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1. 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.
  • 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).
  • 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.
  • [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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2. Fouladi M, Nicholson HS, Zhou T, Laningham F, Helton KJ, Holmes E, Cohen K, Speights RA, Wright J, Pollack IF, Children's Oncology Group: A phase II study of the farnesyl transferase inhibitor, tipifarnib, in children with recurrent or progressive high-grade glioma, medulloblastoma/primitive neuroectodermal tumor, or brainstem glioma: a Children's Oncology Group study. Cancer; 2007 Dec 1;110(11):2535-41
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  • [Title] A phase II study of the farnesyl transferase inhibitor, tipifarnib, in children with recurrent or progressive high-grade glioma, medulloblastoma/primitive neuroectodermal tumor, or brainstem glioma: a Children's Oncology Group study.
  • BACKGROUND: An open-label Phase II study of tipifarnib was conducted to evaluate its safety and efficacy in children with recurrent or refractory medulloblastoma (MB)/primitive neuroectodermal tumor (PNET), high-grade glioma (HGG), and diffuse intrinsic brainstem glioma (BSG).
  • METHODS: Between January 2004 and July 2005, patients were enrolled and stratified as follows: Stratum 1, recurrent or refractory MB/PNET; Stratum 2, recurrent or refractory HGG; and Stratum 3, recurrent or refractory BSG.
  • No responses were observed in 15 patients with MB/PNET.
  • The 6-month progression-free survival rate (+/-standard deviation) was 14%+/-6% for HGG, 6%+/-6% for MB/PNET and 3%+/-3% for BSG.
  • CONCLUSIONS: Tipifarnib tolerated well but had little activity as a single agent in children with recurrent central nervous system malignancies.
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Disease Progression. Female. Humans. Male. Neuroectodermal Tumors / drug therapy. Treatment Outcome


3. Allen J, Donahue B, Mehta M, Miller DC, Rorke LB, Jakacki R, Robertson P, Sposto R, Holmes E, Vezina G, Muraszko K, Puccetti D, Prados M, Chan KW: A phase II study of preradiotherapy chemotherapy followed by hyperfractionated radiotherapy for newly diagnosed high-risk medulloblastoma/primitive neuroectodermal tumor: a report from the Children's Oncology Group (CCG 9931). Int J Radiat Oncol Biol Phys; 2009 Jul 15;74(4):1006-11
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  • [Title] A phase II study of preradiotherapy chemotherapy followed by hyperfractionated radiotherapy for newly diagnosed high-risk medulloblastoma/primitive neuroectodermal tumor: a report from the Children's Oncology Group (CCG 9931).
  • PURPOSE: To verify feasibility and monitor progression-free survival and overall survival in children with high-risk medulloblastoma and noncerebellar primitive neuroectodermal tumors (PNETs) treated in a Phase II study with preradiotherapy chemotherapy (CHT) followed by high-dose, hyperfractionated craniospinal radiotherapy (CSRT).
  • METHODS AND MATERIALS: Eligibility criteria included age >3 years at diagnosis, medulloblastoma with either high M stage and/or >1.5 cm(2) postoperative residual disease, and all patients with noncerebellar PNET.
  • Treatment was initiated with five alternating monthly cycles of CHT (A [cisplatin, cyclophosphamide, etoposide, and vincristine], B [carboplatin and etoposide], A, B, and A) followed by hyperfractionated CSRT (40 Gy) with a boost to the primary tumor (72 Gy) given in twice-daily 1-Gy fractions.
  • No significant differences were detected in subset analysis related to response to CHT, site of primary tumor, postoperative residual disease, or M stage.

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  • (PMID = 19356859.001).
  • [ISSN] 1879-355X
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA003888-44; United States / NCI NIH HHS / CA / U10 CA003888; United States / NCI NIH HHS / CA / U10 CA098543; United States / NCI NIH HHS / CA / U10 CA003888-44
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Multicenter Study
  • [Publication-country] United States
  • [Chemical-registry-number] 143011-72-7 / Granulocyte Colony-Stimulating Factor; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; BG3F62OND5 / Carboplatin; Q20Q21Q62J / Cisplatin
  • [Other-IDs] NLM/ NIHMS128135; NLM/ PMC2739055
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4. Nicholson HS, Kretschmar CS, Krailo M, Bernstein M, Kadota R, Fort D, Friedman H, Harris MB, Tedeschi-Blok N, Mazewski C, Sato J, Reaman GH: Phase 2 study of temozolomide in children and adolescents with recurrent central nervous system tumors: a report from the Children's Oncology Group. Cancer; 2007 Oct 1;110(7):1542-50
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  • [Title] Phase 2 study of temozolomide in children and adolescents with recurrent central nervous system tumors: a report from the Children's Oncology Group.
  • BACKGROUND: Effective chemotherapy is lacking for most types of central nervous system (CNS) tumors in children.
  • Temozolomide, an agent with activity against adult brain tumors, was investigated in children and adolescents with recurrent CNS tumors.
  • RESULTS: The cohort comprised 122 patients, including 113 with CNS tumors.
  • Among 104 evaluable patients with CNS tumors, 5 PRs and 1 CR were observed.
  • PRs occurred in 1 of 23 evaluable patients with high-grade astrocytoma, 1 of 21 with low-grade astrocytoma, and 3 of 25 with medulloblastoma/primitive neuroectodermal tumor (PNET).
  • The CR occurred in an additional patient with medulloblastoma/PNET.
  • No responses were observed in patients with ependymoma, brain-stem glioma, or other CNS tumors.
  • [MeSH-major] Antineoplastic Agents, Alkylating / therapeutic use. Brain Neoplasms / drug therapy. Dacarbazine / analogs & derivatives. Neoplasm Recurrence, Local / drug therapy
  • [MeSH-minor] Administration, Oral. Adolescent. Adult. Astrocytoma / drug therapy. Central Nervous System Neoplasms / drug therapy. Child. Child, Preschool. Drug Administration Schedule. Ependymoma / drug therapy. Female. Humans. Infant. Male. Medulloblastoma / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy. Treatment Outcome


5. Cardy AH, Little J, McKean-Cowdin R, Lijinsky W, Choi NW, Cordier S, Filippini G, Holly EA, Lubin F, McCredie M, Mueller BA, Peris-Bonet R, Arslan A, Preston-Martin S: Maternal medication use and the risk of brain tumors in the offspring: the SEARCH international case-control study. Int J Cancer; 2006 Mar 1;118(5):1302-8
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  • Therefore, these compounds, when ingested by women during pregnancy, may be important risk factors for tumors of the central nervous system in the offspring.
  • Analysis was conducted for all participants combined, by tumor type (astroglial, primitive neuroectodermal tumors and other glioma), and by age at diagnosis (< or =5 years; >5 years).
  • [MeSH-minor] Adolescent. Adult. Amides / pharmacology. Amines / pharmacology. Case-Control Studies. Child. Child, Preschool. Disease Susceptibility. Female. Humans. Infant. Male. Pregnancy. Risk Factors

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  • [Copyright] (c) 2005 Wiley-Liss, Inc.
  • (PMID = 16161045.001).
  • [ISSN] 0020-7136
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 17054; United States / NCI NIH HHS / CA / CA 47082; United States / NCI NIH HHS / CN / N01 CN 25403; United States / NCI NIH HHS / CA / R01 CA 51117-03
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Amides; 0 / Amines
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6. Inda MM, Castresana JS: RASSF1A promoter is highly methylated in primitive neuroectodermal tumors of the central nervous system. Neuropathology; 2007 Aug;27(4):341-6
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  • [Title] RASSF1A promoter is highly methylated in primitive neuroectodermal tumors of the central nervous system.
  • Primitive neuroectodermal tumors (PNET) of the central nervous system can be divided into infratentorial PNET or medulloblastoma (MB), and supratentorial (sPNET) tumors.
  • The RASSF1A (Ras Association Domain Family Protein 1) gene, located at 3p21.3, is highly methylated in multiple primary tumor samples, including neuroblastoma.
  • In order to define whether there are genetic differences in the methylation frequency of RASSF1A between MB and sPNET, we analyzed 32 PNET paraffin-embedded samples (23 MB and 9 sPNET) by methylation specific polymerase chain reaction (MSP).
  • We also analyzed RASSF1A expression by reverse transcription polymerase chain reaction in five PNET cell lines.
  • All PNET cell lines showed lack of RASSF1A expression that was correlated with RASSF1A promoter hypermethylation.
  • Therefore, the inactivation of the RASSF1A gene seems to be an important step in the tumorigenesis of PNET of the central nervous sytem.
  • [MeSH-major] Cerebellar Neoplasms / genetics. Medulloblastoma / genetics. Neuroectodermal Tumors, Primitive / genetics. Promoter Regions, Genetic. Supratentorial Neoplasms / genetics. Tumor Suppressor Proteins / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Child, Preschool. DNA Methylation. Female. Humans. Male. Middle Aged. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 17899687.001).
  • [ISSN] 0919-6544
  • [Journal-full-title] Neuropathology : official journal of the Japanese Society of Neuropathology
  • [ISO-abbreviation] Neuropathology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / RASSF1 protein, human; 0 / Tumor Suppressor Proteins
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7. Alameda F, Lloreta J, Ariza A, Salido M, Espinet B, Baro T, Garcia-Fructoso G, Galito E, Munne A, Cruz Sanchez FF, Sole F, Serrano S: Primitive neuroectodermal tumor of the central nervous system with glial differentiation: a FISH study of an adult case. Clin Neuropathol; 2007 Jan-Feb;26(1):12-6
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  • [Title] Primitive neuroectodermal tumor of the central nervous system with glial differentiation: a FISH study of an adult case.
  • Primitive neuroectodermal tumors (PNETs) of the central nervous system (CNS), a rare occurrence in adults, may show glial differentiation and can be misinterpreted as pure astrocytic neoplasms.
  • We present the case of an adult in which we performed a FISH study of both the glial and neuronal components.
  • [MeSH-major] Brain Neoplasms / genetics. Brain Neoplasms / pathology. Chromosomes, Human, Pair 17 / genetics. Neuroectodermal Tumors, Primitive / genetics. Neuroectodermal Tumors, Primitive / pathology. Trisomy / genetics
  • [MeSH-minor] Adult. Humans. In Situ Hybridization, Fluorescence. Male

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  • (PMID = 17290931.001).
  • [ISSN] 0722-5091
  • [Journal-full-title] Clinical neuropathology
  • [ISO-abbreviation] Clin. Neuropathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
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8. Behdad A, Perry A: Central nervous system primitive neuroectodermal tumors: a clinicopathologic and genetic study of 33 cases. Brain Pathol; 2010 Mar;20(2):441-50
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  • [Title] Central nervous system primitive neuroectodermal tumors: a clinicopathologic and genetic study of 33 cases.
  • Central nervous system (CNS) primitive neuroectodermal tumors (PNETs) include supratentorial, brain stem, and spinal cord tumors with medulloblastoma-like histopathology.
  • After re-diagnosis of three infantile cases as atypical teratoid/rhabdoid tumor (AT/RT), 33 remaining CNS PNETs were retrieved for clinicopathologic and fluorescence in situ hybridization studies.
  • We conclude that in CNS PNET: (i) routine application of INI1 immunohistochemistry helps rule out AT/RT, particularly in infants;.
  • (iii) involvement of CNS parenchyma by Ewing sarcoma/peripheral PNET is rare enough that EWS gene testing is not necessary unless significant dural involvement is present; and (iv) both anaplastic/large cell features and polysomies of 2 and 8 are associated with more aggressive clinical behavior.
  • [MeSH-major] Brain Neoplasms / genetics. Brain Neoplasms / pathology. Neuroectodermal Tumors, Primitive / genetics. Neuroectodermal Tumors, Primitive / pathology. Spinal Cord Neoplasms / genetics. Spinal Cord Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Aneuploidy. Child. Child, Preschool. Chromosomes, Human, Pair 2. Chromosomes, Human, Pair 22. Chromosomes, Human, Pair 8. Female. Humans. Infant. Male. Middle Aged. Nuclear Proteins / genetics. Oncogene Proteins / genetics. RNA-Binding Protein EWS / genetics. Rhabdoid Tumor / diagnosis. Rhabdoid Tumor / genetics. Rhabdoid Tumor / pathology. Teratoma / diagnosis. Teratoma / genetics. Teratoma / pathology. Young Adult

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  • (PMID = 19725831.001).
  • [ISSN] 1750-3639
  • [Journal-full-title] Brain pathology (Zurich, Switzerland)
  • [ISO-abbreviation] Brain Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / MYCN protein, human; 0 / Nuclear Proteins; 0 / Oncogene Proteins; 0 / RNA-Binding Protein EWS
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9. Mobley BC, Roulston D, Shah GV, Bijwaard KE, McKeever PE: Peripheral primitive neuroectodermal tumor/Ewing's sarcoma of the craniospinal vault: case reports and review. Hum Pathol; 2006 Jul;37(7):845-53
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  • [Title] Peripheral primitive neuroectodermal tumor/Ewing's sarcoma of the craniospinal vault: case reports and review.
  • The peripheral primitive neuroectodermal tumor/Ewing's sarcoma family tumor (pPNET/ESFT) group includes small round cell tumors of the bone, soft tissue, and nerve with morphological attributes of the germinal neuroepithelium.
  • Peripheral PNETs/ESFTs also occur within the craniospinal vault, a region including the central nervous system, the meninges, and the cranial and spinal nerve roots.
  • An intradural tumor arising from the nerve roots of the cauda equina was discovered in a 32-year-old man presenting with radiculopathic back pain and lower-extremity weakness.
  • [MeSH-major] Brain Neoplasms / pathology. Cauda Equina / pathology. Neuroectodermal Tumors, Primitive, Peripheral / pathology. Peripheral Nervous System Neoplasms / pathology. Sarcoma, Ewing / pathology
  • [MeSH-minor] Adult. Antigens, CD / metabolism. Back Pain / etiology. Blotting, Southern. Cell Adhesion Molecules / metabolism. Chromosome Aberrations. Chromosomes, Human, Pair 22 / genetics. Diagnosis, Differential. Headache / etiology. Humans. Male. Meningioma / pathology. Oncogene Proteins, Fusion / genetics. Proto-Oncogene Protein c-fli-1 / genetics. RNA-Binding Protein EWS. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 16784984.001).
  • [ISSN] 0046-8177
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules; 0 / EWS-FLI fusion protein; 0 / Oncogene Proteins, Fusion; 0 / Proto-Oncogene Protein c-fli-1; 0 / RNA-Binding Protein EWS
  • [Number-of-references] 68
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10. De Tommasi A, De Tommasi C, Occhiogrosso G, Cimmino A, Parisi M, Sanguedolce F, Ciappetta P: Primary intramedullary primitive neuroectodermal tumor (PNET)--case report and review of the literature. Eur J Neurol; 2006 Mar;13(3):240-3
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  • [Title] Primary intramedullary primitive neuroectodermal tumor (PNET)--case report and review of the literature.
  • Spinal primitive neuroectodermal tumors (PNET) are very rare tumors, and intramedullary localization is even less common.
  • Indeed, amongst the 23 cases of the spinal PNET described in the literature, only eight cases had an intramedullary localization.
  • They have been considered to arise from a neoplastic transformation of primitive neuroepithelial cells, thereby making their presence possible in any part of the central nervous system.
  • The described case is that of a 38-year-old man with a primary intra-extramedullary PNET, treated by laminectomy, open biopsy and chemotherapy.
  • The patient, 18 months after the onset of his symptomatology, died without cerebral tumor involvement.
  • [MeSH-major] Brain Neoplasms. Laminectomy / methods. Neuroectodermal Tumors, Primitive
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Female. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Middle Aged

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  • (PMID = 16618339.001).
  • [ISSN] 1351-5101
  • [Journal-full-title] European journal of neurology
  • [ISO-abbreviation] Eur. J. Neurol.
  • [Language] eng
  • [Publication-type] Case Reports; Comparative Study; Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 25
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11. Ohgaki K, Horiuchi K, Mizutani S, Sato M, Kondo Y: Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney that responded to low-dose chemotherapy with ifosfamide, etoposide, and doxorubicin. Int J Clin Oncol; 2010 Apr;15(2):210-4
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  • [Title] Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney that responded to low-dose chemotherapy with ifosfamide, etoposide, and doxorubicin.
  • A primitive neuroectodermal tumor (PNET) is a small round cell tumor that arises from the nerve crest.
  • This tumor usually occurs in the central nervous system or soft tissue, but it can occur in the kidney in rare cases.
  • Herein we report a case with severe multiple liver metastases after surgery for right renal PNET.
  • Hemorrhage in a right renal malignant tumor was diagnosed, and radical nephrectomy was performed.
  • Some of the cells formed a rosette structure and the tumor cells were positive for CD99, leading to diagnosis of PNET.
  • After this treatment, residual tumor was removed, but the tumor cells were absent histologically.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / surgery. Liver Neoplasms / drug therapy. Nephrectomy. Neuroectodermal Tumors, Primitive / drug therapy. Sarcoma, Ewing / drug therapy
  • [MeSH-minor] Biopsy. Doxorubicin / administration & dosage. Etoposide / administration & dosage. Humans. Ifosfamide / administration & dosage. Male. Positron-Emission Tomography. Tomography, X-Ray Computed. Treatment Outcome. Young Adult

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  • (PMID = 20186557.001).
  • [ISSN] 1437-7772
  • [Journal-full-title] International journal of clinical oncology
  • [ISO-abbreviation] Int. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; UM20QQM95Y / Ifosfamide
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12. Johnson MD, Moots PL, Zhuang Z, Weil RJ: Molecular genetic analysis of a primitive neuroectodermal tumor arising after intracranial radiation and chemotherapy for leukemia. Ann Clin Lab Sci; 2009;39(3):295-302
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  • [Title] Molecular genetic analysis of a primitive neuroectodermal tumor arising after intracranial radiation and chemotherapy for leukemia.
  • Primitive neuroectodermal tumors are aggressive tumors of the central nervous system (CNS), yet their etiology remains unclear.
  • We report a case of a primitive neuroectodermal tumor (PNET) arising in the cerebellum and pons 7 yr after intracranial radiation and chemotherapy for leukemia involving the CNS.
  • [MeSH-major] Cranial Irradiation / adverse effects. Neuroectodermal Tumors, Primitive / etiology. Neuroectodermal Tumors, Primitive / genetics. Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy. Precursor Cell Lymphoblastic Leukemia-Lymphoma / radiotherapy
  • [MeSH-minor] Adult. Bone Marrow Transplantation. Brain / pathology. Chromosomes, Human, Pair 10 / genetics. Chromosomes, Human, Pair 17 / genetics. Humans. Loss of Heterozygosity / genetics. Magnetic Resonance Imaging. Male. Methotrexate / administration & dosage. Methotrexate / therapeutic use. PTEN Phosphohydrolase / genetics. Proto-Oncogene Proteins c-ret / genetics. Tumor Suppressor Protein p53 / genetics. Whole-Body Irradiation

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  • (PMID = 19667415.001).
  • [ISSN] 1550-8080
  • [Journal-full-title] Annals of clinical and laboratory science
  • [ISO-abbreviation] Ann. Clin. Lab. Sci.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Tumor Suppressor Protein p53; EC 2.7.10.1 / Proto-Oncogene Proteins c-ret; EC 2.7.10.1 / RET protein, human; EC 3.1.3.48 / PTEN protein, human; EC 3.1.3.67 / PTEN Phosphohydrolase; YL5FZ2Y5U1 / Methotrexate
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13. Perry A, Miller CR, Gujrati M, Scheithauer BW, Zambrano SC, Jost SC, Raghavan R, Qian J, Cochran EJ, Huse JT, Holland EC, Burger PC, Rosenblum MK: Malignant gliomas with primitive neuroectodermal tumor-like components: a clinicopathologic and genetic study of 53 cases. Brain Pathol; 2009 Jan;19(1):81-90
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  • [Title] Malignant gliomas with primitive neuroectodermal tumor-like components: a clinicopathologic and genetic study of 53 cases.
  • Central nervous system neoplasms with combined features of malignant glioma and primitive neuroectodermal tumor (MG-PNET) are rare, poorly characterized, and pose diagnostic as well as treatment dilemmas.
  • The PNET-like component consisted of sharply demarcated hypercellular nodules with evidence of neuronal differentiation.
  • Within the primitive element, N-myc or c-myc gene amplifications were seen in 43%.
  • Cerebrospinal fluid (CSF) dissemination developed in eight patients, with response to PNET-like therapy occurring in at least three.
  • We conclude that the primitive component of the MG-PNET: (i) arises within a pre-existing MG, most often a secondary glioblastoma;.
  • (ii) may represent a metaplastic process or expansion of a tumor stem/progenitor cell clone;.
  • [MeSH-major] Glioma / pathology. Neuroectodermal Tumors, Primitive / pathology. Proto-Oncogene Proteins / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Antineoplastic Agents, Alkylating / therapeutic use. Brain Neoplasms / genetics. Brain Neoplasms / pathology. Brain Neoplasms / therapy. Combined Modality Therapy. Dacarbazine / analogs & derivatives. Dacarbazine / therapeutic use. Female. Follow-Up Studies. Genes, myc / genetics. Humans. In Situ Hybridization, Fluorescence. Medulloblastoma / genetics. Medulloblastoma / pathology. Medulloblastoma / therapy. Neoplasm Metastasis. Neoplasms, Multiple Primary / genetics. Neoplasms, Multiple Primary / pathology. Neoplasms, Multiple Primary / therapy. Prognosis. Radiotherapy / methods. Treatment Outcome. Young Adult

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  • (PMID = 18452568.001).
  • [ISSN] 1750-3639
  • [Journal-full-title] Brain pathology (Zurich, Switzerland)
  • [ISO-abbreviation] Brain Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 0 / Proto-Oncogene Proteins; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide
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14. Theeler BJ, Keylock J, Yoest S, Forouhar M: Ewing's sarcoma family tumors mimicking primary central nervous system neoplasms. J Neurol Sci; 2009 Sep 15;284(1-2):186-9
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  • [Title] Ewing's sarcoma family tumors mimicking primary central nervous system neoplasms.
  • Ewing's sarcoma family tumors (ESFTs) and embyronal tumors of the central nervous system are malignant primitive neuroectodermal tumors (PNETs) that can arise in the central nervous system, bones, or soft tissues.
  • When ESFTs involve the central nervous system or nearby structures the diagnosis depends on cytogenetics and immunohistochemistry as these tumors can appear otherwise histologically identical to central PNETs.
  • We present two cases of isolated central nervous system presentations of ESFTs mimicking primary central nervous system neoplasms.
  • [MeSH-major] Central Nervous System Neoplasms / diagnosis. Neuroectodermal Tumors, Primitive / diagnosis. Sarcoma, Ewing / diagnosis. Skull Neoplasms / diagnosis. Spinal Neoplasms / diagnosis. Temporal Bone / pathology. Thoracic Vertebrae
  • [MeSH-minor] Adolescent. Adult. Antigens, CD / analysis. Biomarkers, Tumor / analysis. Calmodulin-Binding Proteins / genetics. Cell Adhesion Molecules / analysis. Diagnosis, Differential. Diffusion Magnetic Resonance Imaging. Epidural Space. Female. Headache / etiology. Humans. Male. Memory Disorders / etiology. RNA-Binding Proteins / genetics. Spinal Cord Compression / etiology. Tomography, X-Ray Computed

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  • (PMID = 19394051.001).
  • [ISSN] 1878-5883
  • [Journal-full-title] Journal of the neurological sciences
  • [ISO-abbreviation] J. Neurol. Sci.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Biomarkers, Tumor; 0 / CD99 protein, human; 0 / Calmodulin-Binding Proteins; 0 / Cell Adhesion Molecules; 0 / EWSR1 protein, human; 0 / RNA-Binding Proteins
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15. Wang Z, Fan QH, Yu MN, Zhang WM: [Clinicopathologic and immunohistochemical study of atypical teratoid/rhabdoid tumor of central nervous system]. Zhonghua Bing Li Xue Za Zhi; 2006 Aug;35(8):458-61
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  • [Title] [Clinicopathologic and immunohistochemical study of atypical teratoid/rhabdoid tumor of central nervous system].
  • OBJECTIVE: To study the clinicopathologic features and differential diagnosis of atypical teratoid/rhabdoid tumor (AT/RT) occurring in the central nervous system.
  • RESULTS: Histologically, AT/RT was characterized by the presence of rhabdoid cells associated with various degrees of primitive neuroectodermal, epithelial or mesenchymal differentiation.
  • The tumor cells were positive for vimentin, CD99, epithelial membrane antigen, cytokeratin, glial fibrillary acidic protein, S-100 protein, neurofilament, desmin and smooth muscle actin.
  • CONCLUSIONS: AT/RT is a highly malignant tumor occurring in the central nervous system.
  • The tumor is characterized by a heterogeneous histologic and immunohistochemical phenotype.
  • It needs to be distinguished from a number of central nervous system tumors, including medulloblastoma, primitive neuroectodermal tumor, germ cell neoplasm and rhabdoid meningioma.
  • [MeSH-major] Brain Neoplasms / pathology. Rhabdoid Tumor / pathology. Teratoma / pathology
  • [MeSH-minor] Actins / analysis. Adult. Antigens, CD / analysis. Cell Adhesion Molecules / analysis. Child, Preschool. Desmin / analysis. Glial Fibrillary Acidic Protein / analysis. Humans. Immunohistochemistry. Keratins / analysis. Male. Mucin-1 / analysis. Muscle, Smooth / chemistry. Neurofilament Proteins / analysis. S100 Proteins / analysis. Vimentin / analysis

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  • (PMID = 17069697.001).
  • [ISSN] 0529-5807
  • [Journal-full-title] Zhonghua bing li xue za zhi = Chinese journal of pathology
  • [ISO-abbreviation] Zhonghua Bing Li Xue Za Zhi
  • [Language] chi
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Actins; 0 / Antigens, CD; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules; 0 / Desmin; 0 / Glial Fibrillary Acidic Protein; 0 / Mucin-1; 0 / Neurofilament Proteins; 0 / S100 Proteins; 0 / Vimentin; 68238-35-7 / Keratins
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16. Yasuda K, Taguchi H, Sawamura Y, Ikeda J, Aoyama H, Fujieda K, Ishii N, Kashiwamura M, Iwasaki Y, Shirato H: Low-dose craniospinal irradiation and ifosfamide, cisplatin and etoposide for non-metastatic embryonal tumors in the central nervous system. Jpn J Clin Oncol; 2008 Jul;38(7):486-92
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  • [Title] Low-dose craniospinal irradiation and ifosfamide, cisplatin and etoposide for non-metastatic embryonal tumors in the central nervous system.
  • OBJECTIVE: The current study was conducted to evaluate the effects of low-dose craniospinal irradiation (CSI) combined with chemotherapy on non-metastatic embryonal tumors in the central nervous system (CNS), including medulloblastoma and supra-tentorial primitive neuroectodermal tumors (ST-PNET).
  • The dose to the primary tumor bed was 39.6-54 Gy.
  • Both 5-year OAS and PFS were 82% (CI: 59-100%) for the patients with medulloblastoma and 80% (CI: 45-100%) for the patients with ST-PNET.
  • CONCLUSION: Low-dose CSI and ICE chemotherapy may have a role as a treatment option for a subset of patients with non-metastatic embryonal tumors in the CNS.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Brain Neoplasms / therapy. Cranial Irradiation. Medulloblastoma / therapy. Neuroectodermal Tumors, Primitive / therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Cisplatin / administration & dosage. Combined Modality Therapy. Dose-Response Relationship, Radiation. Etoposide / administration & dosage. Female. Humans. Ifosfamide / administration & dosage. Infant. Male. Radiotherapy Dosage. Supratentorial Neoplasms / pathology. Supratentorial Neoplasms / therapy. Survival Analysis

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  • (PMID = 18573848.001).
  • [ISSN] 1465-3621
  • [Journal-full-title] Japanese journal of clinical oncology
  • [ISO-abbreviation] Jpn. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; Q20Q21Q62J / Cisplatin; UM20QQM95Y / Ifosfamide
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17. Kordes U, Hagel C: Expression of SOX9 and SOX10 in central neuroepithelial tumor. J Neurooncol; 2006 Nov;80(2):151-5
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  • [Title] Expression of SOX9 and SOX10 in central neuroepithelial tumor.
  • Pediatric and adult high grade tumors display strong nuclear staining for both SOX9 and SOX10 (astrocytic, oligodendroglial and primitive neuroectodermal tumors).
  • [MeSH-major] Central Nervous System Neoplasms / metabolism. DNA-Binding Proteins / biosynthesis. DNA-Binding Proteins / genetics. High Mobility Group Proteins / biosynthesis. High Mobility Group Proteins / genetics. Neoplasms, Neuroepithelial / metabolism. Transcription Factors / biosynthesis. Transcription Factors / genetics
  • [MeSH-minor] Adolescent. Adult. Astrocytes / metabolism. Cell Nucleus / pathology. Child. Child, Preschool. Gene Expression Regulation, Neoplastic / genetics. Gliosis / pathology. Humans. Immunohistochemistry. Oligodendroglia / metabolism. Paraffin Embedding. SOX9 Transcription Factor. SOXE Transcription Factors

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  • (PMID = 16791471.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / DNA-Binding Proteins; 0 / High Mobility Group Proteins; 0 / SOX10 protein, human; 0 / SOX9 Transcription Factor; 0 / SOX9 protein, human; 0 / SOXE Transcription Factors; 0 / Transcription Factors
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18. Fukuda T, Akiyama N, Ikegami M, Takahashi H, Sasaki A, Oka H, Komori T, Tanaka Y, Nakazato Y, Akimoto J, Tanaka M, Okada Y, Saito S: Expression of hydroxyindole-O-methyltransferase enzyme in the human central nervous system and in pineal parenchymal cell tumors. J Neuropathol Exp Neurol; 2010 May;69(5):498-510
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  • [Title] Expression of hydroxyindole-O-methyltransferase enzyme in the human central nervous system and in pineal parenchymal cell tumors.
  • Pineal parenchymal tumor (PPT) cells usually show immunoreactivity for synaptophysin, neuron-specific enolase, neurofilament protein, class III beta-tubulin, tau protein, PGP9.5, chromogranin, serotonin, retinal S-antigen, and rhodopsin, but these markers are not specific for PPTs.
  • We hypothesized that HIOMT could serve as a tumor marker of PPTs, and we investigated HIOMT localization and HIOMT expression in samples of normal human tissue and in PPTs, primitive neuroectodermal tumors, and medulloblastomas.
  • The proportions of HIOMT-immunoreactive cells successively decreased in the following tumors: pineocytoma, pineal parenchymal tumor of intermediate differentiation, and pineoblastoma.
  • A few HIOMT-immunoreactive cells were observed in one of 6 primitive neuroectodermal tumors and 23 of 42 medulloblastomas.
  • [MeSH-major] Acetylserotonin O-Methyltransferase / metabolism. Brain Neoplasms / pathology. Central Nervous System / enzymology. Pineal Gland / pathology. Pinealoma / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Animals. Arrestin / metabolism. Cell Line, Tumor. Child. Child, Preschool. Eye Proteins / genetics. Eye Proteins / metabolism. Female. Green Fluorescent Proteins / genetics. Humans. Infant. Male. Middle Aged. Nerve Tissue Proteins / metabolism. Neurons / metabolism. Plant Lectins / metabolism. RNA, Messenger / metabolism. Retina / pathology. Transfection / methods

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  • (PMID = 20418777.001).
  • [ISSN] 1554-6578
  • [Journal-full-title] Journal of neuropathology and experimental neurology
  • [ISO-abbreviation] J. Neuropathol. Exp. Neurol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Arrestin; 0 / Eye Proteins; 0 / Nerve Tissue Proteins; 0 / Plant Lectins; 0 / RNA, Messenger; 0 / Ricinus communis agglutinin-1; 0 / enhanced green fluorescent protein; 147336-22-9 / Green Fluorescent Proteins; EC 2.1.1.4 / Acetylserotonin O-Methyltransferase
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19. Mühlisch J, Bajanowski T, Rickert CH, Roggendorf W, Würthwein G, Jürgens H, Frühwald MC: Frequent but borderline methylation of p16 (INK4a) and TIMP3 in medulloblastoma and sPNET revealed by quantitative analyses. J Neurooncol; 2007 May;83(1):17-29
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  • Certain risk groups among tumors of the central nervous system (CNS) in children take an almost inevitably fatal course.
  • Therapeutic and diagnostic implications urge into depth analyses of methylation as a mechanism, which might fill some of the gaps of our understanding of brain tumor origin.
  • [MeSH-major] Brain Neoplasms / genetics. Cerebellar Neoplasms / genetics. DNA Methylation. Genes, p16. Medulloblastoma / genetics. Neuroectodermal Tumors, Primitive / genetics. Tissue Inhibitor of Metalloproteinase-3 / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Apoptosis Regulatory Proteins / genetics. Calcium-Calmodulin-Dependent Protein Kinases / genetics. Child. Child, Preschool. Death-Associated Protein Kinases. Female. Gene Silencing. Humans. Infant. Male. Middle Aged. Nerve Tissue Proteins / genetics. Receptors, Immunologic / genetics

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  • (PMID = 17206475.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Apoptosis Regulatory Proteins; 0 / Nerve Tissue Proteins; 0 / Receptors, Immunologic; 0 / TIMP3 protein, human; 0 / Tissue Inhibitor of Metalloproteinase-3; 0 / roundabout protein; EC 2.7.11.1 / DAPK1 protein, human; EC 2.7.11.1 / Death-Associated Protein Kinases; EC 2.7.11.17 / Calcium-Calmodulin-Dependent Protein Kinases
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20. Santagata S, Hornick JL, Ligon KL: Comparative analysis of germ cell transcription factors in CNS germinoma reveals diagnostic utility of NANOG. Am J Surg Pathol; 2006 Dec;30(12):1613-8
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  • [Title] Comparative analysis of germ cell transcription factors in CNS germinoma reveals diagnostic utility of NANOG.
  • To assess the diagnostic utility of NANOG in central nervous system (CNS) germ cell tumors, we analyzed its expression by immunohistochemistry in a series of 12 CNS germinomas and compared its expression with other stem cell markers.
  • Strong nuclear expression of NANOG was demonstrated in >90% of the tumor cells in all cases.
  • NANOG was not detected in tumor types frequently considered in the differential diagnosis of CNS germinoma: pineoblastoma, primitive neuroectodermal tumors, medulloblastoma, lymphoma, pituitary adenoma, atypical teratoid/rhabdoid tumor, Langerhans cell histiocytosis, and gliomas.
  • These findings demonstrate that NANOG is a sensitive and specific marker of CNS germinoma.
  • These findings also suggest a potential biologic role for NANOG in maintenance of CNS germinoma.
  • [MeSH-minor] Adolescent. Adult. Alkaline Phosphatase / metabolism. Biomarkers, Tumor / metabolism. Cell Nucleus / metabolism. Child. HMGB Proteins / metabolism. Humans. Isoenzymes / metabolism. Octamer Transcription Factor-3 / metabolism. SOXB1 Transcription Factors. Transcription Factors / metabolism

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  • (PMID = 17122519.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Grant] United States / NINDS NIH HHS / NS / NS047213
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / DNA-Binding Proteins; 0 / HMGB Proteins; 0 / Homeodomain Proteins; 0 / Isoenzymes; 0 / NANOG protein, human; 0 / Octamer Transcription Factor-3; 0 / POU5F1 protein, human; 0 / SOX2 protein, human; 0 / SOXB1 Transcription Factors; 0 / Transcription Factors; 0 / germ-cell AP isoenzyme; EC 3.1.3.1 / Alkaline Phosphatase
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21. Sugita Y, Nakamura Y, Yamamoto M, Ogasawara S, Ohshima K, Shigemori M: Expression of KIAA 0864 protein in neuroepithelial tumors: an analysis based on the presence of monoclonal antibody HFB-16. J Neurooncol; 2008 Sep;89(2):151-8

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  • Among the 55 NETs, a moderate-to-intense KA protein immunoreactivity was observed in 8 of 8 medulloblastomas, 1 of 1 central nervous system supratentorial primitive neuroectodermal tumor (CNS supratentorial PNET), 4 of 4 retinoblastomas, 1 of 1 neuroblastoma, 8 of 8 central neurocytomas, 4 of 4 oligodendrogliomas, 4 of 4 oligoastrocytomas, 1 of 1 extraventricular neurocytoma, and 1 of 1 gangliocytoma.
  • These results indicate that the antibody HFB-16 could be a useful marker for neuronal tumors and primitive neuroectodermal tumors that may originate from immature neural progenitor cells.
  • In addition, it could be a useful tool for performing the differential diagnosis between GBs and CNS supratentorial PNET.
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Antibodies, Monoclonal. Child. Child, Preschool. Female. Humans. Male. Middle Aged. Retrospective Studies

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  • (PMID = 18458818.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Guanine Nucleotide Exchange Factors; 0 / RASGRP3 protein, human
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22. Harms D, Zahn S, Göbel U, Schneider DT: Pathology and molecular biology of teratomas in childhood and adolescence. Klin Padiatr; 2006 Nov-Dec;218(6):296-302
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  • The biologic behaviour of teratomas depends on various interdependent clinical and epidemiologic variables such as the age at diagnosis, sex, tumor site, histology which all correlate to different cytogenetic and molecular biologic aberrations.
  • The Kiel Pediatric Tumor Registry includes 541 teratoma specimens, and among these, the most frequent tumor sites (in descending order) are: the sacrococcygeal region (33.8 %), the ovaries (31.2 %) and the testes (10.5 %).
  • Rare localizations include the mediastinum, the retroperitoneum, the head and neck region as well as the central nervous system.
  • In immature teratomas, primitive neuroectodermal structures predominate.
  • According to the grading system (Gonzalez-Crussi, 1982), mature teratomas (G0) are more frequent (54.5 %) than immature teratomas (G1-G3, 45.5 %).
  • The frequency of additional microscopic foci of malignant yolk sac tumor correlates with the grade of immaturity.
  • In sacrococcygeal teratomas, the yolk sac tumor microfoci may give rise to a malignant relapse after incomplete resection.
  • Here, molecular genetic analysis has demonstrated the origin of the somatic malignancy from a malignant transformation within the germ cell tumor with retention of the cytogenetic changes characteristic of malignant germ cell tumors.
  • [MeSH-minor] Adolescent. Adult. Age Factors. Child. Chromosome Aberrations. Chromosome Deletion. Cytogenetic Analysis. Endodermal Sinus Tumor / epidemiology. Endodermal Sinus Tumor / pathology. Female. Germany / epidemiology. Heterozygote. Humans. Incidence. Infant. Infant, Newborn. Male. Ovary / pathology. Puberty. Testis / pathology

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  • (PMID = 17080330.001).
  • [ISSN] 0300-8630
  • [Journal-full-title] Klinische Pädiatrie
  • [ISO-abbreviation] Klin Padiatr
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
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23. Benesch M, Siegler N, Hoff Kv, Lassay L, Kropshofer G, Müller H, Sommer C, Rutkowski S, Fleischhack G, Urban C: Safety and toxicity of intrathecal liposomal cytarabine (Depocyte) in children and adolescents with recurrent or refractory brain tumors: a multi-institutional retrospective study. Anticancer Drugs; 2009 Oct;20(9):794-9
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  • Nineteen heavily pretreated patients (males, n = 14; females, n = 5; median age at diagnosis 8.5 years; range, 1.4-22 years) were given intrathecal liposomal cytarabine on a compassionate use basis for recurrent refractory medulloblastoma (n = 12), mixed germ cell tumor (n = 2), central nervous system primitive neuroectodermal tumors of the pons (n = 1), anaplastic ependymoma (n = 1), anaplastic oligodendroglioma (n = 1), atypical teratoid rhabdoid tumor (n = 1), or rhabdoid papillary meningioma (n = 1).
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Compassionate Use Trials. Delayed-Action Preparations. Drug Resistance, Neoplasm. Female. Humans. Infant. Injections, Spinal. Liposomes / administration & dosage. Male. Retrospective Studies. Salvage Therapy. Young Adult

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  • (PMID = 19617818.001).
  • [ISSN] 1473-5741
  • [Journal-full-title] Anti-cancer drugs
  • [ISO-abbreviation] Anticancer Drugs
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antimetabolites, Antineoplastic; 0 / Delayed-Action Preparations; 0 / Liposomes; 04079A1RDZ / Cytarabine
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24. Marcus KJ, Goumnerova L, Billett AL, Lavally B, Scott RM, Bishop K, Xu R, Young Poussaint T, Kieran M, Kooy H, Pomeroy SL, Tarbell NJ: Stereotactic radiotherapy for localized low-grade gliomas in children: final results of a prospective trial. Int J Radiat Oncol Biol Phys; 2005 Feb 1;61(2):374-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • All patients underwent biopsy for diagnosis, with the exception of patients with neurofibromatosis and radiographic evidence of an optic system tumor.
  • The target volume generally included the preoperative tumor plus a 2-mm margin for the planning target volume.
  • Five patients, all with optic system/hypothalamic primary tumors, developed central nervous system dissemination 1.0-7.4 years after SRT.
  • One patient developed a presumed radiation-induced primitive neuroectodermal tumor 6 years after initial treatment.
  • Six patients died, three of dissemination, two of progression to higher grade tumors, and one of a secondary radiation-induced tumor.
  • All 6 cases of local progression were within the primary tumor bed at the time of progression and had received the full prescription dose.
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Confidence Intervals. Disease Progression. Disease-Free Survival. Female. Humans. Male. Neoplasm Recurrence, Local / drug therapy. Prospective Studies. Radiotherapy Dosage

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  • (PMID = 15667955.001).
  • [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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25. Inskip PD, Curtis RE: New malignancies following childhood cancer in the United States, 1973-2002. Int J Cancer; 2007 Nov 15;121(10):2233-40
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Most common were subsequent primary cancers of the female breast, central nervous system, bone, thyroid gland and soft tissue, as well as cutaneous melanoma and acute non-lymphocytic leukemia (ANLL).
  • The greatest risks of subsequent cancers occurred among patients diagnosed previously with Hodgkin lymphoma (HL), Ewing sarcoma, primitive neuroectodermal tumor, or retinoblastoma.
  • [MeSH-minor] Adolescent. Adult. Age Distribution. Child. Child, Preschool. Female. Follow-Up Studies. Humans. Infant. Male. Neoplasm Metastasis. Risk Factors. Sex Characteristics. Time Factors. United States / epidemiology

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17557301.001).
  • [ISSN] 1097-0215
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
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