4):239-46 [8722214.001]
  • [Cites] Diagn Mol Pathol. 1993 Sep;2(3):141-6 [8287228.001]
  • (PMID = 19434734.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z01 BC010709-02
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS124011; NLM/ PMC2760453
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3. 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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  • [Title] 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.
  • BACKGROUND: An open-label Phase II study of oxaliplatin was conducted to evaluate its safety and efficacy in children with recurrent or refractory medulloblastoma (MB), supratentorial primitive neuroectodermal tumors (SPNET), and atypical teratoid rhabdoid tumor (ATRT).
  • 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


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4. Ulbright TM, Hattab EM, Zhang S, Ehrlich Y, Foster RS, Einhorn LH, Cheng L: Primitive neuroectodermal tumors in patients with testicular germ cell tumors usually resemble pediatric-type central nervous system embryonal neoplasms and lack chromosome 22 rearrangements. Mod Pathol; 2010 Jul;23(7):972-80
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  • [Title] Primitive neuroectodermal tumors in patients with testicular germ cell tumors usually resemble pediatric-type central nervous system embryonal neoplasms and lack chromosome 22 rearrangements.
  • Primitive neuroectodermal tumors (PNETs) are one of the most frequent types of 'non-germ cell' tumor in patients with testicular germ cell tumors and have a guarded prognosis when present in metastatic sites after cisplatin-based chemotherapy.
  • Improved treatments, including targeted therapy, require understanding the biology of these neoplasms.
  • We therefore analyzed the morphologic, immunohistochemical and molecular biologic features of 14 PNETs from 14 patients with concurrent or previous testicular germ cell tumors; 12 tumors were from metastatic sites and 2 were primary in the testis.
  • Using standard light microscopic criteria for central nervous system and peripheral PNETs, we classified nine tumors as medulloepithelioma, three as medulloblastoma/supratentorial PNET, one as neuroblastic tumor with abundant neuropil and true rosettes and one as small cell embryonal tumor/PNET (Ewing sarcoma-like).
  • INI1 was diffusely and strongly positive in all tumors whereas the other stains, except for cytoplasmic WT1 (which showed substantial reactivity in most tumors), were mostly focal to negative, including CD99 (eight negative, six focal) and Fli-1 (all negative).
  • Only 1 tumor, classified as medulloepithelioma, was scored positive for chromosome 22 translocation (22% rearranged cells) and the remaining 13 were negative, including the one case that resembled peripheral PNET.
  • We conclude that PNETs derived from testicular germ cell tumors mostly resemble central nervous system PNETs and generally lack the chromosome 22 translocation of peripheral PNETs.
  • Future treatment strategies should take these findings into account.
  • [MeSH-major] Neoplasms, Germ Cell and Embryonal / pathology. Neoplasms, Multiple Primary / pathology. Neuroectodermal Tumors, Primitive, Peripheral / pathology. Testicular Neoplasms / pathology
  • [MeSH-minor] Adult. Biomarkers, Tumor / analysis. Chromosomes, Human, Pair 22 / genetics. Gene Rearrangement. Humans. Immunohistochemistry. In Situ Hybridization, Fluorescence. Male. Young Adult

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  • (PMID = 20348883.001).
  • [ISSN] 1530-0285
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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5. Jennings MT, Cmelak A, Johnson MD, Moots PL, Pais R, Shyr Y: Differential responsiveness among "high risk" pediatric brain tumors in a pilot study of dose-intensive induction chemotherapy. Pediatr Blood Cancer; 2004 Jul;43(1):46-54
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  • [Title] Differential responsiveness among "high risk" pediatric brain tumors in a pilot study of dose-intensive induction chemotherapy.
  • BACKGROUND: These factors have been predictive for progressive disease on therapy (PDOT) among pediatric brain tumors: >1.5 cm(2) unresectable tumor, glioblastoma, supratentorial primitive neuroectodermal tumor, and metastatic medulloblastoma (MBL).
  • Maintenance chemotherapy consisted of eight cycles of carboplatin, etoposide, and vincristine.
  • RESULTS: Twenty newly diagnosed patients [nine primitive neuroectodermal tumors/MBL, one choroid plexus carcinoma, eight malignant gliomas, and two anaplastic ependymomas] were treated.
  • Ten patients, who required neuraxis irradiation, constituted the "PNET" group.
  • Induction chemotherapy produced partial and minor responses (MRs) among 5/10.
  • Their estimated median progression free survival was 6.9 months (P = 0.035 relative to the PNET) with a median survival of 10.7 months (P = 0.04).
  • CONCLUSIONS: This induction regimen produced a cytoreductive response in 6/10 and achieved a significant improvement in progression free survival among 7/10 in the PNET group.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Glioma / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Cisplatin / administration & dosage. Cyclophosphamide / administration & dosage. Disease-Free Survival. Dose Fractionation. Etoposide / administration & dosage. Female. Humans. Male. Neoadjuvant Therapy. Pilot Projects. Statistics, Nonparametric. Survival Rate. Vincristine / administration & dosage

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  • [Copyright] Copyright 2004 Wiley-Liss, Inc.
  • (PMID = 15170889.001).
  • [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] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; Q20Q21Q62J / Cisplatin
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6. Sung KW, Yoo KH, Cho EJ, Koo HH, Lim DH, Shin HJ, Ahn SD, Ra YS, Choi ES, Ghim TT: High-dose chemotherapy and autologous stem cell rescue in children with newly diagnosed high-risk or relapsed medulloblastoma or supratentorial primitive neuroectodermal tumor. Pediatr Blood Cancer; 2007 Apr;48(4):408-15
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  • [Title] High-dose chemotherapy and autologous stem cell rescue in children with newly diagnosed high-risk or relapsed medulloblastoma or supratentorial primitive neuroectodermal tumor.
  • BACKGROUND: Single or tandem double high-dose chemotherapy (HDCT) was used to treat children with newly diagnosed high-risk or relapsed medulloblastoma and supratentorial primitive neuroectodermal tumor (MB/sPNET) in order to defer or avoid radiotherapy in young children.
  • PROCEDURE: Thirty-seven HDCTs were given to 25 children with newly diagnosed high-risk or relapsed MB/sPNET.
  • RESULTS: Three-year EFS (+/-SE) in 6 newly diagnosed high-risk (>3 years old), 8 newly diagnosed (<3 years old), and 11 relapsed MB/sPNET was 83.3 +/- 15.2%, 62.5 +/- 20.5%, and 29.1 +/- 15.7%, respectively.
  • Although four treatment-related mortalities (TRMs) occurred during 25 first HDCTs, no TRM occurred during 12 second HDCTs.
  • CONCLUSIONS: High-dose chemotherapy may improve the survival of children with newly diagnosed high-risk MB/sPNET, and, to some extent, the survival of those with relapsed MB/sPNET.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cerebellar Neoplasms / therapy. Medulloblastoma / therapy. Neuroectodermal Tumors, Primitive / therapy. Peripheral Blood Stem Cell Transplantation. Supratentorial Neoplasms / therapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Cisplatin / administration & dosage. Cisplatin / adverse effects. Cranial Irradiation / contraindications. Cyclophosphamide / administration & dosage. Cyclophosphamide / adverse effects. Etoposide / administration & dosage. Etoposide / adverse effects. Female. Follow-Up Studies. Gastrointestinal Diseases / epidemiology. Gastrointestinal Diseases / etiology. Humans. Infection / epidemiology. Infection / etiology. Kaplan-Meier Estimate. Male. Salvage Therapy. Sepsis / etiology. Sepsis / mortality. Transplantation, Autologous. Treatment Outcome. Vincristine / administration & dosage. Vincristine / adverse effects


7. Geyer JR, Sposto R, Jennings M, Boyett JM, Axtell RA, Breiger D, Broxson E, Donahue B, Finlay JL, Goldwein JW, Heier LA, Johnson D, Mazewski C, Miller DC, Packer R, Puccetti D, Radcliffe J, Tao ML, Shiminski-Maher T, Children's Cancer Group: Multiagent chemotherapy and deferred radiotherapy in infants with malignant brain tumors: a report from the Children's Cancer Group. J Clin Oncol; 2005 Oct 20;23(30):7621-31
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  • [Title] Multiagent chemotherapy and deferred radiotherapy in infants with malignant brain tumors: a report from the Children's Cancer Group.
  • PURPOSE: To evaluate response rate, event-free survival (EFS), and toxicity of two chemotherapeutic regimens for treatment of children younger than 36 months with malignant brain tumors and to estimate control intervals without irradiation in children with no residual tumor after initial surgery and induction chemotherapy and with delayed irradiation in patients with residual tumor or metastatic disease at diagnosis.
  • PATIENTS AND METHODS: Patients were randomly assigned to one of two regimens of induction chemotherapy (vincristine, cisplatin, cyclophosphamide, and etoposide v vincristine, carboplatin, ifosfamide, and etoposide).
  • Maintenance chemotherapy began after induction in children without progressive disease.
  • Children with no residual tumors after induction therapy and no metastatic disease at diagnosis were not to receive radiation therapy unless their tumors progressed.
  • Forty-two percent of patients responded to induction chemotherapy.
  • For medulloblastoma, supratentorial primitive neuroectodermal tumor, ependymoma, and rhabdoid tumors, 5-year EFS rates were 32% +/- 5%, 17% +/- 6%, and 32% +/- 6%, and 14% +/- 7%, respectively.
  • Fifty-eight percent of patients who were alive 5 years after study entry had not received radiation therapy.
  • CONCLUSION: Intensified induction chemotherapy resulted in a high response rate of malignant brain tumors in infants.
  • Survival was comparable to that of previous studies, and most patients who survived did not receive radiation therapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Brain Neoplasms / radiotherapy
  • [MeSH-minor] Child, Preschool. Cisplatin / administration & dosage. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Ependymoma / drug therapy. Ependymoma / radiotherapy. Ependymoma / surgery. Etoposide / administration & dosage. Female. Glioma / drug therapy. Glioma / radiotherapy. Glioma / surgery. Humans. Ifosfamide / administration & dosage. Infant. Infant, Newborn. Male. Medulloblastoma / drug therapy. Medulloblastoma / radiotherapy. Medulloblastoma / surgery. Neoplasms, Germ Cell and Embryonal / drug therapy. Neoplasms, Germ Cell and Embryonal / radiotherapy. Neoplasms, Germ Cell and Embryonal / surgery. Neuroectodermal Tumors, Primitive, Peripheral / drug therapy. Neuroectodermal Tumors, Primitive, Peripheral / radiotherapy. Neuroectodermal Tumors, Primitive, Peripheral / surgery. Survival Rate. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 16234523.001).
  • [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 10382
  • [Publication-type] Clinical Trial; Journal Article; Randomized Controlled Trial; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; Q20Q21Q62J / Cisplatin; UM20QQM95Y / Ifosfamide
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8. Gilheeney SW, Saad A, Chi S, Turner C, Ullrich NJ, Goumnerova L, Scott RM, Marcus K, Lehman L, De Girolami U, Kieran MW: Outcome of pediatric pineoblastoma after surgery, radiation and chemotherapy. J Neurooncol; 2008 Aug;89(1):89-95
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  • [Title] Outcome of pediatric pineoblastoma after surgery, radiation and chemotherapy.
  • INTRODUCTION: Pineoblastomas are a category of supratentorial primitive neuroectodermal tumors (sPNETs) occurring in the pineal gland; some studies support the impression that patients with pineoblastomas have a worse prognosis than those with other sPNETs.
  • METHODS: We reviewed the medical records and tissue sections of all patients with the diagnosis of pineoblastoma that were treated at the Dana-Farber Cancer Institute/Children's Hospital Boston Pediatric Brain Tumor Program between 1986 and 2005.
  • RESULTS: Thirteen patients with the pathologic diagnosis of pineoblastoma were treated at our Hospital; 11 of these cases had complete records suitable for study.
  • Patients who received CSI and multi-agent chemotherapy had improved overall survival.
  • CONCLUSIONS: Seven of eleven patients with pineoblastoma are currently alive and free of disease, reflecting an improved outcome and longer survival than previously appreciated.
  • Gross total surgical resection appeared to correlate with improved survival, as did treatment with craniospinal irradiation and multi-agent chemotherapy.
  • Further study of this group of patients as a distinct diagnostic entity will be necessary to determine optimal therapy.
  • [MeSH-major] Pineal Gland / pathology. Pinealoma
  • [MeSH-minor] Adolescent. Age Factors. Child. Child, Preschool. Drug Therapy / statistics & numerical data. Female. Humans. Infant. Male. Neurosurgical Procedures / standards. Neurosurgical Procedures / statistics & numerical data. Prognosis. Radiotherapy / statistics & numerical data. Retrospective Studies. Secondary Prevention. Survival Rate. Treatment Outcome

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  • [Cites] Childs Nerv Syst. 2006 Jun;22(6):577-85 [16555075.001]
  • [Cites] Childs Nerv Syst. 1999 Oct;15(10):586-91 [10550590.001]
  • [Cites] Int J Radiat Oncol Biol Phys. 1998 Dec 1;42(5):959-67 [9869216.001]
  • [Cites] Acta Neuropathol. 2006 Jul;112(1):5-12 [16685513.001]
  • [Cites] J Clin Oncol. 1995 Jul;13(7):1687-96 [7602359.001]
  • [Cites] J Clin Oncol. 1995 Jun;13(6):1377-83 [7751882.001]
  • [Cites] Clin Cancer Res. 2004 Aug 15;10 (16):5482-93 [15328187.001]
  • [Cites] J Clin Oncol. 2004 Mar 15;22(6):994-8 [14970184.001]
  • [Cites] Cancer. 2002 Jan 15;94(2):552-60 [11900240.001]
  • [Cites] Neurol Clin. 2003 Nov;21(4):897-913 [14743655.001]
  • [Cites] J Clin Oncol. 1997 May;15(5):1814-23 [9164190.001]
  • [Cites] Neurosurg Focus. 2005 Nov 15;19(5):E3 [16398467.001]
  • [Cites] Med Pediatr Oncol. 2002 Sep;39(3):168-74 [12210445.001]
  • [Cites] Am J Surg Pathol. 2004 May;28(5):644-50 [15105654.001]
  • [Cites] Cancer Genet Cytogenet. 2006 Oct 15;170(2):175-9 [17011992.001]
  • [Cites] Pediatr Blood Cancer. 2004 Mar;42(3):261-7 [14752864.001]
  • [Cites] Med Pediatr Oncol. 1995 Jul;25(1):38-44 [7753001.001]
  • [Cites] J Clin Oncol. 2005 Oct 20;23(30):7621-31 [16234523.001]
  • [Cites] Neuro Oncol. 1999 Apr;1(2):152-61 [11554387.001]
  • [Cites] J Clin Oncol. 2003 Jun 1;21(11):2187-91 [12775745.001]
  • [Cites] Cancer. 2000 May 1;88(9):2189-93 [10813733.001]
  • [Cites] J Neuropathol Exp Neurol. 2005 May;64(5):391-7 [15892296.001]
  • [Cites] J Neurooncol. 2007 Jan;81(2):217-23 [16941074.001]
  • [Cites] J Clin Oncol. 2004 Mar 15;22(6):984-93 [14970185.001]
  • (PMID = 18415046.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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9. Broniscer A, Nicolaides TP, Dunkel IJ, Gardner SL, Johnson J Jr, Allen JC, Sposto R, Finlay JL: High-dose chemotherapy with autologous stem-cell rescue in the treatment of patients with recurrent non-cerebellar primitive neuroectodermal tumors. Pediatr Blood Cancer; 2004 Mar;42(3):261-7
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  • [Title] High-dose chemotherapy with autologous stem-cell rescue in the treatment of patients with recurrent non-cerebellar primitive neuroectodermal tumors.
  • BACKGROUND: Recurrent non-cerebellar primitive neuroectodermal tumors (PNETs) carry a dismal prognosis when treated with conventional chemotherapy alone.
  • XSWe tested the efficacy of high-dose chemotherapy (HDC) followed by autologous stem-cell rescue (ASCR) in this setting.
  • PROCEDURE: Eligibility mandated either minimal residual disease or evidence of chemosensitivity before HDC.
  • RESULTS: Among 17 patients treated in this study, there were eight pineoblastoma(s) (pineo), seven cortical PNETs, and two arising elsewhere.
  • Relapse was either local (nine) or metastatic to the brain (four) or spine (four).
  • Two patients received HDC as the sole therapy for recurrence; additionally, eight underwent surgical debulking before HDC, and nine received irradiation, including six after HDC.
  • Two patients died of toxicity (11%) and ten experienced tumor relapse (range: 23-361 days post ASCR).
  • The difference in 5-year event-free survival (EFS) between patients with pineo and other supratentorial PNETs was significant (0 vs. 62.5 +/- 17%, P = 0.0065).
  • Surgical debulking before, and irradiation after HDC play an important role in treatment success.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Hematopoietic Stem Cell Transplantation. Neuroectodermal Tumors / therapy. Salvage Therapy / methods
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Clinical Trials as Topic. Combined Modality Therapy. Disease-Free Survival. Female. Humans. Infant. Male. Multicenter Studies as Topic. Recurrence. Retrospective Studies. Transplantation Conditioning / adverse effects. Transplantation Conditioning / methods. Transplantation, Autologous. Treatment Outcome

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  • [Copyright] Copyright 2003 Wiley-Liss, Inc.
  • (PMID = 14752864.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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10. Dai AI, Backstrom JW, Burger PC, Duffner PK: Supratentorial primitive neuroectodermal tumors of infancy: clinical and radiologic findings. Pediatr Neurol; 2003 Nov;29(5):430-4
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  • [Title] Supratentorial primitive neuroectodermal tumors of infancy: clinical and radiologic findings.
  • One hundred ninety-eight children were entered on POG 8633, "Prolonged Postoperative Chemotherapy and Delayed Radiation for Children <3 years of age with Malignant Brain Tumors" (1986-1990).
  • Thirteen manifested supratentorial nonpineoblastoma primitive neuroectodermal tumors, making this the second most common supratentorial tumor in the study.
  • The average tumor size was 5.96 cm (+/- 0.37) x 5 cm (+/- 0.28) x 5.15 cm (+/- 0.31).
  • Eight tumors were predominantly hemispheral, and five were midline.
  • Computed tomographic scans on nine patients revealed tumor hyperdensity (nine), midline shift (eight), hydrocephalus (seven), cysts (six), well-defined borders (five), and calcification (four).
  • Magnetic resonance imaging appearance of the tumor (six patients) demonstrated midline shift (four), well-defined margins (four), necrosis (two), cysts (three), and hemorrhage (two).
  • The diagnosis of supratentorial nonpineoblastoma primitive neuroectodermal tumors should be suspected when a large, sharply marginated, hyperdense supratentorial mass is observed in a young child, particularly when no peritumoral edema is present.
  • [MeSH-major] Neuroectodermal Tumors, Primitive / diagnosis. Supratentorial Neoplasms / diagnosis. Tomography, X-Ray Computed
  • [MeSH-minor] Child, Preschool. Female. Humans. Infant. Infant, Newborn. Magnetic Resonance Imaging. Male

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  • (PMID = 14684239.001).
  • [ISSN] 0887-8994
  • [Journal-full-title] Pediatric neurology
  • [ISO-abbreviation] Pediatr. Neurol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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11. Biswas S, Burke A, Cherian S, Williams D, Nicholson J, Horan G, Jefferies S, Williams M, Earl HM, Burnet NG, Hatcher H: Non-pineal supratentorial primitive neuro-ectodermal tumors (sPNET) in teenagers and young adults: Time to reconsider cisplatin based chemotherapy after cranio-spinal irradiation? Pediatr Blood Cancer; 2009 Jul;52(7):796-803
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  • [Title] Non-pineal supratentorial primitive neuro-ectodermal tumors (sPNET) in teenagers and young adults: Time to reconsider cisplatin based chemotherapy after cranio-spinal irradiation?
  • BACKGROUND: Supratentorial PNET (sPNET) are rare CNS tumors of embryonal origin arising in children and adults.
  • The treatment of sPNET for all age groups at our cancer center has been based on the management of medulloblastoma (MB), involving neurosurgical debulking followed by cranio-spinal irradiation (CSI) and systemic chemotherapy.
  • METHODS: Medical records were reviewed to gather demographic and clinical data about all embryonal CNS tumors in children and adults from 2001 to 2007.
  • Tumor pathology, clinical management and survival data were also assessed, particularly as regards those patients who received the Packer chemotherapy regimen for either sPNET or MB.
  • RESULTS: Eleven patients (five children and six adults) were identified with non-pineal sPNET, three children with pineal sPNET, and 19 patients (18 children and 1 adult) with MB.
  • There was no difference in overall survival (OS) rates between pediatric and adult sPNET.
  • When all sPNET were compared to all MB, 5-year OS was 14% versus 73%, respectively, but was only 9% for non-pineal sPNET.
  • When only considering those patients treated with the Packer chemotherapy regimen, the 5-year OS was 12% for sPNET versus 79% for MB.
  • CONCLUSION: This retrospective study demonstrates that non-pineal sPNET are clinically distinct from MB and are resistant to the Packer chemotherapy regimen.
  • We suggest that it is time to reconsider the use of this regimen in teenage and young adult non-pineal sPNET and to investigate the utility of alternative approaches.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cerebellar Neoplasms / therapy. Cranial Irradiation. Medulloblastoma / therapy. Neuroectodermal Tumors, Primitive / therapy. Supratentorial Neoplasms / therapy
  • [MeSH-minor] Adolescent. Adult. Child. Cisplatin / administration & dosage. Follow-Up Studies. Humans. Lomustine / administration & dosage. Neoplasm Staging. Retrospective Studies. Survival Rate. Treatment Outcome. Vincristine / administration & dosage. Young Adult

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  • [Copyright] (c) 2009 Wiley-Liss, Inc.
  • (PMID = 19202566.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase I; Clinical Trial, Phase II; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 7BRF0Z81KG / Lomustine; Q20Q21Q62J / Cisplatin
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12. Paulino AC, Cha DT, Barker JL Jr, Lo S, Manera RB: Patterns of failure in relation to radiotherapy fields in supratentorial primitive neuroectodermal tumor. Int J Radiat Oncol Biol Phys; 2004 Mar 15;58(4):1171-6
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  • [Title] Patterns of failure in relation to radiotherapy fields in supratentorial primitive neuroectodermal tumor.
  • PURPOSE: Supratentorial primitive neuroectodermal tumor (PNET) accounts for 2-3% of all pediatric brain tumors.
  • We retrospectively reviewed all supratentorial PNET cases treated with radiotherapy (RT) at our institutions.
  • The primary site location was the pineal region in 7 (28%), temporal lobe in 5 (20%), thalamus in 5 (20%), frontal lobe in 4 (16%), parietal lobe in 2 (8%), and suprasellar region in 2 (8%).
  • The RT treatment volumes were craniospinal (CS) in 17 (68%), whole brain (WB) followed by a boost in 2 (8%), and primary site (PS) alone in 6 (24%).
  • The median dose to the primary site was 54 Gy (range, 31-55.8 Gy).
  • The median dose to patients receiving WB and spinal fields was 36 Gy (range, 23.4-39.6 Gy).
  • Sixteen patients (64%) received chemotherapy; the most common type was the "8 in 1" chemotherapy regimen in 9 children.
  • RESULTS: The 5-year and 10-year progression-free survival rate was 36% and 27%, respectively, and the median time to progression was 22 months.
  • The 5-year and 10-year progression-free survival rate for those with M0 disease was 40.0% and 30.0%, respectively; for those with M+ disease, the corresponding figures were 20.0% and 0%.
  • Four (80%) of 5 M+ children and 4 (33%) of 12 M0 children who underwent CSRT developed recurrence in the neuraxis (p = 0.1, Fisher's exact test).
  • CONCLUSION: The craniospinal axis is the standard volume that needs to be treated in supratentorial PNET.
  • [MeSH-major] Brain Neoplasms / radiotherapy. Neuroectodermal Tumors / radiotherapy
  • [MeSH-minor] Adolescent. Adult. Analysis of Variance. Child. Child, Preschool. Female. Humans. Infant. Male. Radiotherapy Dosage. Retrospective Studies. Supratentorial Neoplasms / radiotherapy. Survival Rate. Treatment Failure


13. Palmer S, Wallace D, Bonner M, Raggl R, Chapieski L, Janzen L, Knight S, Boyle R, Armstrong C, Gajjar A: A longitudinal study of processing speed among children treated for medulloblastoma (MB), supratentorial primitive neuroectodermal tumor (SPNET), or atypical teratoid rhabdoid tumor (ATRT). J Clin Oncol; 2009 May 20;27(15_suppl):10028

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  • [Title] A longitudinal study of processing speed among children treated for medulloblastoma (MB), supratentorial primitive neuroectodermal tumor (SPNET), or atypical teratoid rhabdoid tumor (ATRT).
  • Patients were treated with post-surgical risk-adapted craniospinal irradiation (CSI) followed by 4 cycles of high-dose chemotherapy (cyclophosphamide, cisplatin, vincristine) with stem cell support.
  • High risk (HR, n = 55) patients received 36 - 39.6 Gy CSI and 3D conformal boost to the primary site to 55.8 -59.4 Gy.
  • Average-risk (AR, n=119) patients received 23.4 Gy CSI, 3D conformal boost to the primary site to 55.8 Gy.
  • RESULTS: Multivariate modeling revealed a statistically significant decline in processing speed for those < 7 years of age at time of diagnosis (-3.83 points per year, p = 0.003).
  • CONCLUSIONS: Young age at diagnosis is a prominent risk factor for processing speed impairment among survivors of pediatric embryonal tumors.
  • Processing speed may also be among the first deficits to appear following treatment.
  • This study represents the largest comparison of processing speed ability among patients treated for pediatric embryonal tumors with conventional or reduced dose CSI and adjuvant chemotherapy.

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  • (PMID = 27962588.001).
  • [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
  • [Publication-type] Journal Article
  • [Publication-country] United States
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14. Johnston DL, Keene DL, Lafay-Cousin L, Steinbok P, Sung L, Carret AS, Crooks B, Strother D, Wilson B, Odame I, Eisenstat DD, Mpofu C, Zelcer S, Huang A, Bouffet E: Supratentorial primitive neuroectodermal tumors: a Canadian pediatric brain tumor consortium report. J Neurooncol; 2008 Jan;86(1):101-8
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  • [Title] Supratentorial primitive neuroectodermal tumors: a Canadian pediatric brain tumor consortium report.
  • INTRODUCTION: Supratentorial primitive neuroectodermal tumors (SPNET) are rare tumors accounting for only 2.5% of childhood brain tumors.
  • The purpose of this study was to describe the range of treatment regimens used to treat pediatric SPNET in Canada and to identify prognostic factors for overall survival in this population.
  • A questionnaire was developed and distributed to all institutions in Canada who treat pediatric patients.
  • The best responses to therapy included complete response in 44%, partial response in 8%, still on therapy in 2%, progressive disease in 31%, toxic death in 2%, and no therapy given in 12%.
  • The factors associated with an increase in survival were the use of radiation therapy and chemotherapy, and age >2 years.
  • Overall survival was not affected by metastatic disease at diagnosis, tumor site, or degree of initial resection.
  • CONCLUSIONS: Survival is poor in SPNET patients but highest in those who received chemotherapy and radiation therapy.
  • [MeSH-major] Brain Neoplasms / epidemiology. Neuroectodermal Tumors, Primitive / epidemiology. Pediatrics. Supratentorial Neoplasms / epidemiology
  • [MeSH-minor] Adolescent. Canada / epidemiology. Child. Child, Preschool. Female. Health Surveys. Humans. Infant. Infant, Newborn. Male. Retrospective Studies. Surveys and Questionnaires. Survival Analysis


15. Driever PH, Wagner S, Hofstädter F, Wolff JE: Valproic acid induces differentiation of a supratentorial primitive neuroectodermal tumor. Pediatr Hematol Oncol; 2004 Dec;21(8):743-51
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  • [Title] Valproic acid induces differentiation of a supratentorial primitive neuroectodermal tumor.
  • In preclinical models the antiepileptic drug valproic acid induces differentiation of neoplastic cells, representing an evolving anticancer approach that takes into account that malignant cells resemble immature progenitor cells capable of terminal differentiation.
  • The authors report on a child suffering from a relapsing supratentorial primitive neuroectodermal tumor that received valproic acid for epilepsy treatment over 7 months before the relapse.
  • In contrast to the initial tumor, the relapsing tumor showed glial differentiation and low proliferation index.
  • This is the first report of a relapsed supratentorial primitive neuroectodermal tumor that shows histologically confirmed signs of tumor cell differentiation induction.
  • [MeSH-major] Cell Differentiation / drug effects. Neuroectodermal Tumors, Primitive / pathology. Supratentorial Neoplasms / pathology. Valproic Acid / pharmacology
  • [MeSH-minor] Cell Proliferation. Child. Epilepsy / drug therapy. Family Health. Humans. Male. Neuroglia / drug effects. Recurrence

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  • (PMID = 15739631.001).
  • [ISSN] 0888-0018
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 614OI1Z5WI / Valproic Acid
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16. Stewart CF, Iacono LC, Chintagumpala M, Kellie SJ, Ashley D, Zamboni WC, Kirstein MN, Fouladi M, Seele LG, Wallace D, Houghton PJ, Gajjar A: Results of a phase II upfront window of pharmacokinetically guided topotecan in high-risk medulloblastoma and supratentorial primitive neuroectodermal tumor. J Clin Oncol; 2004 Aug 15;22(16):3357-65
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  • [Title] Results of a phase II upfront window of pharmacokinetically guided topotecan in high-risk medulloblastoma and supratentorial primitive neuroectodermal tumor.
  • PURPOSE: To assess the antitumor efficacy of pharmacokinetically guided topotecan dosing in previously untreated patients with medulloblastoma and supratentorial primitive neuroectodermal tumors, and to evaluate plasma and CSF disposition of topotecan in these patients.
  • Pharmacokinetic studies were conducted on day 1 to attain a topotecan lactone area under the plasma concentration-time curve (AUC) of 120 to 160 ng/mL.h.
  • Toxicity was mostly hematologic, with only one patient experiencing treatment delay.
  • CONCLUSION: Topotecan is an effective agent against pediatric medulloblastoma in patients who have received no therapy other than surgery.
  • This drug warrants testing as part of standard postradiation chemotherapeutic regimens.
  • Furthermore, these results emphasize the importance of translational research in drug development, which in this case identified an effective drug.
  • [MeSH-major] Antineoplastic Agents / pharmacokinetics. Antineoplastic Agents / therapeutic use. Brain Neoplasms / drug therapy. Cerebellar Neoplasms / drug therapy. Medulloblastoma / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy. Topotecan / pharmacokinetics. Topotecan / therapeutic use
  • [MeSH-minor] Adolescent. Adult. Area Under Curve. Child. Child, Preschool. Female. Humans. Infusions, Intravenous. Male. Risk Factors. Treatment Outcome


17. Timmermann B, Kortmann RD, Kühl J, Meisner C, Dieckmann K, Pietsch T, Bamberg M: Role of radiotherapy in the treatment of supratentorial primitive neuroectodermal tumors in childhood: results of the prospective German brain tumor trials HIT 88/89 and 91. J Clin Oncol; 2002 Feb 01;20(3):842-9
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  • [Title] Role of radiotherapy in the treatment of supratentorial primitive neuroectodermal tumors in childhood: results of the prospective German brain tumor trials HIT 88/89 and 91.
  • PURPOSE: To evaluate the outcome of children with supratentorial primitive neuroectodermal tumors after surgery, irradiation, and chemotherapy and to identify factors predictive for survival.
  • Patients were randomized for preirradiation chemotherapy, consisting of two cycles of ifosfamide, etoposide, methotrexate, cisplatin, and cytarabine (n = 40), or chemotherapy after irradiation, consisting of eight cycles with cisplatin, vincristine, and lomustine (n = 23).
  • Irradiation volume was recommended to encompass the neuraxis with 35.2-Gy total dose followed by a boost (20.0 Gy) to the primary tumor site (n = 54).
  • Seven patients were irradiated to the tumor region only with a total dose of 54.0 Gy.
  • The only significant prognostic factor was dose and volume of radiotherapy (progression-free survival after 3 years was 49.3% with correct treatment compared with 6.7% for 15 children with major violations of radiotherapy).
  • Ten early progressions occurred during adjuvant therapy (eight before and two during radiotherapy), nine of them treated with preirradiation chemotherapy.
  • There was a positive trend in outcome for nonmetastatic and pineal tumors.
  • Volume of irradiation should encompass the whole CNS with additional boost to the tumor region.
  • Local doses of at least 54 Gy and a craniospinal dose of 35 Gy are necessary.
  • Preirradiation chemotherapy seems to increase risk of early progression.
  • [MeSH-major] Neuroectodermal Tumors / radiotherapy. Supratentorial Neoplasms / radiotherapy
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Child. Child, Preschool. Cisplatin / administration & dosage. Combined Modality Therapy. Cytarabine / administration & dosage. Drug Administration Schedule. Etoposide / administration & dosage. Female. Humans. Ifosfamide / administration & dosage. Lomustine / administration & dosage. Male. Methotrexate / administration & dosage. Neoplasm Recurrence, Local. Radiotherapy Dosage. Survival Rate. Treatment Outcome. Vincristine / administration & dosage


18. Hong TS, Mehta MP, Boyett JM, Donahue B, Rorke LB, Zeltzer PM: Patterns of treatment failure in infants with primitive neuroectodermal tumors who were treated on CCG-921: a phase III combined modality study. Pediatr Blood Cancer; 2005 Oct 15;45(5):676-82
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  • [Title] Patterns of treatment failure in infants with primitive neuroectodermal tumors who were treated on CCG-921: a phase III combined modality study.
  • PURPOSE: To analyze patterns of treatment failure in infants with primitive neuroectodermal tumors (PNETs) who were treated primarily with chemotherapy in a large multi-institutional study.
  • MATERIALS AND METHODS: Sixty-five prospectively staged patients with PNET confirmed by central pathology review, who were 18 months or younger were treated on Children's Cancer Group Study 921 (CCG-921) primarily with chemotherapy.
  • Forty-six patients had posterior fossa (PF) primary tumors and 19 patients had supratentorial (ST) primaries.
  • Patterns of sites of initial treatment failure were analyzed and compared to failure patterns of 180 older children who had PF-PNETs, and 44 older children with ST-PNETs who were treated on the same protocol.
  • Cumulative 5-year relapse incidence (+/-SE) for younger patients with PF-PNETs was 64.5 +/- 8.9% for patients without metastases (M0) compared to 71.4 +/- 13.4% for patients with metastases (M+).
  • The overall treatment failure rate was significantly higher for younger compared to older patients with PF-PNET and ST-PNET.
  • There was no statistically significant difference in relapse patterns between patients with PF primary tumors and ST primaries when stratified by stage.
  • All patients had a high risk of recurrence at primary tumor site.
  • Younger patients who had PF primary tumors without metastasis at presentation were significantly more likely to relapse in PF than older patients.
  • CONCLUSIONS: Despite aggressive chemotherapy, younger children with PNETs have high rates of treatment failure and fare worse than high-risk, older patients with PF-PNETs, indicating the need to maximize local, regional, and systemic therapies.
  • [MeSH-major] Brain Neoplasms / therapy. Neuroectodermal Tumors, Primitive / therapy
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Clinical Trials, Phase III as Topic. Combined Modality Therapy. Disease Progression. Disease-Free Survival. Humans. Infant. Infratentorial Neoplasms / mortality. Infratentorial Neoplasms / pathology. Infratentorial Neoplasms / therapy. Neoplasm Recurrence, Local. Supratentorial Neoplasms / mortality. Supratentorial Neoplasms / pathology. Supratentorial Neoplasms / therapy. Survival Rate. Treatment Failure

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  • (PMID = 16007595.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA21765
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
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19. Blankenburg F, van Landeghem FK, Plotkin M, Henze G, von Deimling A, Driever PH: Occurrence of a spinal anaplastic pilocytic astrocytoma and a supratentorial PNET in an adolescent. J Pediatr Hematol Oncol; 2007 Dec;29(12):832-5
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  • [Title] Occurrence of a spinal anaplastic pilocytic astrocytoma and a supratentorial PNET in an adolescent.
  • The tumor was subtotally resected and treated with chemotherapy and irradiation.
  • At the age of 17, the patient developed a supratentorial primitive neuroectodermal tumor.
  • We report the first case of a primary anaplastic pilocytic astrocytoma and primary primitive neuroectodermal tumor occurring in the same patient.
  • [MeSH-minor] Adolescent. Codon. Exons. Fatal Outcome. Female. Humans. Mitotic Index. Mutation, Missense. Neuroectodermal Tumors, Primitive / complications. Neuroectodermal Tumors, Primitive / genetics. Neuroectodermal Tumors, Primitive / pathology. Tumor Suppressor Protein p53 / genetics

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  • (PMID = 18090931.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Codon; 0 / Tumor Suppressor Protein p53
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20. Wang CH, Hsu TR, Wong TT, Chang KP: Efficacy of temozolomide for recurrent embryonal brain tumors in children. Childs Nerv Syst; 2009 May;25(5):535-41
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  • [Title] Efficacy of temozolomide for recurrent embryonal brain tumors in children.
  • OBJECTIVE: The salvage therapy of recurrent embryonal brain tumors in children is disappointing.
  • Temozolomide is a newly developed chemotherapeutic agent in central nervous system tumors.
  • This study analyzed the efficacy of temozolomide on the treatment of recurrent embryonal brain tumors in children.
  • MATERIALS AND METHODS: There were eight patients, including four with medulloblastoma (MB), three with atypical teratoid/rhabdoid tumor (AT/RT) and one with supratentorial primitive neuroectodermal tumor, whose tumors recurred after surgery and radiotherapy, with or without conventional intravenous cisplatin-based chemotherapy.
  • The responsiveness of the tumors to temozolomide was judged by magnetic resonance imaging (MRI) during regular follow-up.
  • RESULTS: The median treatment cycles received by these eight patients were 17 (range from two to 59 cycles).
  • The follow-up MRI showed no tumor progression in five patients at 6 months and four patients at 12 months.
  • The observed adverse effects of temozolomide included nausea, vomiting, headache, constipation, mild marrow suppression, and decreased activity; none of them was severe enough to discontinue the treatment.
  • CONCLUSION: In this preliminary study, oral temozolomide shows promising results on recurrent embryonal brain tumors in children.
  • When conventional chemotherapy fails and/or the adverse response is too severe to tolerate, temozolomide is a reasonable alternative.
  • However, a further well-designed, controlled study and more long-term follow-up are needed to assess the exact role of temozolomide in children with embryonal tumors in brain.
  • [MeSH-major] Antineoplastic Agents, Alkylating / therapeutic use. Brain Neoplasms / drug therapy. Dacarbazine / analogs & derivatives. Medulloblastoma / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy. Rhabdoid Tumor / drug therapy. Teratoma / drug therapy
  • [MeSH-minor] Administration, Oral. Adolescent. Disease-Free Survival. Female. Follow-Up Studies. Headache / chemically induced. Humans. Magnetic Resonance Imaging. Male. Nausea / chemically induced. Treatment Outcome. Vomiting / chemically induced

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  • [Cites] Ann Oncol. 2001 Feb;12 (2):259-66 [11300335.001]
  • [Cites] J Neuropathol Exp Neurol. 2002 Mar;61(3):215-25; discussion 226-9 [11895036.001]
  • [Cites] N Engl J Med. 1994 Mar 31;330(13):892-5 [8114859.001]
  • [Cites] Childs Nerv Syst. 2005 Apr;21(4):272-93 [15682321.001]
  • [Cites] Cancer Invest. 2007 Sep;25(6):470-5 [17882660.001]
  • [Cites] Cancer. 2007 Oct 1;110(7):1542-50 [17705175.001]
  • [Cites] Cancer Res. 1995 Jul 1;55(13):2853-7 [7796412.001]
  • [Cites] Oncologist. 2003;8(2):174-86 [12697942.001]
  • [Cites] J Pediatr Hematol Oncol. 2002 Oct;24(7):591-3 [12368706.001]
  • [Cites] J Clin Oncol. 1998 Sep;16(9):3037-43 [9738573.001]
  • [Cites] J Neurosurg. 2003 Aug;99(2):280-6 [12924701.001]
  • [Cites] Eur J Cancer. 2006 Sep;42(14 ):2335-42 [16899365.001]
  • [Cites] Lancet Oncol. 2001 Sep;2(9):552-60 [11905710.001]
  • [Cites] Neoplasma. 2002;49(2):117-20 [12088104.001]
  • [Cites] Br J Cancer. 2000 Sep;83(5):588-93 [10944597.001]
  • [Cites] Cancer. 2005 Nov 15;104(10):2156-67 [16220552.001]
  • [Cites] Acta Neurol Belg. 2007 Jun;107(2):51-4 [17710841.001]
  • (PMID = 19107490.001).
  • [ISSN] 1433-0350
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 7GR28W0FJI / Dacarbazine; YF1K15M17Y / temozolomide
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21. Rostomily RC, Halligan J, Geyer R, Stelzer K, Lindsley K, Berger MS: Permanent low-activity (125)I seed placement for the treatment of pediatric brain tumors: preliminary experience. Pediatr Neurosurg; 2001 Apr;34(4):198-205
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  • [Title] Permanent low-activity (125)I seed placement for the treatment of pediatric brain tumors: preliminary experience.
  • Although external beam radiation therapy is effective in the treatment of many pediatric brain neoplasms its use in this patient population has been associated with the development of significant cognitive and endocrine dysfunction and is severely limited as an option in previously irradiated patients.
  • Therefore, we have adopted a strategy for management of residual microscopic disease by implantation of low-activity (125)I seeds in the tumor bed at the time of surgery.
  • Six patients aged 2-14 years with recurrent tumors including two supratentorial primitive neuroectodermal tumors (n = 2), one medulloblastoma, one malignant ependymoma (n = 1), glioblastoma (n = 1) and one pleomorphic xanthoastrocytoma were implanted at the time of reoperation.
  • A total of 11-126 seeds were implanted resulting in total doses of 16-21.8 Gy (after theoretical infinite time) at a depth of 5 mm from the implanted resection bed.
  • Five patients had prior external beam radiation while the other patient (2 years old at initial diagnosis) progressed after surgery and chemotherapy.
  • Two patients had lasting local tumor control.
  • These results suggest that the use of permanent low-activity (125)I seeds as an adjunct to surgery can provide good local tumor control and is a suitable treatment option for pediatric patients.
  • [MeSH-major] Brachytherapy / adverse effects. Brain Neoplasms / radiotherapy. Iodine Radioisotopes / pharmacokinetics. Iodine Radioisotopes / therapeutic use
  • [MeSH-minor] Adolescent. Brain / metabolism. Brain / pathology. Child. Child, Preschool. Drug Implants. Female. Humans. Magnetic Resonance Imaging. Male. Neoplasm Recurrence, Local. Radiotherapy Dosage. Treatment Outcome

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  • [Copyright] Copyright 2001 S. Karger AG, Basel
  • (PMID = 11359113.001).
  • [ISSN] 1016-2291
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Grant] United States / PHS HHS / / 2T 32-N07144
  • [Publication-type] Journal Article; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Drug Implants; 0 / Iodine Radioisotopes
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22. Herrlinger U, Steinbrecher A, Rieger J, Hau P, Kortmann RD, Meyermann R, Schabet M, Bamberg M, Dichgans J, Bogdahn U, Weller M: Adult medulloblastoma: prognostic factors and response to therapy at diagnosis and at relapse. J Neurol; 2005 Mar;252(3):291-9
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  • [Title] Adult medulloblastoma: prognostic factors and response to therapy at diagnosis and at relapse.
  • Adult medulloblastoma is a rare tumor with few retrospective studies published so far.
  • The role of adjuvant chemotherapy or chemotherapy at relapse is unclear.
  • This study reports therapy and outcome in all adult (>or=16 years old) medulloblastoma (n=34) and supratentorial primitive neuroectodermal tumor (PNET) patients (n=2) treated in 2 neuro-oncological centers between 1976 and 2002.
  • After resection, 16 patients were treated with craniospinal radiotherapy alone, 20 patients also received adjuvant chemotherapy (8 vincristine, CCNU, cisplatin; 7 methotrexate alone or methotrexate/vincristine-based polychemotherapy; 5 other protocols).
  • Adjuvant chemotherapy was associated with a non-significant trend to prolonged survival (relative risk (RR) 1.89; p=0.068).
  • The median progression-free survival (PFS) after primary therapy was 83 months.
  • At relapse, 10 of 12 evaluable patients achieved a complete response upon second-line therapy.
  • The median survival times from first (n=17) and second relapse (n=9) were 21 months (0-67+ months; 5/17 without second relapse) and 20 months (1-29 months).
  • Cox regression analysis revealed the infiltration of the floor of the 4(th) ventricle at diagnosis as the only therapy-independent prognostic factor (RR 0.48; p=0.03).
  • In conclusion, adjuvant chemotherapy may prolong survival in adult medulloblastoma patients.
  • Moreover, second-line therapy may be beneficial for these patients.
  • As in pediatric medulloblastoma patients, primary infiltration of the floor of the 4(th) ventricle indicates a poor prognosis.
  • [MeSH-major] Cerebellar Neoplasms / therapy. Medulloblastoma / therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Analysis of Variance. Combined Modality Therapy. Demography. Disease Progression. Disease-Free Survival. Dose-Response Relationship, Radiation. Drug Therapy / methods. Female. Humans. Male. Middle Aged. Radiotherapy, High-Energy / methods. Recurrence. Regression Analysis. Retrospective Studies. Risk Factors. Time Factors. Treatment Outcome

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  • [Cites] Int J Radiat Oncol Biol Phys. 1990 Apr;18(4):763-72 [2323967.001]
  • [Cites] J Clin Oncol. 1999 Mar;17(3):832-45 [10071274.001]
  • [Cites] Int J Cancer. 1999 Mar 1;80(5):689-92 [10048968.001]
  • [Cites] J Neurosurg. 1990 Apr;72(4):572-82 [2319316.001]
  • [Cites] Int J Radiat Oncol Biol Phys. 1995 Jul 15;32(4):1145-52 [7607936.001]
  • [Cites] Eur J Cancer. 1990 Apr;26(4):464-9 [2141512.001]
  • [Cites] Int J Radiat Oncol Biol Phys. 2002 Nov 1;54(3):855-60 [12377339.001]
  • [Cites] Cancer. 1994 Oct 15;74(8):2352-60 [7922986.001]
  • [Cites] Neurosurgery. 2000 Sep;47(3):623-31; discussion 631-2 [10981749.001]
  • [Cites] J Clin Oncol. 1990 Jul;8(7):1277-80 [2358840.001]
  • [Cites] Cancer Treat Rev. 1999 Feb;25(1):3-12 [10212586.001]
  • [Cites] Neuro Oncol. 2001 Jan;3(1):29-34 [11305414.001]
  • [Cites] Neurosurgery. 1996 Feb;38(2):265-71 [8869053.001]
  • [Cites] J Clin Oncol. 2002 Feb 1;20(3):842-9 [11821469.001]
  • [Cites] Cancer. 1999 Jul 1;86(1):142-8 [10391574.001]
  • [Cites] J Clin Oncol. 1999 Jul;17(7):2127-36 [10561268.001]
  • [Cites] Int J Radiat Oncol Biol Phys. 2000 Jun 1;47(3):573-84 [10837938.001]
  • [Cites] Bone Marrow Transplant. 2002 Nov;30(9):565-9 [12407430.001]
  • [Cites] Neurology. 1995 Mar;45(3 Pt 1):440-2 [7898692.001]
  • [Cites] J Clin Oncol. 2003 Apr 15;21(8):1581-91 [12697884.001]
  • [Cites] Int J Radiat Oncol Biol Phys. 2000 Jan 15;46(2):269-79 [10661332.001]
  • [Cites] Int J Radiat Oncol Biol Phys. 1995 Jul 15;32(4):951-7 [7607969.001]
  • [Cites] J Clin Oncol. 1998 Jan;16(1):222-8 [9440746.001]
  • [Cites] J Neurosurg. 1994 Nov;81(5):690-8 [7931615.001]
  • [Cites] J Clin Oncol. 2000 Aug;18(16):3004-11 [10944134.001]
  • [Cites] Med Pediatr Oncol. 2002 Aug;39(2):99-108 [12116057.001]
  • [Cites] Radiology. 1969 Dec;93(6):1351-9 [4983156.001]
  • (PMID = 16189725.001).
  • [ISSN] 0340-5354
  • [Journal-full-title] Journal of neurology
  • [ISO-abbreviation] J. Neurol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Comparative Study; Journal Article
  • [Publication-country] Germany
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23. Gutenberg A, Schulten HJ, Gunawan B, Ludwig HC, Brück W, Larsen J, Rohde V: CNS tumor 22 years after spinal neuroblastoma IV: diagnostic dilemma between recurrence and secondary malignancy. Pediatr Neurosurg; 2009;45(1):61-8
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  • [Title] CNS tumor 22 years after spinal neuroblastoma IV: diagnostic dilemma between recurrence and secondary malignancy.
  • We present the very unusual case of a young woman suffering from a brain tumor 22 years after a stage IV spinal neuroblastoma as an infant, demonstrating the difficulties of differentiating late neuroblastoma relapse from secondary supratentorial primitive neuroectodermal tumor (sPNET).
  • Lacking specific immunohistochemical features, the first cerebral tumor at the age of 21 was regarded as sPNET, and we pursued a therapeutic approach consisting of neurosurgical resection as well as irradiation and high-dose alkylator-based chemotherapy according to the HIT2000 protocol.
  • Moreover, the lack of PNET-specific translocations (EWS/FLI1 gene fusion) in both brain tumors as well as the development of hepatic metastases was more compatible with the diagnosis of a very late relapse 22 years after initial stage IV spinal neuroblastoma.
  • [MeSH-major] Brain Neoplasms / pathology. Neoplasm Recurrence, Local / pathology. Neoplasms, Second Primary / pathology. Neuroblastoma / pathology. Spinal Neoplasms / pathology
  • [MeSH-minor] Adult. DNA, Neoplasm / genetics. Diagnosis, Differential. Female. Genetic Markers. Humans. Immunohistochemistry. Infant. Magnetic Resonance Imaging. Neoplasm Staging. Time Factors

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  • (PMID = 19258732.001).
  • [ISSN] 1423-0305
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / Genetic Markers
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24. Gardner SL: Application of stem cell transplant for brain tumors. Pediatr Transplant; 2004 Jun;8 Suppl 5:28-32
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  • [Title] Application of stem cell transplant for brain tumors.
  • Brain tumors are the second most common malignancy in children and the most common solid tumor.
  • The majority of children are treated with surgery alone or in combination with radiation and/or chemotherapy.
  • Recently investigators have used high dose chemotherapy with autologous stem cell rescue (ASCR) in patients with malignant brain tumors.
  • This approach has been most successful in chemosensitive tumors including medulloblastoma, supratentorial primitive neuroectodermal tumors (SPNET) and central nervous system germ cell tumors (CNS GCT).
  • In addition, the use of high dose chemotherapy has enabled the reduction and in many cases elimination of radiation therapy to very young children.
  • To date there have been no prospective randomized studies comparing high dose chemotherapy and ASCR with conventional therapy.
  • Radiation therapy is often not an option for patients with recurrent disease and conventional dose chemotherapy rarely if ever results in long-term survival.
  • Unfortunately, the majority of studies using conventional therapy in order to delay irradiation in young children newly diagnosed with malignant brain tumors have been unsuccessful.
  • Although the numbers are small, preliminary data suggest that not only is survival but also quality of life is superior with the use of high dose chemotherapy.
  • In addition, through the use of peripheral blood stem cells and improvements in supportive care, multiple courses of high dose chemotherapy can be administered.
  • High dose chemotherapy with ASCR is a foundation upon which many different types of therapies can be built.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / therapy. Hematopoietic Stem Cell Transplantation
  • [MeSH-minor] Combined Modality Therapy. Humans. Pediatrics

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  • (PMID = 15125703.001).
  • [ISSN] 1397-3142
  • [Journal-full-title] Pediatric transplantation
  • [ISO-abbreviation] Pediatr Transplant
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Denmark
  • [Number-of-references] 25
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25. Varan A, Akalan N, Söylemezoğlu F, Zorlu F, Yalçin B, Akyüz C, Kutluk T, Büyükpamukçu M: Central nervous system tumors in patients under three years of age: treatment results of a single institute. Pediatr Neurosurg; 2006;42(2):89-94
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  • [Title] Central nervous system tumors in patients under three years of age: treatment results of a single institute.
  • Eighty-six patients under 3 years of age with central nervous system tumors were retrospectively analyzed between 1972 and 2003.
  • Surgical resection was done in all patients except for those with optic glioma, pons glioma and pineal tumor.
  • Three different chemotherapy regimens were used in different time periods.
  • In 48 patients, the tumor was located in the posterior fossa, and 29 patients had a supratentorial tumor.
  • We had 32 (37.2%) patients with embryonic tumors (21 medulloblastoma, 4 ependymoblastoma, 5 with atypical teratoid rhabdoid and 2 with supratentorial primitive neuroectodermal tumors), 21 (24.4%) with ependymoma, 14 (16.3%) with optic glioma, 10 (11.6%) with astrocytoma, 3 (3.5%) with pons glioma and 6 (7.0%) with others.
  • OS rates according to the tumor localizations were 40.9 and 68.1% in the posterior fossa and supratentorial localizations, respectively (p=0.001).
  • OS rates were 33.7, 41.3 and 88.8% for the medulloblastoma+primitive neuroectodermal tumor groups, ependymoma and astrocytoma, respectively (p=0.0001).
  • Most of the patients had primitive embryonic tumors (37.2%).
  • The best prognostic factors were tumor localization and histology.
  • [MeSH-major] Central Nervous System Neoplasms / mortality. Central Nervous System Neoplasms / therapy. Neoplasms, Germ Cell and Embryonal / mortality. Neoplasms, Germ Cell and Embryonal / therapy
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child, Preschool. Female. Humans. Infant. Infant, Newborn. Male. Prognosis. Retrospective Studies. Rhabdoid Tumor / mortality. Rhabdoid Tumor / pathology. Rhabdoid Tumor / therapy. Survival Rate

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  • [Copyright] Copyright (c) 2006 S. Karger AG, Basel.
  • (PMID = 16465077.001).
  • [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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26. Yanofsky RA, Seshia SS, Dawson AJ, Stobart K, Greenberg CR, Booth FA, Prasad C, Del Bigio MR, Wrogemann JJ, Fike F, Gatti RA: Ataxia-telangiectasia: atypical presentation and toxicity of cancer treatment. Can J Neurol Sci; 2009 Jul;36(4):462-7
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  • [Title] Ataxia-telangiectasia: atypical presentation and toxicity of cancer treatment.
  • BACKGROUND: The onset of progressive cerebellar ataxia in early childhood is considered a key feature of ataxia-telangiectasia (A-T), accompanied by ocular apraxia, telangiectasias, immunodeficiency, cancer susceptibility and hypersensitivity to ionizing radiation.
  • METHODS: We describe the clinical features and course of three Mennonite children who were diagnosed with A-T following the completion of therapy for lymphoid malignancies.
  • RESULTS: Prior to cancer therapy, all had non-progressive atypical neurological abnormalities, with onset by age 30 months, including dysarthria, dyskinesia, hypotonia and/or dystonia, without telangiectasias.
  • All three children were "cured" of their lymphoid malignancies, but experienced severe adverse effects from the treatments administered.
  • The two children who received cranial irradiation developed supratentorial primitive neuroectodermal tumors of the brain, an association not previously described, with fatal outcomes.
  • Radiation therapy and radiomimetic drugs should be avoided in individuals with A-T.
  • [MeSH-major] Antineoplastic Agents / adverse effects. Ataxia Telangiectasia / chemically induced. Ataxia Telangiectasia / diagnosis. Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy
  • [MeSH-minor] Adolescent. Ataxia Telangiectasia Mutated Proteins. Cell Cycle Proteins / metabolism. Child. Child, Preschool. DNA-Binding Proteins / metabolism. Humans. Magnetic Resonance Imaging. Male. Nerve Tissue Proteins / metabolism. Protein-Serine-Threonine Kinases / metabolism. Retrospective Studies. Tumor Suppressor Proteins / metabolism. alpha-Fetoproteins / metabolism

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  • (PMID = 19650357.001).
  • [ISSN] 0317-1671
  • [Journal-full-title] The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
  • [ISO-abbreviation] Can J Neurol Sci
  • [Language] eng
  • [Grant] United States / NIAID NIH HHS / AI / AI 067769; United States / NINDS NIH HHS / NS / NS 052528
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] Canada
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Cell Cycle Proteins; 0 / DNA-Binding Proteins; 0 / Nerve Tissue Proteins; 0 / Tumor Suppressor Proteins; 0 / alpha-Fetoproteins; EC 2.7.11.1 / ATM protein, human; EC 2.7.11.1 / Ataxia Telangiectasia Mutated Proteins; EC 2.7.11.1 / Protein-Serine-Threonine Kinases
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