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1. Gaspar N, Hartmann O, Munzer C, Bergeron C, Millot F, Cousin-Lafay L, Babin-Boilletot A, Blouin P, Pajot C, Coze C: Neuroblastoma in adolescents. Cancer; 2003 Jul 15;98(2):349-55
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  • [Title] Neuroblastoma in adolescents.
  • Previous studies of patients who were treated with less intensive chemotherapy regimens relative to currently available regimens suggested that adolescents survived longer than younger children, and this finding was related to a lack of myc-N amplification.
  • METHODS: The authors investigated the presentation, treatment, and outcome in 28 adolescent patients who were enrolled in studies of the French Society of Pediatric Oncology during the period from 1987 to 1999 and who were older than age 10 years at the time they were diagnosed with neuroblastoma.
  • RESULTS: None of the six patients with Stage I-II disease either developed recurrent disease or died.
  • At 5 years, disease progression was high (progression-free survival [PFS], 28%) for the 9 adolescents with Stage III disease, but so was survival (overall survival [OS], 86%).
  • The 13 adolescent patients with metastatic neuroblastoma had very poor outcomes (PFS, 18%; OS, 27%).
  • Despite intensive therapy, advanced neuroblastoma appeared to carry a poorer prognosis in adolescent patients compared with children, although patients with Stage III disease had a more indolent course.
  • No difference was found between adolescent patients and children regarding the clinical presentation, treatment schedule, or doses and tolerance of chemotherapy.
  • CONCLUSIONS: Adolescent patients with advanced neuroblastoma had less favorable outcomes compared with children, even if survival in adolescents with Stage III disease seemed longer.
  • [MeSH-major] Abdominal Neoplasms / diagnosis. Neuroblastoma / diagnosis
  • [MeSH-minor] Adolescent. Case-Control Studies. Child. Disease-Free Survival. Female. Humans. Male. Neoplasm Recurrence, Local. Neoplasm Staging. Prognosis. Retrospective Studies. Treatment Outcome

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  • [Copyright] Copyright 2003 American Cancer Society.
  • (PMID = 12872356.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] United States
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2. Laprie A, Michon J, Hartmann O, Munzer C, Leclair MD, Coze C, Valteau-Couanet D, Plantaz D, Carrie C, Habrand JL, Bergeron C, Chastagner P, Défachelles AS, Delattre O, Combaret V, Bénard J, Pérel Y, Gandemer V, Rubie H, Neuroblastoma Study Group of the French Society of Pediatric Oncology: High-dose chemotherapy followed by locoregional irradiation improves the outcome of patients with international neuroblastoma staging system Stage II and III neuroblastoma with MYCN amplification. Cancer; 2004 Sep 1;101(5):1081-9
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  • [Title] High-dose chemotherapy followed by locoregional irradiation improves the outcome of patients with international neuroblastoma staging system Stage II and III neuroblastoma with MYCN amplification.
  • BACKGROUND: The objective of this study was to determine whether systemic and regional, intensified treatment can improve the outcome of children who present with a localized neuroblastoma (NB) with MYCN amplification (MNA).
  • METHODS: Between 1990 and 2000, 610 children with localized NB were included in the Localized Neuroblastoma 90 (NBL 90) and NBL 94 study from the French Society of Pediatric Oncology.
  • Among them, 32 children had MNA with Stage II or III NB.
  • During the first period of the study, 20 children (Group A) received postoperative conventional chemotherapy (CT) and/or radiotherapy (RT), depending on each patient's postoperative status.
  • RESULTS: The two groups were comparable with regard to prognostic factors (age, location of the primary lesion, International Neuroblastoma Staging System stage, lymph node invasion) and response to preoperative CT.
  • CONCLUSIONS: Postoperative intensification treatment with HDC, SCR, and locoregional RT resulted in higher survival rates when compared with standard treatment alone and should be considered in the treatment plan for children who are diagnosed with Stage II or III NB and MYCN amplification.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Gene Amplification. Genes, myc. Neuroblastoma / genetics. Neuroblastoma / therapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Combined Modality Therapy. Gamma Rays. Humans. Infant. Infant, Newborn. Lymph Nodes / pathology. Neoplasm Invasiveness. Neoplasm Recurrence, Local. Neoplasm Staging. Postoperative Care. Prognosis. Survival Rate. Treatment Outcome

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  • [Copyright] Copyright 2004 American Cancer Society.
  • (PMID = 15329919.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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3. Modak S, Kushner BH, LaQuaglia MP, Kramer K, Cheung NK: Management and outcome of stage 3 neuroblastoma. Eur J Cancer; 2009 Jan;45(1):90-8
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  • [Title] Management and outcome of stage 3 neuroblastoma.
  • PURPOSE: The management of patients with International Neuroblastoma Staging System (INSS) stage 3 neuroblastoma (NB) is not consistent worldwide.
  • We describe a single centre approach at Memorial Sloan-Kettering Cancer Centre (MSKCC) from 1991 to 2007 that minimises therapy except for those patients with MYCN-amplified NB.
  • RESULTS: Fourteen patients with non-MYCN-amplified tumours were treated with surgery alone (group A) and the remaining 39 (group B) with surgery following chemotherapy that was initiated and administered at non-MSKCC institutions.
  • Chemotherapy was discontinued after surgery in 38/39 of the latter.
  • Patients with MYCN-amplified disease (group C) underwent dose-intensive induction, tumour resection and local radiotherapy: 13 achieved complete or very good partial remission, and 10 received myeloablative chemotherapy.
  • The 10-year EFS and OS for patients with MYCN-amplified neuroblastoma treated with immunotherapy were both 90.9+/-8.7%.
  • CONCLUSION: Patients with MYCN-non-amplified stage 3 NB can be successfully treated with surgery without the need for radiotherapy or continuation of chemotherapy.
  • Combination of dose-intensive chemotherapy, surgery, radiotherapy and immunotherapy was associated with a favourable outcome for most patients with MYCN-amplified stage 3 NB.
  • [MeSH-major] Neuroblastoma / therapy
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Gene Amplification. Humans. Infant. Infant, Newborn. Kaplan-Meier Estimate. Male. N-Myc Proto-Oncogene Protein. Neoplasm Staging. Nuclear Proteins / genetics. Oncogene Proteins / genetics. Retrospective Studies. Survival Rate

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  • (PMID = 18996003.001).
  • [ISSN] 1879-0852
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA134274; United States / NCI NIH HHS / CA / P30 CA134274; United States / NCI NIH HHS / CA / CA106450; United States / NCI NIH HHS / CA / CA72868; United States / NCI NIH HHS / CA / CA61017; United States / NCI NIH HHS / CA / P01 CA106450
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / MYCN protein, human; 0 / N-Myc Proto-Oncogene Protein; 0 / Nuclear Proteins; 0 / Oncogene Proteins
  • [Other-IDs] NLM/ NIHMS88689; NLM/ PMC3727624
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4. Baker DL, Schmidt ML, Cohn SL, Maris JM, London WB, Buxton A, Stram D, Castleberry RP, Shimada H, Sandler A, Shamberger RC, Look AT, Reynolds CP, Seeger RC, Matthay KK, Children’s Oncology Group: Outcome after reduced chemotherapy for intermediate-risk neuroblastoma. N Engl J Med; 2010 Sep 30;363(14):1313-23
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  • [Title] Outcome after reduced chemotherapy for intermediate-risk neuroblastoma.
  • BACKGROUND: The survival rate among patients with intermediate-risk neuroblastoma who receive dose-intensive chemotherapy is excellent, but the survival rate among patients who receive reduced doses of chemotherapy for shorter periods of time is not known.
  • METHODS: We conducted a prospective, phase 3, nonrandomized trial to determine whether a 3-year estimated overall survival of more than 90% could be maintained with reductions in the duration of therapy and drug doses, using a tumor biology-based therapy assignment.
  • Eligible patients had newly diagnosed, intermediate-risk neuroblastoma without MYCN amplification; these patients included infants (<365 days of age) who had stage 3 or 4 disease, children (≥365 days of age) who had stage 3 tumors with favorable histopathological features, and infants who had stage 4S disease with a diploid DNA index or unfavorable histopathological features.
  • Patients who had disease with favorable histopathological features and hyperdiploidy were assigned to four cycles of chemotherapy, and those with an incomplete response or either unfavorable feature were assigned to eight cycles.
  • RESULTS: Between 1997 and 2005, a total of 479 eligible patients were enrolled in this trial (270 patients with stage 3 disease, 178 with stage 4 disease, and 31 with stage 4S disease).
  • The 3-year estimate (±SE) of overall survival for the entire group was 96±1%, with an overall survival rate of 98±1% among patients who had tumors with favorable biologic features and 93±2% among patients who had tumors with unfavorable biologic features.
  • CONCLUSIONS: A very high rate of survival among patients with intermediate-risk neuroblastoma was achieved with a biologically based treatment assignment involving a substantially reduced duration of chemotherapy and reduced doses of chemotherapeutic agents as compared with the regimens used in earlier trials.
  • These data provide support for further reduction in chemotherapy with more refined risk stratification. (Funded by the National Cancer Institute; ClinicalTrials.gov number, NCT00003093. )
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Neuroblastoma / drug therapy
  • [MeSH-minor] Carboplatin / administration & dosage. Child. Child, Preschool. Cyclophosphamide / administration & dosage. Doxorubicin / administration & dosage. Etoposide / administration & dosage. Humans. Infant. Intention to Treat Analysis. Neoplasm Staging. Prospective Studies. Survival Analysis. Treatment Outcome

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  • (PMID = 20879880.001).
  • [ISSN] 1533-4406
  • [Journal-full-title] The New England journal of medicine
  • [ISO-abbreviation] N. Engl. J. Med.
  • [Language] eng
  • [Databank-accession-numbers] ClinicalTrials.gov/ NCT00003093
  • [Grant] United States / NCI NIH HHS / CA / UI0-CA29139; United States / NCI NIH HHS / CA / U10 CA017829-26; United States / NCI NIH HHS / CA / U10 CA098413-09; United States / NCI NIH HHS / CA / U10 CA029139-22; United States / NCI NIH HHS / CA / U10 CA098413; United States / NCI NIH HHS / CA / UI0-CA98413; United States / NCI NIH HHS / CA / UI0-CA98543; United States / NCI NIH HHS / CA / U10 CA029139; United States / NCI NIH HHS / CA / U10 CA098543; United States / NCI NIH HHS / CA / U10 CA098543-04
  • [Publication-type] Clinical Trial, Phase III; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; BG3F62OND5 / Carboplatin
  • [Other-IDs] NLM/ NIHMS246674; NLM/ PMC2993160
  • [Investigator] Greenwood M; Salvi S; Kanwar V; Strother D; Frantz C; Daghistani D; Grewal S; Kretschmar C; Bond M; Mody R; Yanofsky R; Ferguson W; Atkinson M; McMahon D; Michon B; Yu L; Feusner J; Crouse V; Kuerbitz S; Halton J; Ravindranath Y; Broxson E; Dome J; Mascarenhas L; Shen V; Balis F; Lowe E; Bostrom B; Walterhouse D; Perentesis J; Tekautz T; Lee A; Isakoff M; Granger M; Prasannan L; Rodriguez-Galindo C; Chaffee S; Lockhart S; Johnson MC; Kreissman S; Pais R; Katzenstein H; Selsky C; Taylor J; Shad A; Stroud C; Appel B; Finklestein J; Dickens D; Samson Y; Grant R; Fallon R; Schorin M; Hand J; Arceci R; Kiley V; Lobel J; Silva M; Bertolone S; Salman E; Olson J; Bedros A; Kwon JH; Larsen E; McManus M; Louie R; Weinstein H; Arndt C; Portwine C; Lenarsky C; McDonough C; Kraveka J; Johnston J; Hanif I; Jasty R; Kobrinsky N; Estrada J; Gera R; Kelly M; Ricafort R; Chang E; Kirkpatrick G; Termuhlen A; Sandler E; Bernstein J; Ozkaynak M; Kamalakar P; Neely J; Boklan J; Bryant P; Barnette P; Cole C; Roberts W; Matloub YJ; Mitchell T; Schwartz C; Brecher M; Ashley D; Irving H; Felgenhauer J; Chatchawin A; Cameron T; John H; Bassem R; Luis C; Daniel G; Christopher M; Arlene R; Douglas H; Joseph W; Furman W; Smith S; Gowda N; Marina N; Desai SJ; Cherrick I; Lee Y; Ammann R; Beck Popovic M; Bomgaars L; Turner C; Maloney K; McCowage G; Hetherington M; Cohn S; Stein D; Scher C; Fu C; Drachtman R; Reddy A; Wittman B; Becton D; Goldsby R; Ducore J; Smith A; Schmidt M; Tannous R; Kumar M; York T; Keuker C; Neglia J; Megason G; Loew T; Winter S; Gold S; McNall-Knapp R; Ritchey A; Hackney L; Thomas P; Homans A; De Santes K; Winick N; Kuttesch J; Cairney A; Godder K; McLean T; Hayashi R; Chauvenet A; Main C; Weinblatt M; Kupfer G
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5. Ye QD, Tang JY, Pan C, Chen J, Xue HL, Chen J, Dong L, Zhou M, Shen SH: [Therapeutic experience of childhood stage III neuroblastoma]. Zhonghua Yi Xue Za Zhi; 2010 Jun 8;90(22):1556-8
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  • [Title] [Therapeutic experience of childhood stage III neuroblastoma].
  • OBJECTIVE: To evaluate the long-term outcomes of childhood stage III neuroblastoma (NB) and its associated prognostic factors.
  • RESULTS: Thirty children with stage III NB were found among all 101 children with NB.
  • Eleven NB children were treated according to the mediate-risk protocol, 6 children received autologous stem cell transplantation (ASCT) after chemotherapy and 5 patients had no therapy of cis-retinoic acid.
  • On univariate analysis, pathological type, high levels of LDH and ferritin, non-therapy of cis-retinoic acid were associated with a worse survival (chi2 = 9.48, 6.82, 9.17, 9.06, all P < 0.05).
  • As to the multivariate estimates of hazards ratio, high levels of LDH and ferritin, no ASCT and non-therapy of cis-retinoic acid were associated with a worse survival (OR = 3.95, 3.44, 2.64, 1.27, all P < 0.05).
  • CONCLUSION: Stage III NB children with favorable histologic features, normal LDH, normal serum ferritin, receive ASCT, and treated with cis-retinoic acid have a lower risk of relapse.
  • [MeSH-major] Neuroblastoma / drug therapy. Neuroblastoma / pathology
  • [MeSH-minor] Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Neoplasm Staging. Prognosis. Retrospective Studies. Treatment Outcome

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  • (PMID = 20973238.001).
  • [ISSN] 0376-2491
  • [Journal-full-title] Zhonghua yi xue za zhi
  • [ISO-abbreviation] Zhonghua Yi Xue Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] China
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6. Feng C, Tang SQ, Wang JW, Liu Y, Yang G: [Curative effects of the protocol of CDV combined with CiE as pre-operative chemotherapy in high-risk childhood neuroblastoma]. Zhongguo Dang Dai Er Ke Za Zhi; 2009 Nov;11(11):885-7
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  • [Title] [Curative effects of the protocol of CDV combined with CiE as pre-operative chemotherapy in high-risk childhood neuroblastoma].
  • OBJECTIVE: To evaluate the effects and the toxicity of the protocol of CDV combined with CiE as pre-operative chemotherapy in childhood stage IV neuroblastoma.
  • METHODS: The clinical data of 27 children aged from 1.2 to 8 years with neuroblastoma in stage IV was retrospectively studied.
  • Neuroblastoma therapeutic response evaluation criterion and common terminology criteria for adverse events of National Cancer Institute were used to evaluate effects and chemotherapy related toxicity.
  • RESULTS: All patients received the pre-operative chemotherapy.
  • After chemotherapy, 24 patients received operations.
  • The most common chemotherapy related toxicity was bone marrow suppression: grade IV suppression of neutrophils (n = 27), reduction in hemoglobin (III grade, n = 7; IV grade, n = 20) and reduction in platelet (III grade, n = 2; IV grade, n = 25).
  • I or II grade lesions of digestive, liver and kidney were found and could be recovered after therapy.
  • CONCLUSIONS: The protocol of CDV combined with CiE as pre-operative chemotherapy might be effective in children with stage IV neuroblastoma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Neuroblastoma / drug therapy

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  • (PMID = 20113653.001).
  • [ISSN] 1008-8830
  • [Journal-full-title] Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics
  • [ISO-abbreviation] Zhongguo Dang Dai Er Ke Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 7GR28W0FJI / Dacarbazine; 8N3DW7272P / Cyclophosphamide; Q20Q21Q62J / Cisplatin; CVD protocol
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7. Don S, Verrills NM, Liaw TY, Liu ML, Norris MD, Haber M, Kavallaris M: Neuronal-associated microtubule proteins class III beta-tubulin and MAP2c in neuroblastoma: role in resistance to microtubule-targeted drugs. Mol Cancer Ther; 2004 Sep;3(9):1137-46
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  • [Title] Neuronal-associated microtubule proteins class III beta-tubulin and MAP2c in neuroblastoma: role in resistance to microtubule-targeted drugs.
  • Advanced stage neuroblastoma has a poor clinical outcome and microtubule-destabilizing agents, such as the Vinca alkaloids, are an important component in the treatment of this childhood cancer.
  • To date, studies examining the contribution of microtubules and associated proteins to the efficacy of microtubule-destabilizing agents in neuroblastoma have been limited.
  • In this study, BE2-C neuroblastoma cells previously selected for resistance to either vincristine (BE/VCR10) or colchicine (BE/CHCb0.2) were found to display significant decreases in neuronal-specific class III beta-tubulin.
  • Interestingly, vincristine-selected cells exhibited increased levels of polymerized tubulin that were not due to alpha-tubulin and class I, II, or III beta-tubulin mutations.
  • This is the first report describing specific microtubule alterations in neuroblastoma cells resistant to tubulin-targeted agents.
  • The results indicate a need to identify the factors responsible for resistance to tubulin-targeted agents in neuroblastoma so that improved and novel treatment strategies can be developed for this drug refractory disease.
  • [MeSH-major] Drug Resistance, Neoplasm. Microtubule-Associated Proteins / metabolism. Microtubules / drug effects. Neuroblastoma / drug therapy. Neuroblastoma / metabolism. Tubulin / metabolism

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  • (PMID = 15367708.001).
  • [ISSN] 1535-7163
  • [Journal-full-title] Molecular cancer therapeutics
  • [ISO-abbreviation] Mol. Cancer Ther.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / MAP2 protein, human; 0 / MAP4; 0 / Microtubule Proteins; 0 / Microtubule-Associated Proteins; 0 / Phosphoproteins; 0 / Protein Isoforms; 0 / STMN1 protein, human; 0 / Stathmin; 0 / Tubulin; P88XT4IS4D / Paclitaxel
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8. Stefanowicz J, Izycka-Swieszewska E, Drozyńska E, Pienczk J, Połczyńska K, Czauderna P, Sierota D, Bień E, Stachowicz-Stencel T, Kosiak W, Balcerska A: Neuroblastoma and opsoclonus-myoclonus-ataxia syndrome--clinical and pathological characteristics. Folia Neuropathol; 2008;46(3):176-85
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  • [Title] Neuroblastoma and opsoclonus-myoclonus-ataxia syndrome--clinical and pathological characteristics.
  • All cases were in stage II or III of the disease, with no metastases or MYCN amplification.
  • The group included two ganglioneuroblastomas, one ganglioneuroma and one differentiating neuroblastoma.
  • Immunohistochemical analysis of inflammatory infiltrations revealed mixed type populations of lymphocytes with prevalence of the cytotoxic type (CD8 and CD56-positive cells).
  • All patients were treated by surgery alone or with adjuvant chemotherapy with a positive outcome.
  • Children with OMA and neuroblastoma despite a good oncological prognosis often present permanent neurological and developmental deficits.


9. Kato M, Hirata S, Kikuchi A, Ogawa K, Kishimoto H, Hanada R: Neuroblastoma presenting with dilated cardiomyopathy. Pediatr Blood Cancer; 2008 Feb;50(2):391-2
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  • [Title] Neuroblastoma presenting with dilated cardiomyopathy.
  • We report an infant with neuroblastoma who presented with dilated cardiomyopathy.
  • A 3-month-old girl presented with dilated cardiomyopathy diagnosed as stage III neuroblastoma.
  • Since total resection was impossible, chemotherapy was started.
  • Cardiomyopathy was normalized by improvement of neuroblastoma.
  • The prompt improvement of cardiac function following treatment of neuroblastoma suggested that cardiomyopathy in this patient was caused by the increase in catecholamines secreted by neuroblastoma and that reduction in catecholamines by treatment of neuroblastoma led to improvement in the cardiomyopathy.
  • [MeSH-major] Cardiomyopathy, Dilated / etiology. Neuroblastoma / complications

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17066463.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Catecholamines
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10. Meir HM, Balawi I, Nayel H, Yousef MK, Badawood S, Al-Mobarak M: Neuroblastoma. Saudi Med J; 2001 Aug;22(8):674-80
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  • [Title] Neuroblastoma.
  • OBJECTIVE: Due to the poor results achieved on combination chemotherapy and the unproven cost-effectiveness value of myeloablative therapy, the question has been raised; should patients with stage IV neuroblastoma be actively treated?
  • The aim of the current study is to analyze retrospectively treatment results of 43 children with neuroblastoma with special stress on the rate and duration of remission in children with disseminated neuroblastoma.
  • METHODS: Treatment of children with neuroblastoma consisted of surgical removal of the tumor, if possible, followed by chemotherapy for unresectable residual tumor including metastases.
  • Second look surgery was performed to resect residual masses rendered resectable on chemotherapy in the absence of distal metastases.
  • The chemotherapy protocol used in the current study consisted of alternating combination chemotherapy regimens containing, Cyclophosphamide, Vincristine and Doxorubicin, alternating with Cis-platinum and Etoposide.
  • Two patients (5%) were found to have stage IV.
  • Stage III was documented in 5 patients (12%).
  • The bone marrow was the most common site of metastatic deposit, encountered in 23 patients out of the 30 with stage IV disease (77%).
  • Out of the 12 evaluable non-stage IV patients, only one patient (8%) showed treatment failure.
  • Assessment of response by the end of the 6th month from the date of diagnosis revealed that out of the 27 evaluable patients with stage IV, 4 patients achieved complete remission, 7 patients achieved very good partial remission, 8 patients achieved partial remission and 4 patients achieved mixed response.
  • Three patients showed progressive disease on chemotherapy.
  • Assessment of response to treatment by the end of the 12th month from diagnosis revealed that 6 patients (2 complete remissions, 1 very good partial response, 3 partial responses) were maintaining their remission.
  • CONCLUSION: Results of treatment by multiagent chemotherapy regimens used in the current study show that children with neuroblastoma, even those with advanced stages, should receive the benefit of intensive multimodal therapy, even those with partial response to initial therapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Neuroblastoma / drug therapy. Neuroblastoma / surgery
  • [MeSH-minor] Child. Child, Preschool. Cisplatin / administration & dosage. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Disease Progression. Doxorubicin / administration & dosage. Etoposide / administration & dosage. Female. Humans. Infant. Male. Neoplasm Staging. Retrospective Studies. Second-Look Surgery. Survival Rate. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 11573111.001).
  • [ISSN] 0379-5284
  • [Journal-full-title] Saudi medical journal
  • [ISO-abbreviation] Saudi Med J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Saudi Arabia
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; Q20Q21Q62J / Cisplatin
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11. López-Aguilar E, Cerecedo-Díaz F, Rivera-Márquez H, Valdéz-Sánchez M, Sepúlveda-Vildósola AC, Delgado Huerta S, Vera-Hermosillo H, Vázquez-Langle JR, Wanzke del Angel V: [Neuroblastoma: prognostic factors and survival. Experience in Hospital de Pediatria del Centro Medico Nacional del Siglo XXI and review of the literature]. Gac Med Mex; 2003 May-Jun;139(3):209-14
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  • [Title] [Neuroblastoma: prognostic factors and survival. Experience in Hospital de Pediatria del Centro Medico Nacional del Siglo XXI and review of the literature].
  • [Transliterated title] Neuroblastoma: factores pronósticos y sobrevida. Experiencia en el Hospital de Pediatría del Centro Médico Nacional Siglo XXI y revisión de la literatura.
  • Neuroblastoma (NB) is the most frequent extracranial solid tumor in children according to the literature.
  • We included all patients admitted to our hospital during the previous five years and who had not received any treatment.
  • Patients with stages were III and IV received the same chemotherapy alternating with cisplatinum., ifosfamide and etoposide during 12 months as well as massive doses of 131-MIBG and surgical ablation of the remaining tumor when possible.
  • The most important prognostic factors are still considered to be age, stage and histology.
  • [MeSH-major] Neuroblastoma / diagnosis
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Female. Humans. Infant. Infant, Newborn. Male. Mexico. Neoplasm Staging. Prognosis. Prospective Studies. Survival Analysis

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  • (PMID = 12872413.001).
  • [ISSN] 0016-3813
  • [Journal-full-title] Gaceta médica de México
  • [ISO-abbreviation] Gac Med Mex
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Mexico
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12. Biswas G, Kurkure P, Banavali S, Achrekar S, Kulkarni P, Bhagwat R, Sharma L: Challenges in management of advanced neuroblastoma: Experience at Tata Memorial Hospital, Mumbai, India. J Clin Oncol; 2004 Jul 15;22(14_suppl):8562

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  • [Title] Challenges in management of advanced neuroblastoma: Experience at Tata Memorial Hospital, Mumbai, India.
  • : 8562 Background: Poor therapeutic response in advanced neuroblastoma prompted us to implement St. Jude's Protocol [NB - 84, TMH NB-2] since 1987 and Ifosfamide based chemotherapy [ICE, TMH NB-3] since 1996 with the aim to improve survival rate with manageable toxicities.
  • METHODS: 128 cases of neuroblastoma from 1987 to 2000 were analysed.
  • Stage III(17), IV(1) received 6 # of adriamycin & cyclophosphamide or 1 -2 # of adriamycin, cyclophosphamide, Cisplatin & Etoposide.
  • Surgery considered after 3/6 # or 1/2# of chemotherapy respectively.
  • Stage IIB(3),III(30), IV(60) received 1cycle of TMH NB-2 as induction followed by either 2# of TMH NB-2 or 4 # TMH NB-3 as consolidation.
  • Radiotherapy or 131I MIBG treatment was given where surgery not feasible or in R1 resection.
  • ABMT was offered for gross residual disease when economically feasible & 13 Cis Retinoic acid for minimal residual disease in stage IV.
  • RESULTS: Infantile group 62% constituted intermediate risk (stage III, IV).
  • Overall response (CR+VGPR+PR) to therapy was 97%. OS is 75.06%.
  • In children>1year 94% constituted high risk (IIB, III, IV).74% showed response to therapy.
  • Proper risk stratification has not been possible at our center & remains the main constraint in tailoring therapy.
  • Innovative & aggressive treatment strategies to improve survival & response rates now remains a need of time.

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  • (PMID = 28013871.001).
  • [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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13. Arafat W, Abdelghany A, Awad N: A clinical trial to study the effect of adding cis-retinoic acid during and after conventional treatment for pediatric neuroblastoma patient. J Clin Oncol; 2009 May 20;27(15_suppl):10057

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  • [Title] A clinical trial to study the effect of adding cis-retinoic acid during and after conventional treatment for pediatric neuroblastoma patient.
  • : 10057 Background: Neuroblastoma is predominantly a tumor of early childhood, with two thirds of the cases presenting in children younger than 5 years.
  • Cis-retinoic acid has been used as maintenance therapy for treatment of advanced neuroblastoma after BMT.
  • Patients who received cis-retinoic acid had significantly better 3-year event-free survival than patients receiving no maintenance therapy.
  • However, there is no data available about usingcis-retinoic acid during induction phase of chemotherapy.
  • The aim of this study is to evaluate the efficacy of cis-retinoic acid when used in combination with conventional chemotherapy in pediatric patient who is newly diagnosed with locally advanced neuroblastoma.
  • METHODS: Seventeen newly diagnosed children with locally advanced neuroblastoma who is candidate to receive chemotherapy were also received cis-retinoic acid starting at a dose of 160 /m<sup>2</sup> day (day 2-15 of chemotherapy) first the 3 patients, 20% dose reduction were allowed if toxicity occurred in first cohort of patient. . Patients were giving the drugs for at least 4 cycles.
  • RT-PCR analysis of several related genes was done from tumor, sample after treatment.
  • Patients were stage III, IV (70%), II (30%).
  • CONCLUSIONS: Cis-retinoic acid at dose 130mg/m<sup>2</sup> is a well tolerated drug with chemotherapy.
  • Response to treatment is better than historical control.
  • Randomized phase III trial is warranted.

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  • (PMID = 27962453.001).
  • [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. Bansal D, Marwaha RK, Trehan A, Rao KL, Gupta V: Profile and outcome of neuroblastoma with convertional chemotherapy in children older than one year: a 15-years experience. Indian Pediatr; 2008 Feb;45(2):135-9
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  • [Title] Profile and outcome of neuroblastoma with convertional chemotherapy in children older than one year: a 15-years experience.
  • The clinical profile and outcome of neuroblastoma in 103 children, older than one-year is presented.
  • 74 had Stage IV, 27 Stage III and one patient each had Stage I or II disease.
  • Treatment included chemotherapy followed by surgical resection/debulking.
  • Chemotherapy consisted of OPEC (vincristine, cyclophosphamide, cisplatin and etoposide).
  • The caretakers of 54 (52.4%) children either did not opt for or defaulted therapy, whilst 3 patients died before chemotherapy could be initiated.
  • Of the remaining 46 patients, the tumor progressed during therapy in 19 (41.3%).
  • Majority of children presented with advanced disease and the outcome was dismal with conventional non-myloablative chemotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Brain Neoplasms / pathology. Neuroblastoma / drug therapy. Neuroblastoma / pathology


15. Paulino AC: Palliative radiotherapy in children with neuroblastoma. Pediatr Hematol Oncol; 2003 Mar;20(2):111-7
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  • [Title] Palliative radiotherapy in children with neuroblastoma.
  • Limited information is available regarding the efficacy of external beam radiation therapy in the palliation of metastatic disease from neuroblastoma.
  • There were 26 soft tissue (group I), 19 bone (group II), 5 brain (group III), and 3 hepatic (group IV) treated sites.
  • Median radiotherapy doses for groups I, II, III, and IV sites were 2000, 2000, 2400, and 450 cGy, respectively.
  • For group II sites, complete response was complete pain relief without medication, partial response was > or =50% pain relief with or without medication, no response was <50% change in pain with medication, and progressive disease was increase in pain and/or medication.
  • Duration of response was until death in 18 responders (90%); 2 patients relapsed with an increasing soft tissue mass at 5 months and 1 year after palliative radiotherapy.
  • For the 5 group III children, the median survival was 2.5 months with a range of 2 days to 13 months.
  • The only patient who survived had a stage IV-S neuroblastoma with liver metastases and is alive 13 years after hepatic irradiation.
  • Radiotherapy is an effective treatment for palliation of symptomatic metastatic disease in children with neuroblastoma.
  • [MeSH-major] Neuroblastoma / radiotherapy. Palliative Care
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / radiotherapy. Bone Neoplasms / secondary. Brain Neoplasms / radiotherapy. Brain Neoplasms / secondary. Child. Child, Preschool. Combined Modality Therapy. Cranial Irradiation. Cyclophosphamide / administration & dosage. Disease Progression. Doxorubicin / administration & dosage. Female. Humans. Infant. Liver Neoplasms / drug therapy. Liver Neoplasms / radiotherapy. Liver Neoplasms / secondary. Male. Pain / radiotherapy. Radioisotope Teletherapy. Remission Induction. Retrospective Studies. Soft Tissue Neoplasms / radiotherapy. Soft Tissue Neoplasms / secondary. Survival Analysis. Treatment Outcome

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  • (PMID = 12554522.001).
  • [ISSN] 0888-0018
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide
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16. Suita S, Tajiri T, Sera Y, Takamatsu H, Mizote H, Nagasaki A, Kurosaki N, Hara T, Okamura J, Miyazaki S, Sugimoto T, Kawakami K, Eguchi H, Tsuneyoshi M: Improved survival for patients with advanced neuroblastoma after high-dose combined chemotherapy based in part on N-myc amplification. J Pediatr Surg; 2000 Dec;35(12):1737-41
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Improved survival for patients with advanced neuroblastoma after high-dose combined chemotherapy based in part on N-myc amplification.
  • BACKGROUND/PURPOSE: In spite of many different kinds of chemotherapy for neuroblastoma, the prognosis for advanced neuroblastoma remains unsatisfactory.
  • In particular, the outcome of advanced neuroblastoma with high copies of the N-myc gene tend to be poor.
  • Therefore, the new high-dosage combined chemotherapy regimens for advanced neuroblastoma based in part on the N-myc amplification status has been utilized in the Kyushu area of Japan since 1991.
  • This study aims to investigate whether these new regimens based in part on N-myc amplification have improved the survival rate of stage III and stage IV patients in comparison with the old regimens.
  • METHODS: Between 1983 and 1995, 77 patients over 1 year of age and with stage III or IV neuroblastoma were registered in the Kyushu Area.
  • Between 1983 and 1990, 49 patients received 1 of 2 combined chemotherapy regimens consisting of cyclophosphamide, cisplatin plus VM-26, and Adriamycin plus DTIC.
  • However, in the 19 stage IV patients treated by the new regimens, the 3-year survival rate (11.1%) of the 9 cases of more than 10 copies was significantly lower than that (77.8%) of the 10 cases of less than 10 copies of N-myc (P <.01).
  • CONCLUSIONS: These results suggest that high-dose combined chemotherapy based in part on the N-myc amplification status significantly improved the prognosis of patients with advanced neuroblastoma.
  • However, stage IV patients with N-myc amplification still require a more effective treatment modality.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Gene Amplification. Genes, myc / genetics. Neuroblastoma / genetics. Neuroblastoma / mortality

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  • (PMID = 11101726.001).
  • [ISSN] 0022-3468
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] UNITED STATES
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17. Gao XN, Tang SQ, Lin J: [Clinical features and prognosis of advanced neuroblastoma in children]. Zhongguo Dang Dai Er Ke Za Zhi; 2007 Aug;9(4):351-4
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  • [Title] [Clinical features and prognosis of advanced neuroblastoma in children].
  • OBJECTIVE: To investigate the clinical features, treatment modalities and the prognosis of advanced neuroblastoma in children.
  • METHODS: The medical records of 63 children with stage III or IV neuroblastoma from January 1996 to December 2005 were retrospectively reviewed.
  • Sixty patients were treated by tumor resection and (or) chemotherapy and (or) radiation.
  • RESULTS: Of the 63 patients with advanced neuroblastoma, the male/female ratio was 2.7:1 and the median age at diagnosis was 4 years old.
  • The median survival time of the 63 patients was 32.7 months.
  • Intensive chemotherapy in combination with autologous peripheral blood stem cell transplantation could also result in a prolonged overall survival period (P < 0.01).
  • CONCLUSIONS: Neuroblastoma with advanced stages often presents with various clinical manifestations and has a poor prognosis.
  • It is beneficial to improve the prognosis of neuroblastoma through an early diagnosis and a comprehensive therapy including total resection of the primary tumor, autologous peripheral blood stem cell transplantation and intensive chemotherapy.
  • [MeSH-major] Neuroblastoma / mortality
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Prognosis

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  • (PMID = 17706038.001).
  • [ISSN] 1008-8830
  • [Journal-full-title] Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics
  • [ISO-abbreviation] Zhongguo Dang Dai Er Ke Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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18. Schalk E, Mohren M, Koenigsmann M, Buhtz P, Franke A, Jentsch-Ullrich K: Metastatic adrenal neuroblastoma in an adult. Onkologie; 2005 Jun;28(6-7):353-5
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  • [Title] Metastatic adrenal neuroblastoma in an adult.
  • BACKGROUND: Neuroblastoma (NB) is a common malignancy in children, but rarely occurs in adults.
  • Accepted unfavourable prognostic factors include age > 1 year, low histologic grade and advanced stage, MYCN amplification, chromosomal aberrations, elevations of neuron specific enolase and lactate dehydrogenase, and increased catecholamine metabolites in urine or serum.
  • Tumour resection revealed adrenal NB grade III.
  • Chemotherapy according to the paediatric German Neuroblastoma Trial (NB97) was started.
  • Follow-up computed tomography showed regression of the enlarged mediastinal and retroperitoneal lymph nodes.
  • Currently there are no standard treatment guidelines for patients with NB in adulthood.
  • Collection and evaluation of data in adult patients with this tumour are warranted in order to optimise treatment strategies.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Neuroblastoma / pathology. Neuroblastoma / secondary

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  • (PMID = 15933424.001).
  • [ISSN] 0378-584X
  • [Journal-full-title] Onkologie
  • [ISO-abbreviation] Onkologie
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
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19. Meir HM, Balawi I, Nayel H, Yousef MK, Badawood S, Al-Mobarak M: Neuroblastoma: A clinical review. Neurosciences (Riyadh); 2001 Oct;6(4):213-9

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Neuroblastoma: A clinical review.
  • OBJECTIVE: Due to the poor results achieved on combination chemotherapy and the unproven cost-effectiveness value of myeloablative therapy, the question has been raised; should patients with stage IV neuroblastoma be actively treated?
  • The aim of the current study is to analyze retrospectively treatment results of 43 children with neuroblastoma with special stress on the rate and duration of remission in children with disseminated neuroblastoma.
  • METHODS: Treatment of children with neuroblastoma consisted of surgical removal of the tumor, if possible, followed by chemotherapy for unresectable residual tumor including metastases.
  • Second look surgery was performed to resect residual masses rendered resectable on chemotherapy in the absence of distal metastases.
  • The chemotherapy protocol used in the current study consisted of alternating combination chemotherapy regimens containing, Cyclophosphamide, Vincristine and Doxorubicin, alternating with Cis-platinum and Etoposide.
  • Two patients (5%) were found to have stage IV.
  • Stage III was documented in 5 patients (12%).
  • The bone marrow was the most common site of metastatic deposit, encountered in 23 patients out of the 30 with stage IV disease (77%).
  • Out of the 12 evaluable non-stage IV patients, only one patient (8%) showed treatment failure.
  • Assessment of response by the end of the 6th month from the date of diagnosis revealed that out of the 27 evaluable patients with stage IV, 4 patients achieved complete remission, 7 patients achieved very good partial remission, 8 patients achieved partial remission and 4 patients achieved mixed response.
  • Three patients showed progressive disease on chemotherapy.
  • Assessment of response to treatment by the end of the 12th month from diagnosis revealed that 6 patients (2 complete remissions, 1 very good partial response, 3 partial responses) were maintaining their remission.
  • CONCLUSION: Results of treatment by multiagent chemotherapy regimens used in the current study show that children with neuroblastoma, even those with advanced stages, should receive the benefit of intensive multimodal therapy, even those with partial response to initial therapy.

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  • (PMID = 24185182.001).
  • [ISSN] 1319-6138
  • [Journal-full-title] Neurosciences (Riyadh, Saudi Arabia)
  • [ISO-abbreviation] Neurosciences (Riyadh)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Saudi Arabia
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20. Martínez Ibáñez V, Abad P, Gallego S, Sánchez de Toledo J, Marqués A, Boix Ochoa J: [Neuroblastoma: biological markers, surgery, and clinical course]. Cir Pediatr; 2000 Apr;13(2):47-53
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  • [Title] [Neuroblastoma: biological markers, surgery, and clinical course].
  • INTRODUCTION: The treatment of neuroblastoma is basically chemotherapy, and surgery, in spite of advances, this kind of tumor is nowadays a surgical challenge.
  • PURPOSE: The aim of this study was to evaluate the impact of our therapy in this kind of pediatric tumors.
  • MATERIAL AND METHODS: 32 consecutive patients with abdominal neuroblastoma, aged between 1 month and 10 years old, median age 3 years old, observed from 1993 through 1997 have been studied.
  • Several parameters: age, ferritin, deletion of the chromosome 1p36, chromosomic ploidy, LDH, N-myc gene amplification and enolase neuron specific were studied and were related with the histology by Joshi and the International Neuroblastoma Staging System (INSS) in order to know the prognosis.
  • All the patients were treated by means of chemotherapy and surgery, and some cases with radiotherapy and bone marrow transplantation.
  • RESULTS: Two patients presented the tumor in stage I (INSS) and three in stage II.
  • In the state III, two patients did not maintain the follow-up; five live with disease (two with QT without surgery yet, two local recurrences, and one metastasis), and four live free of disease.
  • In the stage IV: five died, two live with disease (1 local recurrence and one metastasis), five live free of disease, and one did not maintain the follow-up.
  • In the stage IV-S, the three patients live free of disease.
  • Of all the parameters studied, we consider the ones with biggest prognostic efficacy are, the age, the stage INSS, the histology Shimada and the N-myc amplification.
  • [MeSH-major] Abdominal Neoplasms / surgery. Neuroblastoma / surgery

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  • (PMID = 12602001.001).
  • [ISSN] 0214-1221
  • [Journal-full-title] Cirugía pediátrica : organo oficial de la Sociedad Española de Cirugía Pediátrica
  • [ISO-abbreviation] Cir Pediatr
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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21. Suh JM, Yoo KH, Sung KW, Kim JY, Cho EJ, Koo HH, Lee SK, Kim J, Lim DH, Suh YL, Kim DW: High-dose chemotherapy and autologous stem cell rescue in patients with high-risk stage 3 neuroblastoma: 10-year experience at a single center. J Korean Med Sci; 2009 Aug;24(4):660-7
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  • [Title] High-dose chemotherapy and autologous stem cell rescue in patients with high-risk stage 3 neuroblastoma: 10-year experience at a single center.
  • High-dose chemotherapy and autologous stem cell rescue (HDCT/ASCR) was applied to improve the prognosis of patients with high-risk stage 3 neuroblastoma.
  • From January 1997 to December 2006, 28 patients were newly diagnosed as stage 3 neuroblastoma.
  • Patients without high-risk features received conventional treatment modalities only.
  • Toxicities associated with HDCT/ASCR were tolerable and there was no treatment-related mortality.
  • The present study demonstrates that HDCT/ASCR may improve the survival of patients with high-risk stage 3 neuroblastoma.
  • [MeSH-major] Neuroblastoma / therapy. Peripheral Blood Stem Cell Transplantation
  • [MeSH-minor] Adolescent. Adult. Child. Combined Modality Therapy. Female. Granulocyte Colony-Stimulating Factor / therapeutic use. Humans. Male. Middle Aged. Neoplasm Staging. Proto-Oncogene Proteins c-myc / analysis. Proto-Oncogene Proteins c-myc / genetics. Recombinant Proteins. Survival Rate. Tomography, X-Ray Computed. Transplantation, Autologous

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  • (PMID = 19654949.001).
  • [ISSN] 1598-6357
  • [Journal-full-title] Journal of Korean medical science
  • [ISO-abbreviation] J. Korean Med. Sci.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Korea (South)
  • [Chemical-registry-number] 0 / Proto-Oncogene Proteins c-myc; 0 / Recombinant Proteins; 143011-72-7 / Granulocyte Colony-Stimulating Factor
  • [Other-IDs] NLM/ PMC2719186
  • [Keywords] NOTNLM ; Autologous Stem Cell Rescue / High-dose Chemotherapy / N-myc / Neuroblastoma / Prognosis
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22. Becker J, Pavlakovic H, Ludewig F, Wilting F, Weich HA, Albuquerque R, Ambati J, Wilting J: Neuroblastoma progression correlates with downregulation of the lymphangiogenesis inhibitor sVEGFR-2. Clin Cancer Res; 2010 Mar 1;16(5):1431-41
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  • [Title] Neuroblastoma progression correlates with downregulation of the lymphangiogenesis inhibitor sVEGFR-2.
  • Previous studies have indicated the upregulation of VEGF-A and -C in progressed neuroblastoma, however, quantification was performed using semiquantitative methods, or patients who had received radiotherapy or chemotherapy were studied.
  • EXPERIMENTAL DESIGN: We have analyzed primary neuroblastoma from 49 patients using real-time reverse transcription-PCR and quantified VEGF-A, -C, and -D and VEGF receptors (VEGFR)-1, 2, 3, as well as the soluble form of VEGFR2 (sVEGFR-2), which has recently been characterized as an endogenous inhibitor of lymphangiogenesis.
  • None of the patients had received radiotherapy or chemotherapy before tumor resection.
  • RESULTS: We did not observe upregulation of VEGF-A, -C, and -D in metastatic neuroblastoma, but found significant downregulation of the lymphangiogenesis inhibitor sVEGFR-2 in metastatic stages III, IV, and Ivs. In stage IV neuroblastoma, there were tendencies for the upregulation of VEGF-A and -D and the downregulation of the hemangiogenesis/lymphangiogenesis inhibitors VEGFR-1 and sVEGFR-2 in MYCN-amplified tumors.
  • Similarly, MYCN transfection of the neuroblastoma cell line SH-EP induced the upregulation of VEGF-A and -D and the switching-off of sVEGFR-2.
  • CONCLUSION: We provide evidence for the downregulation of the lymphangiogenesis inhibitor sVEGFR-2 in metastatic neuroblastoma stages, which may promote lymphogenic metastases.
  • Downregulation of hemangiogenesis and lymphangiogenesis inhibitors VEGFR-1 and sVEGFR-2, and upregulation of angiogenic activators VEGF-A and VEGF-D in MYCN-amplified stage IV neuroblastoma supports the crucial effect of this oncogene on neuroblastoma progression.

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  • (PMID = 20179233.001).
  • [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
  • [Grant] United States / NEI NIH HHS / EY / EY020672-01; United States / NEI NIH HHS / EY / EY018350-05; United States / NEI NIH HHS / EY / R01 EY018350-05; United States / NEI NIH HHS / EY / R01 EY018350; United States / NEI NIH HHS / EY / R01 EY018350-04; United States / NEI NIH HHS / EY / R01 EY020672-01; United States / NEI NIH HHS / EY / R01 EY020672; United States / NEI NIH HHS / EY / R01 EY020672-02; United States / NEI NIH HHS / EY / EY020672-02; United States / NEI NIH HHS / EY / EY018350-04
  • [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 / MYCN protein, human; 0 / Nuclear Proteins; 0 / Oncogene Proteins; 0 / Vascular Endothelial Growth Factor A; 0 / Vascular Endothelial Growth Factor C; 0 / Vascular Endothelial Growth Factor D; EC 2.7.10.1 / Vascular Endothelial Growth Factor Receptor-1; EC 2.7.10.1 / Vascular Endothelial Growth Factor Receptor-2; EC 2.7.10.1 / Vascular Endothelial Growth Factor Receptor-3
  • [Other-IDs] NLM/ NIHMS280227; NLM/ PMC3065717
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23. Moon SB, Park KW, Jung SE, Youn WJ: Neuroblastoma: treatment outcome after incomplete resection of primary tumors. Pediatr Surg Int; 2009 Sep;25(9):789-93
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  • [Title] Neuroblastoma: treatment outcome after incomplete resection of primary tumors.
  • PURPOSE: For International Neuroblastoma Staging System (INSS) stages III or IV neuroblastoma (intermediate or high risk), complete excision of the primary tumor is not always feasible.
  • Most current studies on the treatment outcome of these patients have reported on the complete excision status.
  • The aim of this study is to review the treatment outcome after the incomplete resection.
  • Incomplete resection was assessed by review of the operative notes and postoperative computerized tomography.
  • Age, gender, tumor location, INSS stage, N-myc gene copy number, pre- and postoperative therapy, and treatment outcome were reviewed.
  • The treatment outcome was evaluated according to the postoperative treatment protocol in the high-risk group.
  • RESULTS: Intermediate-risk patients were treated with conventional chemotherapy, isotretinoin (ITT) and interleukin-2 (IL-2).
  • Before the introduction of PBSCT, the high-risk patients were also treated with the conventional chemotherapy (N = 19).
  • For the high-risk patients (N = 32), 19 patients were treated with chemotherapy alone; 15 patients died of their disease while four patients currently have an NED status.
  • CONCLUSIONS: For intermediate risk, conventional chemotherapy appears to be acceptable treatment.
  • However, for high-risk patients, every effort should be made to control residual disease including the use of myeloablative chemotherapy, differentiating agents and immune-modulating agents.
  • [MeSH-major] Neuroblastoma / mortality. Neuroblastoma / therapy
  • [MeSH-minor] Adrenal Gland Neoplasms / mortality. Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / therapy. Antineoplastic Agents / therapeutic use. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Dermatologic Agents / therapeutic use. Female. Follow-Up Studies. Genes, myc. Humans. Infant. Interleukin-2 / therapeutic use. Isotretinoin / therapeutic use. Male. Mediastinal Neoplasms / mortality. Mediastinal Neoplasms / pathology. Mediastinal Neoplasms / therapy. Neoplasm, Residual. Pelvic Neoplasms / mortality. Pelvic Neoplasms / pathology. Pelvic Neoplasms / therapy. Peripheral Blood Stem Cell Transplantation. Radiotherapy, Adjuvant. Retroperitoneal Neoplasms / mortality. Retroperitoneal Neoplasms / pathology. Retroperitoneal Neoplasms / therapy. Retrospective Studies

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  • (PMID = 19629500.001).
  • [ISSN] 1437-9813
  • [Journal-full-title] Pediatric surgery international
  • [ISO-abbreviation] Pediatr. Surg. Int.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Dermatologic Agents; 0 / Interleukin-2; EH28UP18IF / Isotretinoin
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24. Seeger RC, Reynolds CP, Gallego R, Stram DO, Gerbing RB, Matthay KK: Quantitative tumor cell content of bone marrow and blood as a predictor of outcome in stage IV neuroblastoma: a Children's Cancer Group Study. J Clin Oncol; 2000 Dec 15;18(24):4067-76
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  • [Title] Quantitative tumor cell content of bone marrow and blood as a predictor of outcome in stage IV neuroblastoma: a Children's Cancer Group Study.
  • PURPOSE: This study investigated the prognostic value of quantifying tumor cells in bone marrow and blood by immunocytology in children with high-risk, metastatic neuroblastoma.
  • PATIENTS AND METHODS: Patients with stage IV neuroblastoma (N = 466) registered on Children's Cancer Group study 3891 received five cycles of induction chemotherapy and were randomized either to myeloablative chemoradiotherapy with autologous purged bone marrow rescue or to nonmyeloablative chemotherapy.
  • Subsequently, they were randomized to 13-cis-retinoic acid or no further treatment.
  • Immunocytologic analyses of bone marrow and blood were performed at diagnosis, week 4, week 12, bone marrow collection, and end induction and were correlated with tumor biology, clinical variables, treatment regimen, and event-free survival (EFS).
  • RESULTS: Immunocytology identified neuroblastoma cells in bone marrow of 81% at diagnosis, 55% at 4 weeks, 27% at 12 weeks, 19% at bone marrow collection, and 14% at end induction.
  • CONCLUSION: Immunocytologic quantification of neuroblastoma cells in bone marrow and blood at diagnosis and in bone marrow during induction chemotherapy provides prognostic information that can identify patients with very high-risk disease who should be considered for experimental therapy that might improve outcome.
  • [MeSH-major] Bone Marrow Cells / pathology. Neoplastic Cells, Circulating / pathology. Neuroblastoma / pathology
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Marrow Transplantation. Child. Child, Preschool. Disease-Free Survival. Humans. Infant. Isotretinoin / therapeutic use. Neoplasm Staging. Remission Induction. Risk Factors. Treatment Outcome

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  • (PMID = 11118468.001).
  • [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 / CA02649; United States / NCI NIH HHS / CA / CA13539; United States / NCI NIH HHS / CA / CA22794; etc
  • [Publication-type] Clinical Trial; Clinical Trial, Phase III; Journal Article; Multicenter Study; Randomized Controlled Trial; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] EH28UP18IF / Isotretinoin
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25. Nzegwu MA, Aghaji A: Neuroblastoma occurring in a 38-year old Nigerian man: a rare finding. Rare Tumors; 2009;1(1):e15

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  • [Title] Neuroblastoma occurring in a 38-year old Nigerian man: a rare finding.
  • Neuroblastoma (NB) is a common malignancy in children, but rarely occurs in adults.
  • Accepted unfavorable prognostic factors include age over one year, low histological grade and advanced stage, MYCN amplification, chromosomal aberrations, elevations of neuron specific enolase and lactate dehydrogenase, and increased catecholamine metabolites in urine or serum.
  • Tumor resection revealed a grade III NB.
  • Chemotherapy using a combination of vincristine, adriamycin and cyclophosphamide was started.
  • He resurfaced with new masses and he had a repeat chemotherapy with disappearance of the masses and is currently undergoing further treatment.
  • Currently, there are no standard treatment guidelines for patients with NB in adulthood.
  • This study emphasizes the need for a standard treatment regime for adult onset neuroblastoma and its recognition as a possible differential in intra-abdominal mass in adults.

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  • (PMID = 21139886.001).
  • [ISSN] 2036-3613
  • [Journal-full-title] Rare tumors
  • [ISO-abbreviation] Rare Tumors
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
  • [Other-IDs] NLM/ PMC2994447
  • [Keywords] NOTNLM ; adult. / neuroblastoma
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26. Sun XF, Liu DG, Su YS, Lin TY, Chen XQ, He YJ: [Evaluation of efficacy of treatment for 30 children with neuroblastoma]. Ai Zheng; 2003 Dec;22(12):1343-5
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  • [Title] [Evaluation of efficacy of treatment for 30 children with neuroblastoma].
  • BACKGROUND & OBJECTIVE: Neuroblastoma is one of common solid tumors in children.
  • The major treatment modality for neuroblastoma (NB) is chemotherapy combined with operation or irradiation.
  • Further study is needed for improving cure rate of neuroblastoma.
  • This study was designed to evaluate the efficacy of children with neuroblastoma treated in Cancer Center, Sun Yat-sen University, and to explore reasonable therapy strategy.
  • These patients were treated with chemotherapy plus operation or radiation.
  • The stages were as follow: II, n=2; III, n=12; IV, n=15; IVS, n=1.
  • Chemotherapy regimen was CAV (cyclophosphamide 750 mg/m(2) d1, vincristine 1.5 mg/m(2), d1, Adriamycin 50 mg/m(2) d1) alternated with EP (teniposide or etoposide 60 mg/m(2) d1-d5, cisplatin 20 mg/m(2) d1-d5).
  • The resection would be done after 4 to 6 cycles of chemotherapy if possible.
  • The chemotherapy or radiation would be done after resection.
  • If operation was not available, the patients continued to receive the chemotherapy.
  • The patients with stage IVS only received cyclophosphamide plus vincristine.
  • RESULTS: Among 30 patients, 2 cases achieved complete remission (CR 6.7%) by chemotherapy alone; 21 cases achieved partial remission (PR 70%); 6 cases showed no change (NC 20%); 1 cases showed progressive diseases (3.3%).
  • Overall response rate (CR+PR) were 76.7% by chemotherapy alone.
  • The 2-year overall survival rate was 47.8% for all patients; 100% for Stage II/IVS, 34% for stage III, 22% for stage IV, respectively.
  • Grade III/IV hematological toxicity occurred in 41.2% of the CAV regimen and 26.6% of the EP regimen.
  • CONCLUSION: Chemotherapy plus operation or radiation is the major treatment for neuroblastoma.
  • CAV/EP alternative chemotherapy is the active regimen for NB.
  • Advance stage NB needs further study for improving the prognosis.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cyclophosphamide / therapeutic use. Doxorubicin / therapeutic use. Neuroblastoma / therapy. Vincristine / therapeutic use
  • [MeSH-minor] Child. Child, Preschool. Combined Modality Therapy. Etoposide / administration & dosage. Female. Humans. Infant. Male. Neoplasm Staging. Retrospective Studies. Teniposide / administration & dosage. Treatment Outcome

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  • (PMID = 14693065.001).
  • [Journal-full-title] Ai zheng = Aizheng = Chinese journal of cancer
  • [ISO-abbreviation] Ai Zheng
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; 957E6438QA / Teniposide; CAV protocol
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27. von Allmen D, Grupp S, Diller L, Marcus K, Ecklund K, Meyer J, Shamberger RC: Aggressive surgical therapy and radiotherapy for patients with high-risk neuroblastoma treated with rapid sequence tandem transplant. J Pediatr Surg; 2005 Jun;40(6):936-41; discussion 941
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  • [Title] Aggressive surgical therapy and radiotherapy for patients with high-risk neuroblastoma treated with rapid sequence tandem transplant.
  • BACKGROUND/PURPOSE: The treatment approach for patients with high-risk neuroblastoma has been one of dose intensification chemotherapy and aggressive treatment of the primary tumor.
  • Local tumor control is examined in high-risk patients treated with tandem stem cell transplant, aggressive surgery, and selected radiation therapy (XRT).
  • METHODS: Seventy-six patients with high-risk stage III/IV neuroblastoma were treated on a standard protocol incorporating aggressive surgical resection with or without local XRT followed by tandem high-dose chemotherapy and stem cell rescue.
  • Surgeon assessment of completeness of resection agreed with postoperative radiological findings 66% of the time.
  • CONCLUSION: Aggressive surgical treatment with local XRT and myeloablative chemotherapy with stem cell rescue provides excellent local control in high-risk neuroblastoma, although distant failures, particularly osseous, remain a problem.
  • [MeSH-major] Abdominal Neoplasms / surgery. Adrenal Gland Neoplasms / surgery. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / surgery. Head and Neck Neoplasms / surgery. Neuroblastoma / surgery
  • [MeSH-minor] Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Humans. Male. Neoplasm Staging. Peripheral Blood Stem Cell Transplantation. Radiotherapy, Adjuvant. Remission Induction / methods. Transplantation, Autologous. Treatment Outcome. Whole-Body Irradiation


28. Tang JY, Pan C, Chen J, Xu M, Chen J, Xue HL, Gu LJ, Dong R, Ye H, Zhou M, Wang YP: [Comprehensive protocol for diagnosis and treatment of childhood neuroblastoma--results of 45 cases]. Zhonghua Er Ke Za Zhi; 2006 Oct;44(10):770-3
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  • [Title] [Comprehensive protocol for diagnosis and treatment of childhood neuroblastoma--results of 45 cases].
  • OBJECTIVE: The aim of the paper was to improve the prognosis of neuroblastoma (NB) stage III and IV in children through the comprehensive therapy including chemotherapy, delayed tumor resection, autologous stem cell transplantation (ASCT) and inducing differentiation and to analyze the factors affecting the prognosis.
  • METHODS: Newly diagnosed neuroblastoma patients seen from Oct.1998 to Dec.2003 were divided into high, medium and low risk groups depending on clinical stage and age.
  • Comprehensive protocol included accurate staging, delayed and/or second tumor resection for stage III and IV patients, chemotherapy of different intensity mainly composed of cell cycle nonspecific drugs and 13-cis-retinoid for inducing cell differentiation.
  • ASCT was given at the end of therapy for high risk group.
  • Nine cases had stage I, 1 case had stage II, 8 cases had III, 26 cases had stage IV and 1 case had stage IVs of the tumor.
  • Depending on the age and stage of the tumor, 26 cases were aligned into high risk protocol, 10 into medium risk and 9 into low risk groups.
  • Of the thirty-nine patients, 31 achieved complete remission (CR) and 8 partial remission (PR) after surgery and/or chemotherapy.
  • No death occurred from treatment complication.
  • Statistical analysis showed that the age older than 18 months, and stage III and IV of the tumor were the factors predicting poor prognosis (P = 0.04 and 0.003, respectively).
  • Age older than 18 months, and stage III and IV were the factors suggesting poor prognosis.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Hematopoietic Stem Cell Transplantation. Neuroblastoma / diagnosis. Neuroblastoma / therapy. Transplantation, Autologous
  • [MeSH-minor] Age Factors. Chemotherapy, Adjuvant. Child. Child, Preschool. Female. Follow-Up Studies. Humans. Infant. Male. Neoplasm Recurrence, Local / prevention & control. Neoplasm Recurrence, Local / surgery. Neoplasm Recurrence, Local / therapy. Neoplasm Staging. Prognosis. Remission Induction / methods. Retrospective Studies. Severity of Illness Index. Survival Rate. Time Factors. Treatment Outcome

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  • (PMID = 17229383.001).
  • [ISSN] 0578-1310
  • [Journal-full-title] Zhonghua er ke za zhi = Chinese journal of pediatrics
  • [ISO-abbreviation] Zhonghua Er Ke Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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29. Ryu KH, Ahn HS, Koo HH, Kook H, Kim MK, Kim HK, Ghim T, Moon HN, Seo JJ, Sung KW, Shin HY, Yoo ES, Lyu CJ, Lee YH, Lee H, Cho B, Cho HS, Choi HS, Hah JO, Hwang TJ: Autologous stem cell transplantation for the treatment of neuroblastoma in Korea. J Korean Med Sci; 2003 Apr;18(2):242-7
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  • [Title] Autologous stem cell transplantation for the treatment of neuroblastoma in Korea.
  • Autologous stem cell transplantation (ASCT) for the treatment of high-risk neuroblastoma (NBL) is an accepted method for restoring bone marrow depression after high dose chemotherapy.
  • We retrospectively analyzed eighty eight cases of NBL that underwent ASCT following marrow ablative therapy at 12 transplant centers of the Korean Society of Pediatric Hematology-Oncology between January 1996 and September 2000.
  • Seventy nine children were of stage IV NBL and 9 were of stage III with N-myc amplification.
  • Post-transplantation therapies included the second transplantation in 18 patients, interleukin2 therapy in 45, 13-cis retinoic acid in 40, 131-meta-iodobenzylguanidine in 4, conventional chemotherapy in 11, and local radiotherapy in 8.
  • [MeSH-major] Neuroblastoma / therapy. Stem Cell Transplantation
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Korea. Male. Myeloablative Agonists / therapeutic use. Retrospective Studies. Survival Rate. Transplantation Conditioning. Transplantation, Autologous. Treatment Outcome

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  • (PMID = 12692423.001).
  • [ISSN] 1011-8934
  • [Journal-full-title] Journal of Korean medical science
  • [ISO-abbreviation] J. Korean Med. Sci.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Korea (South)
  • [Chemical-registry-number] 0 / Myeloablative Agonists
  • [Other-IDs] NLM/ PMC3055035
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30. Imaizumi M, Watanabe A, Kikuta A, Takano T, Ito E, Shimizu T, Tsuchiya S, Iinuma K, Konno T, Ohi R, Hayashi Y, Tohoku Neuroblastoma Study Group: Improved survival of children with advanced neuroblastoma treated by intensified therapy including myeloablative chemotherapy with stem cell transplantation: a retrospective analysis from the Tohoku Neuroblastoma Study Group. Tohoku J Exp Med; 2001 Oct;195(2):73-83
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  • [Title] Improved survival of children with advanced neuroblastoma treated by intensified therapy including myeloablative chemotherapy with stem cell transplantation: a retrospective analysis from the Tohoku Neuroblastoma Study Group.
  • In the hospitals of the Tohoku Neuroblastoma Study Group (TNBSG), treatment for children with advanced neuroblastoma (NB) was intensified in the mid-1990's with the introduction of myeloablative therapy (MT) with stem cell transplantation (SCT) including the use of autologous peripheral blood stem cells (PBSC) and bone marrow transplantation (BMT).
  • In this report, we examined whether the intensified therapy improved the outcome of children with advanced NB (age> 12 months) who were diagnosed between 1991 and 1997.
  • Six of them had stage III disease, and the other 30 had stage IV.
  • They were treated initially with induction chemotherapy, surgery, and post-operative chemoradiotherapy, after which 17 of them continued further chemotherapy and the other 19 received MT/SCT (18 with PBSCT and 1 with BMT).
  • Progression-free survival (PFS) rate at seven years from diagnosis was 43.5% for all patients, 66.7% for stage III patients and 38.2% for stage IV patients.
  • The difference between stage III and IV patients was not significant.
  • Among the 30 patients with stage IV disease, PFS at seven years was significantly higher in the 19 patients who received MT/SCT (55.6%) than in the 11 patients who did not receive it (12.5%).
  • There was no difference in clinical and biological risk factors between these two groups, except for the proportion of patients with favorable response to initial therapy (36% and 80% for patients without and with MT/SCT, respectively).
  • The results of this retrospective study of children with advanced NB suggest that therapy intensification involving MT/SCT might result in lengthened survival time for patients with stage IV disease, and that post-transplant PD remains a risk for patients with high levels of N-myc amplification.
  • [MeSH-major] Hematopoietic Stem Cell Transplantation / methods. Melphalan / therapeutic use. Myeloablative Agonists / therapeutic use. Neuroblastoma / mortality. Transplantation Conditioning / methods
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Antineoplastic Combined Chemotherapy Protocols. Carboplatin / therapeutic use. Child. Child, Preschool. Combined Modality Therapy. Doxorubicin / analogs & derivatives. Doxorubicin / therapeutic use. Etoposide / therapeutic use. Humans. Infant. Japan. Retrospective Studies. Risk Factors. Survival Rate. Treatment Outcome. Universities

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  • (PMID = 11846211.001).
  • [ISSN] 0040-8727
  • [Journal-full-title] The Tohoku journal of experimental medicine
  • [ISO-abbreviation] Tohoku J. Exp. Med.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Myeloablative Agonists; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; BG3F62OND5 / Carboplatin; D58G680W0G / pirarubicin; Q41OR9510P / Melphalan
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31. Seefelder C, Sparks JW, Chirnomas D, Diller L, Shamberger RC: Perioperative management of a child with severe hypertension from a catecholamine secreting neuroblastoma. Paediatr Anaesth; 2005 Jul;15(7):606-10
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  • [Title] Perioperative management of a child with severe hypertension from a catecholamine secreting neuroblastoma.
  • We report the perioperative management of a 5 year old child with stage III adrenal neuroblastoma who presented with malignant hypertension and high norepinephrine and dopamine levels.
  • Hypertensive crises occurred during anesthesia for surgical biopsy and during chemotherapy.
  • [MeSH-major] Catecholamines / metabolism. Catecholamines / physiology. Hypertension / etiology. Hypertension / therapy. Kidney Neoplasms / complications. Kidney Neoplasms / metabolism. Neuroblastoma / complications. Neuroblastoma / metabolism
  • [MeSH-minor] Anesthesia. Antihypertensive Agents / therapeutic use. Child, Preschool. Dopamine / blood. Epinephrine / blood. Female. Humans. Magnetic Resonance Imaging. Norepinephrine / blood. Perioperative Care. Preanesthetic Medication


32. Raguénez G, Douc-Rasy S, Blanc E, Goldschneider D, Barrois M, Valteau-Couanet D, Bénard J: [A functional gene map is required to adapt therapy of metastatic neuroblastoma]. Bull Cancer; 2001 Mar;88(3):295-304
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  • [Title] [A functional gene map is required to adapt therapy of metastatic neuroblastoma].
  • [Transliterated title] Vers une carte génétique fonctionnelle des neuroblastomes métastiques pour une thérapeutique adaptée.
  • Neuroblastoma is a very common solid tumor which arises in childhood and shows an extreme heterogeneity at the clinical, histological and genetic levels.
  • Besides age and stage, N-myc amplification and 1p deletion are prognostic factors of the disease: in Europe, these genetic markers are used to conduct therapy.
  • In France, N-myc amplification is a factor of bad prognosis which leads, in all forms of the disease including localised forms and metastatic forms of children aged of less than 1 year, to a myeloablative treatment with autologous hematopoietic stem cells transplantation.
  • Moreover, experimental models of human metastatic neuroblastoma have been obtained in which variations of genes transcript levels involved in these pathways, are observed.
  • The current break-through of cDNA microarrays allows to develop a dynamic transcriptomic scanning of these models as well as of tumors and bone marrows from patients upon conventional chemotherapy.
  • ii) to apply adapted treatment;.
  • iii) to develop new therapeutic strategies.
  • [MeSH-major] Genes, myc / genetics. Neuroblastoma / genetics

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  • (PMID = 11313207.001).
  • [ISSN] 0007-4551
  • [Journal-full-title] Bulletin du cancer
  • [ISO-abbreviation] Bull Cancer
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Neoplasm Proteins; 0 / Nerve Growth Factors
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33. Inagaki J, Yasui M, Sakata N, Inoue M, Yagi K, Kawa K: Successful treatment of chemoresistant stage 3 neuroblastoma using irinotecan as a single agent. J Pediatr Hematol Oncol; 2005 Nov;27(11):604-6
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  • [Title] Successful treatment of chemoresistant stage 3 neuroblastoma using irinotecan as a single agent.
  • The authors describe a 1-year-old boy who was diagnosed with neuroblastoma by mass screening at age 6 months.
  • Although seven courses of chemotherapy consisting of vincristine sulfate, cyclophosphamide, pirarubicin hydrochloride, and cisplatin were administered, there was no reduction in tumor size or decrease in tumor markers.
  • This therapy was given a total of four courses every 4 weeks, with the tumor shrinking successively in each session.
  • Four years after treatment there is no sign of recurrence and the patient is doing well.
  • This case may be the first report showing the dramatic efficacy of irinotecan in the treatment of chemoresistant neuroblastoma without the use of other antitumor agents.
  • Irinotecan might be a promising drug in the management of patients with high-risk neuroblastoma.
  • [MeSH-major] Antineoplastic Agents, Phytogenic / therapeutic use. Camptothecin / analogs & derivatives. Drug Resistance, Neoplasm. Neuroblastoma / drug therapy. Retroperitoneal Neoplasms / drug therapy

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  • (PMID = 16282892.001).
  • [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 / Antineoplastic Agents, Phytogenic; 0 / Topoisomerase I Inhibitors; 7673326042 / irinotecan; XT3Z54Z28A / Camptothecin
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34. Di Bella G, Colori B: Complete objective response of neuroblastoma to biological treatment. Neuro Endocrinol Lett; 2009;30(4):437-49
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  • [Title] Complete objective response of neuroblastoma to biological treatment.
  • OBJECTIVES: The combined use (MDB) of Somatostatin, Melatonin, Retinoids, Vitamins E, C, and D3, with Calcium, Chondroitin sulfate, and microdoses of Cyclophosphamide in a seven-month old baby affected by a voluminous retroperitoneal neuroblastoma measuring 4 x 8 cm produced a 50% objective response in six months, an almost total response in one year and a complete response at 14 months, with cure and absence of disease for over ten years.
  • RESULTS: This paper discusses the rationale and the molecular mechanisms of action of the treatment which has a differentiating, apoptotic and antiproliferative effect, preserving and enhancing both the trophism and functionality of organs and tissues, and the neuroimmunoendocrine and antiblastic homeostasis.
  • This result is in agreement with the positive results already published on the use of the MDB in lymphoproliferative diseases, in stage III and IV lung cancer, in breast cancer and in cancers of the upper aerodigestive epithelia.
  • CONCLUSIONS: We believe it is of use to report this case in order to invite greater interest in the oncological possibilities offered by the immunoneuroendocrine and biological-receptorial properties of the MDB treatment.
  • [MeSH-major] Melatonin / therapeutic use. Neuroblastoma / drug therapy. Retinoids / therapeutic use. Retroperitoneal Neoplasms / drug therapy. Somatostatin / therapeutic use. Vitamins / therapeutic use
  • [MeSH-minor] Antioxidants / therapeutic use. Drug Therapy, Combination. Humans. Infant. Magnetic Resonance Imaging. Male. Positron-Emission Tomography. Remission Induction

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  • (PMID = 20010503.001).
  • [ISSN] 0172-780X
  • [Journal-full-title] Neuro endocrinology letters
  • [ISO-abbreviation] Neuro Endocrinol. Lett.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Sweden
  • [Chemical-registry-number] 0 / Antioxidants; 0 / Retinoids; 0 / Vitamins; 51110-01-1 / Somatostatin; JL5DK93RCL / Melatonin
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35. Pritchard J, Cotterill SJ, Germond SM, Imeson J, de Kraker J, Jones DR: High dose melphalan in the treatment of advanced neuroblastoma: results of a randomised trial (ENSG-1) by the European Neuroblastoma Study Group. Pediatr Blood Cancer; 2005 Apr;44(4):348-57
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  • [Title] High dose melphalan in the treatment of advanced neuroblastoma: results of a randomised trial (ENSG-1) by the European Neuroblastoma Study Group.
  • BACKGROUND: High dose myeloablative chemotherapy ("megatherapy"), with haematopoietic stem cell support, is now widely used to consolidate response to induction chemotherapy in patients with advanced neuroblastoma.
  • PROCEDURE: In this study (European Neuroblastoma Study Group, ENSG1), the value of melphalan myeloablative "megatherapy" was evaluated in a randomised, multi-centre trial.
  • Between 1982 and 1985, 167 children with stages IV and III neuroblastoma (123 stage IV > 1 year old at diagnosis and 44 stage III and stage IV from 6 to 12 months old at diagnosis) were treated with oncovin, cisplatin, epipodophyllotoxin, and cyclophosphamide (OPEC) induction chemotherapy every 3 weeks.
  • After surgical excision of primary tumour, the 90 patients (69% of the total) who achieved complete response (CR) or good partial response (GPR) were eligible for randomisation either to high dose melphalan (180 mg per square meter) with autologous bone marrow support or to no further treatment.
  • RESULTS: Sixty-five (72%) of eligible children were actually randomised and 21 of these patients were surviving at time of this analysis, with median follow-up from randomisation of 14.3 years.
  • This difference was not statistically significant (P = 0.08, log rank test) but for the 48 randomised stage IV patients aged >1 year at diagnosis outcome was significantly better in the melphalan-treated group-5 year EFS 33% versus 17% (P = 0.01, log rank test).
  • CONCLUSIONS: In this trial, high dose melphalan improved the length of EFS and overall survival of children with stage IV neuroblastoma >1 year of age who achieved CR or GPR after OPEC induction therapy and surgery.
  • Multi-agent myeloablative regimens are now widely used as consolidation therapy for children with stage IV disease and in those with other disease stages when the MYCN gene copy number in tumour cells is amplified.
  • [MeSH-major] Antineoplastic Agents, Alkylating / therapeutic use. Melphalan / therapeutic use. Neuroblastoma / drug therapy
  • [MeSH-minor] Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Europe / epidemiology. Female. Humans. Infant. Life Tables. Male. Survival Rate

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  • (PMID = 15546135.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Randomized Controlled Trial; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; Q41OR9510P / Melphalan
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36. Hase T, Ohta S, Tani T, Mizukuro T, Mekata E, Naitoh H, Shimadera S, Fujino S, Taga T: Outcome of infants with neuroblastoma detected by mass screening and surgically treated in Shiga Prefecture, Japan: what is the role of surgery? Pediatr Surg Int; 2002 Sep;18(5-6):289-94
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  • [Title] Outcome of infants with neuroblastoma detected by mass screening and surgically treated in Shiga Prefecture, Japan: what is the role of surgery?
  • To investigate retrospectively the clinical and biological features that influence the outcome of infants with neuroblastoma (NB) detected by mass screening (NBMS), and to construct surgical strategies to deal with NBMS, 20 infants diagnosed as having either NB or ganglioneuroblastoma (GNB) between 1986 and 1998 were enrolled in a study.
  • The following factors were analyzed by multivariate analysis: age, stage according to the Japanese staging system at the time of diagnosis, site of the primary tumor, histologic findings, preoperative urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels, VMA/HVA ratio, lactate dehydrogenase, neuron-specific enolase, Shimada's histologic classification, amplification of the N- myc oncogene by Southern blot analysis, nuclear content, and chromosomal abnormality.
  • Four infants had stage I, 6 stage II, 3 stage III, 3 stage IVB, and 4 stage IV disease.
  • The infant with N- myc-positive NB (stage II) died 23 months after surgery in spite of aggressive postoperative chemotherapy.
  • The N- myc-positive NB case implies that even in locoregional NB detected by NBMS, surgical excision should play a central role in the diagnosis of its oncogenic characteristics and indicate any subsequent therapy.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Neuroblastoma / surgery. Retroperitoneal Neoplasms / surgery
  • [MeSH-minor] Biomarkers, Tumor / urine. Female. Homovanillic Acid / urine. Humans. Infant. Japan. Male. Mass Screening. Neoplasm Staging. Prognosis. Retrospective Studies. Treatment Outcome. Vanilmandelic Acid / urine

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  • [CommentIn] Pediatr Surg Int. 2002 Sep;18(5-6):288 [12415340.001]
  • (PMID = 12415341.001).
  • [ISSN] 0179-0358
  • [Journal-full-title] Pediatric surgery international
  • [ISO-abbreviation] Pediatr. Surg. Int.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 55-10-7 / Vanilmandelic Acid; X77S6GMS36 / Homovanillic Acid
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37. Kim EK, Kang HJ, Park JA, Choi HS, Shin HY, Ahn HS: Retrospective analysis of peripheral blood stem cell transplantation for the treatment of high-risk neuroblastoma. J Korean Med Sci; 2007 Sep;22 Suppl:S66-72
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  • [Title] Retrospective analysis of peripheral blood stem cell transplantation for the treatment of high-risk neuroblastoma.
  • Disease relapse after autologous peripheral blood stem cell transplantation (APBSCT) is the main cause of treatment failure in high-risk neuroblastoma (NBL).
  • The medical records of 36 patients with stage III or IV NBL who underwent APBSCT at Seoul National University Children's Hospital between May 1996 and May 2004 were reviewed.
  • The patients were allocated to three groups according to the APBSCT type.
  • To improve long-term survival, various efforts should be made such as chemotherapy dose intensification, more effective tumor purging, and control of minimal residual disease via the use of differentiating and immune-modulating agents.
  • [MeSH-major] Neuroblastoma / therapy

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  • (PMID = 17923758.001).
  • [ISSN] 1011-8934
  • [Journal-full-title] Journal of Korean medical science
  • [ISO-abbreviation] J. Korean Med. Sci.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Korea (South)
  • [Chemical-registry-number] 0 / Antigens, CD34
  • [Other-IDs] NLM/ PMC2694391
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38. Garaventa A, Luksch R, Biasotti S, Severi G, Pizzitola MR, Viscardi E, Prete A, Mastrangelo S, Podda M, Haupt R, De Bernardi B: A phase II study of topotecan with vincristine and doxorubicin in children with recurrent/refractory neuroblastoma. Cancer; 2003 Dec 1;98(11):2488-94
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A phase II study of topotecan with vincristine and doxorubicin in children with recurrent/refractory neuroblastoma.
  • BACKGROUND: A Phase II trial in children with advanced neuroblastoma was carried out in five Italian institutions to evaluate the antitumor activity and tolerability of topotecan followed by vincristine and doxorubicin.
  • METHODS: Children older than age 1 year with Stage III or Stage IV neuroblastoma, all of whom had been treated previously with chemotherapy and were diagnosed with either refractory or recurrent disease, were treated with topotecan at an intravenous dose of 1.5 mg/m(2) (the dose was 0.75 mg/m(2) for patients who were treated within 1 year of previous megatherapy) per day for 5 days followed by 48-hour intravenous infusions of 2 mg/m(2) vincristine and 45 mg/m(2) doxorubicin.
  • Cycles of therapy were repeated every 3 weeks.
  • RESULTS: Twenty-five patients (2 with Stage III disease and 23 with Stage IV disease; 19 with refractory disease and 6 with recurrent disease) were treated with a total of 115 cycles.
  • Fifteen patients were alive at the time of the current report and were progression free at 4-16 months (median, 9 months) after the first course of this treatment.
  • CONCLUSIONS: The topotecan-vincristine-doxorubicin combination was active and well tolerated in previously treated patients with advanced neuroblastoma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Neuroblastoma / drug therapy
  • [MeSH-minor] Child. Child, Preschool. Disease Progression. Doxorubicin / administration & dosage. Female. Humans. Infant. Male. Survival Analysis. Topotecan / administration & dosage. Treatment Outcome. Vincristine / administration & dosage

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  • [Copyright] Copyright 2003 American Cancer Society.
  • (PMID = 14635085.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 7M7YKX2N15 / Topotecan; 80168379AG / Doxorubicin
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39. Rahi R, Vijyendra K, Sharma SP, Aryya NC, Shukla RC, Pradhan S, Singh TB, Gangopadhyay AN: A comparative study of intratumoral chemotherapy in advanced childhood common solid tumors. Indian J Urol; 2007 Oct;23(4):358-65

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A comparative study of intratumoral chemotherapy in advanced childhood common solid tumors.
  • BACKGROUND: Advanced and inoperable solid tumors in children are great killer despite aggressive multimodality treatment.
  • Intravenous chemotherapy, due to high dose of drug given systemically, at times leads to abandonment of therapy due to systemic toxicities.
  • To overcome this problem lots of studies are going on to explore alternative modes of giving anticancer drugs so as to decrease the systemic toxicities of the drugs and increase their therapeutic index at the same time.
  • AIM: The study was conducted to know the results of anterior intratumoral chemotherapy and its comparison to anterior intravenous chemotherapy.
  • MATERIALS AND METHODS: Forty patients of advanced inoperable solid tumors in children (Wilms' tumor and neuroblastoma) between 2000-2004 were randomly allocated to two groups.
  • Group A (20 patients) was given intratumoral chemotherapy while Group B (20 patients) was given intravenous chemotherapy.
  • Both the groups were compared in terms of reduction in size and volume, resectability of tumor, histopathological changes and side-effects of chemotherapeutic drugs.
  • RESULTS: Males were predominant in both type of cases (Wilms' tumor and neuroblastoma) in both the groups (Group A and Group B).
  • All cases in Group A had Stage III disease except three cases which had Stage IV disease (one case of Wilms' tumor and two cases of neuroblastoma) while in Group B only two cases had Stage IV disease (one case of Wilms' tumor and one case of neuroblastoma).
  • Intratumoral chemotherapy was found to be superior over intravenous chemotherapy in terms of reduction of size and volume (63% in Group A vs. 22% in Group B).
  • Moreover, the incidence and severity of side-effects of chemotherapy and morbidity was less in intratumoral chemotherapy.
  • CONCLUSION: In this study intratumoral chemotherapy was found to be superior over intravenous chemotherapy in terms of better and early tumor regression, minimal side-effects, better tumor resectability and well response on histopathological criteria.
  • This study is still going on at our center where different drug combinations, different drug doses, their toxicities, their mechanisms of action, their serum levels and long-term results of intratumoral mode of chemotherapy are to be evaluated thoroughly in future.

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  • (PMID = 19718288.001).
  • [ISSN] 0970-1591
  • [Journal-full-title] Indian journal of urology : IJU : journal of the Urological Society of India
  • [ISO-abbreviation] Indian J Urol
  • [Language] eng
  • [Publication-type] Journal Article; Retracted Publication
  • [Publication-country] India
  • [Other-IDs] NLM/ PMC2721564
  • [Keywords] NOTNLM ; Advanced solid tumors / Wilms' tumor / intratumoral chemotherapy / intravenous chemotherapy / neuroblastoma
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40. Marcus KJ, Shamberger R, Litman H, von Allmen D, Grupp SA, Nancarrow CM, Goldwein J, Grier HE, Diller L: Primary tumor control in patients with stage 3/4 unfavorable neuroblastoma treated with tandem double autologous stem cell transplants. J Pediatr Hematol Oncol; 2003 Dec;25(12):934-40
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  • [Title] Primary tumor control in patients with stage 3/4 unfavorable neuroblastoma treated with tandem double autologous stem cell transplants.
  • OBJECTIVE: To assess the efficacy and toxicity of local radiotherapy in achieving local control in patients with stage 4 or high-risk stage 3 neuroblastoma treated with induction chemotherapy and tandem stem cell transplants.
  • METHODS: Fifty-two children with stage 4 or high-risk stage 3 neuroblastoma were treated on a standardized protocol that included five cycles of induction chemotherapy, surgical resection of the primary tumor when feasible, local radiotherapy, and then consolidation with tandem myeloablative cycles with autologous peripheral blood stem cell rescue.
  • Local radiotherapy (10.5-18 Gy) was administered to patients with gross or microscopic residual disease prior to the myeloablative cycles.
  • The second of the myeloablative regimens included 12 Gy of total body irradiation.
  • Of 11 patients with disease recurrence after completion of therapy, 9 failed in bony metastatic sites 3 to 21 months after the completion of therapy, 1 recurred 67 months following therapy in the previously bulky metastatic site that had been irradiated, and 1 had local recurrence concurrent with distant progression 15 months following the second transplant.
  • CONCLUSIONS: The use of induction chemotherapy, aggressive multimodality therapy for the primary tumor, followed by tandem myeloablative cycles with stem cell transplant in patients with stage 4 or high risk stage 3 neuroblastoma has resulted in acceptable toxicity, a very low local recurrence risk, and an improvement in survival.
  • [MeSH-major] Neuroblastoma / radiotherapy. Peripheral Blood Stem Cell Transplantation / mortality
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / secondary. Child. Child, Preschool. Cohort Studies. Female. Humans. Infant. Male. Myeloablative Agonists / therapeutic use. Neoplasm Staging. Radiotherapy, Adjuvant / adverse effects. Radiotherapy, Adjuvant / standards. Recurrence. Remission Induction / methods. Survival Analysis. Transplantation, Autologous. Treatment Outcome

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  • (PMID = 14663275.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Myeloablative Agonists
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41. Lobato R, Queizán A, Martínez L, Díaz M, Gámez M, Tovar JA: [Impact of complete resection on survival of patients with large neuroblastoma]. Cir Pediatr; 2000 Jan;13(1):14-5
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  • [Title] [Impact of complete resection on survival of patients with large neuroblastoma].
  • The aim of our study is to assess the role of complete resection after chemotherapy in stage 3 and 4 (INSS) neuroblastoma.
  • RESULTS: 14 of 17 patients with stage 3 tumors and only 8 of 23 with stage 4 survive.
  • All patients with stage 3 undergoing complete resection are alive, whereas only 4 of 7 with incomplete resection survive (p < 0.01).
  • In contrast, the effort and risk of resection do not appear to be worth in stage 4.
  • CONCLUSIONS: Complete resection in stage 3 neuroblastoma after chemotherapy improves survival, and radical surgery seems justified even if neighboring structures have to be removed.
  • Radical surgery does not seem to be useful in stage 4 neuroblastoma.
  • [MeSH-major] Ganglioneuroma / mortality. Ganglioneuroma / surgery. Neuroblastoma / mortality. Neuroblastoma / surgery

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  • (PMID = 12602016.001).
  • [ISSN] 0214-1221
  • [Journal-full-title] Cirugía pediátrica : organo oficial de la Sociedad Española de Cirugía Pediátrica
  • [ISO-abbreviation] Cir Pediatr
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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42. Garaventa A, Boni L, Lo Piccolo MS, Tonini GP, Gambini C, Mancini A, Tonegatti L, Carli M, di Montezemolo LC, Di Cataldo A, Casale F, Mazzocco K, Cecchetto G, Rizzo A, Bernardi B, Italian Cooperative Group for Neuroblastoma: Localized unresectable neuroblastoma: results of treatment based on clinical prognostic factors. Ann Oncol; 2002 Jun;13(6):956-64
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  • [Title] Localized unresectable neuroblastoma: results of treatment based on clinical prognostic factors.
  • BACKGROUND: We previously reported that stage 3 neuroblastoma comprises (i) a low-risk group including all infants (age 0-11 months) as well as older children with non-abdominal primaries, and (ii) a high-risk group made up of children >1 year of age with abdominal primaries.
  • Aggressive chemotherapy was effective only in the latter group.
  • PATIENTS AND TREATMENT: On this basis, in 1990 we designed a new protocol by which all low-risk patients received standard-dose chemotherapy, while the high-risk ones received very aggressive chemotherapy.
  • CONCLUSIONS: In low-risk stage 3 neuroblastoma, standard-dose chemotherapy is associated with an excellent chance of being cured.
  • Aggressive chemotherapy is effective for high-risk patients, but results are still unsatisfactory.
  • MYCN gene amplification is a prognostic indicator for most, but not all, treatment failures.

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  • (PMID = 12123342.001).
  • [ISSN] 0923-7534
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide
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43. Sharp SE, Shulkin BL, Gelfand MJ, Salisbury S, Furman WL: 123I-MIBG scintigraphy and 18F-FDG PET in neuroblastoma. J Nucl Med; 2009 Aug;50(8):1237-43
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] 123I-MIBG scintigraphy and 18F-FDG PET in neuroblastoma.
  • The purpose of this study was to compare the diagnostic utility of (123)I-metaiodobenzylguanidine ((123)I-MIBG) scintigraphy and (18)F-FDG PET in neuroblastoma.
  • METHODS: A total of 113 paired (123)I-MIBG and (18)F-FDG PET scans in 60 patients with neuroblastoma were retrospectively reviewed.
  • RESULTS: For stage 1 and 2 neuroblastoma (13 scans, 10 patients), (18)F-FDG depicted more extensive primary or residual neuroblastoma in 9 of 13 scans. (123)I-MIBG and (18)F-FDG showed equal numbers of lesions in 1 of 13 scans, and 3 of 13 scan results were normal.
  • For stage 3 neuroblastoma (15 scans, 10 patients), (123)I-MIBG depicted more extensive primary neuroblastoma or local or regional metastases in 5 of 15 scans. (18)F-FDG depicted more extensive primary neuroblastoma or local or regional metastases in 4 of 15 scans. (123)I-MIBG and (18)F-FDG were equal in 2 of 15 scans, and 4 of 15 scan results were normal.
  • For stage 4 neuroblastoma (85 scans, 40 patients), (123)I-MIBG depicted more neuroblastoma sites in 44 of 85 scans. (18)F-FDG depicted more neuroblastoma sites in 11 of 85 scans. (123)I-MIBG and (18)F-FDG were equivalent or complementary in 13 of 85 scans, and 17 of 85 scan results were normal.
  • CONCLUSION: (18)F-FDG is superior in depicting stage 1 and 2 neuroblastoma, although (123)I-MIBG may be needed to exclude higher-stage disease. (18)F-FDG also provides important information for patients with tumors that weakly accumulate (123)I-MIBG and at major decision points during therapy (i.e., before stem cell transplantation or before surgery). (18)F-FDG can also better delineate disease extent in the chest, abdomen, and pelvis. (123)I-MIBG is overall superior in the evaluation of stage 4 neuroblastoma, especially during initial chemotherapy, primarily because of the better detection of bone or marrow metastases.
  • [MeSH-major] 3-Iodobenzylguanidine. Fluorodeoxyglucose F18. Neuroblastoma / radionuclide imaging. Positron-Emission Tomography / methods. Radiopharmaceuticals

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  • [CommentIn] J Nucl Med. 2010 Feb;51(2):330-1; author reply 331 [20080890.001]
  • [CommentIn] J Nucl Med. 2010 Feb;51(2):330; author reply 331 [20080883.001]
  • (PMID = 19617326.001).
  • [ISSN] 0161-5505
  • [Journal-full-title] Journal of nuclear medicine : official publication, Society of Nuclear Medicine
  • [ISO-abbreviation] J. Nucl. Med.
  • [Language] eng
  • [Publication-type] Comparative Study; Evaluation Studies; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18; 35MRW7B4AD / 3-Iodobenzylguanidine
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44. Albert A, Cruz O, Montaner A, Vela A, Badosa J, Castañón M, Morales L: [Congenital solid tumors. A thirteen-year review]. Cir Pediatr; 2004 Jul;17(3):133-6
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  • This is an interesting group of tumors because their type, relative incidence, natural history and response to treatment differ from those seen in older children.
  • Neuroblastoma was the commonest tumor (10 cases, 37%), of which 4 were stage I, 4 stage IV-S and 2 stage III.
  • Treatment was surgical resection alone in 17 cases (68%) and surgery + chemotherapy in 8 (32%) (5 neuroblastomas, one CNS tumor, one Wilms tumor and one presacral teratoma who developed a yolk sac tumor); 3 patients died (11%): one at surgery, one of tumoural airway obstruction at birth and one with craniopharyngioma.
  • Among the 14 tumors that were initially not malignant, two can be locally agressive, one was an immature teratoma, the giant nevus with hamartoma developed in situ melanoma, the other nevus had meningeal melanosis with hydrocephalus, and one mature presacral teratoma developed a yolk sac tumor.
  • Complete surgical excision is the treatment of choice, most cases not need adjuvant chemotherapy.
  • [MeSH-major] Central Nervous System Neoplasms / congenital. Kidney Neoplasms / congenital. Liver Neoplasms / congenital. Neuroblastoma / congenital. Skin Neoplasms / congenital. Soft Tissue Neoplasms / congenital. Teratoma / congenital. Wilms Tumor / congenital
  • [MeSH-minor] Female. Follow-Up Studies. Humans. Infant, Newborn. Male. Neoplasm Recurrence, Local. Postoperative Complications. Pregnancy. Prenatal Diagnosis. Time Factors

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  • (PMID = 15503950.001).
  • [ISSN] 0214-1221
  • [Journal-full-title] Cirugía pediátrica : organo oficial de la Sociedad Española de Cirugía Pediátrica
  • [ISO-abbreviation] Cir Pediatr
  • [Language] spa
  • [Publication-type] Comparative Study; English Abstract; Journal Article
  • [Publication-country] Spain
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45. Zachariou Z, Sieverts H, Eble MJ, Gfrörer S, Zavitzanakis A: IORT (intraoperative radiotherapy) in neuroblastoma: experience and first results. Eur J Pediatr Surg; 2002 Aug;12(4):251-4
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  • [Title] IORT (intraoperative radiotherapy) in neuroblastoma: experience and first results.
  • Intraoperative radiotherapy (IORT) permits the application of a single large radiation dose to a malignant mass at the time of surgery sparing adjacent normal tissue from irradiation.
  • Since 1996 we have used IORT to treat 13 children with neuroblastoma, stage 3 - 4.
  • Ultrasound, CT and MRI were performed and patients were treated with chemotherapy according to the NB90 protocol.
  • Localised radiation of the residual tumour was 8 - 10 Gy.
  • The follow-up time was 6 - 69 months (May 2001).
  • [MeSH-major] Abdominal Neoplasms / radiotherapy. Neuroblastoma / radiotherapy
  • [MeSH-minor] Child. Child, Preschool. Combined Modality Therapy. Follow-Up Studies. Humans. Intraoperative Period. Neoplasm Staging. Retrospective Studies. Second-Look Surgery

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  • (PMID = 12369003.001).
  • [ISSN] 0939-7248
  • [Journal-full-title] European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift für Kinderchirurgie
  • [ISO-abbreviation] Eur J Pediatr Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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46. Morovic A, Damjanov I: Neuroectodermal ovarian tumors: a brief overview. Histol Histopathol; 2008 06;23(6):765-71
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  • Primary neuroectodermal tumors of the ovary are rare monophasic teratomas composed exclusively or almost exclusively of neuroectodemal tissue.
  • These tumors were classified as ependymoma, astrocytoma, glioblastoma multiforme, ependymoblastoma or as primitive neuroepithelial tumors such as medullo-blastoma, medulloepithelioma and neuroblastoma.
  • The review of the literature shows that most patients with clinical stage I and II were treated surgically, whereas those with stage III or IV tumors received additional radiation or chemotherapy, or both.
  • The clinical stage at the time of diagnosis is the most important prognostic parameter of these tumors.
  • [MeSH-minor] Biomarkers, Tumor / analysis. Combined Modality Therapy. Female. Humans. Immunohistochemistry. Neoplasm Staging. Prognosis

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  • (PMID = 18366014.001).
  • [ISSN] 1699-5848
  • [Journal-full-title] Histology and histopathology
  • [ISO-abbreviation] Histol. Histopathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Spain
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
  • [Number-of-references] 30
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47. Terenziani M, D'Angelo P, Bisogno G, Boldrini R, Cecchetto G, Collini P, Conte M, De Laurentis T, Ilari I, Indolfi P, Inserra A, Piva L, Siracusa F, Spreafico F, Tamaro P, Lo Curto M: Teratoma with a malignant somatic component in pediatric patients: the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) experience. Pediatr Blood Cancer; 2010 Apr;54(4):532-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • PROCEDURE: The Associazione Italiana Ematologia Oncologia Pediatrica identified 14 cases of TMSC.
  • RESULTS: The series (9 female, 5 male) showed the following disease: testis (2), sacrococcygeal (3), ovary (3), retroperitoneum (3), mediastinum (2), and foot soft tissue (1).
  • Distribution of the somatic component was: carcinoma (4), pancreatic neuroendocrine tumor (1), neuroblastoma (3), rhabdomyosarcoma (3), rhabdomyosarcoma plus liposarcoma, chondrosarcoma, neurogenic sarcoma (1), chondrosarcoma plus neuroectodermal sarcoma (1), malignant peripheral nerve sheath tumor (1).
  • Three patients were in stage I, four in stage II, three in stage III, and four in stage IV.
  • Chemotherapy optimized for histology should include reagents directed to the somatic malignancy, if chemosensitive.
  • Malignant GCT warrants GCT-directed chemotherapy.
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Female. Humans. Infant. Infant, Newborn. Italy. Male. Neoplasm Staging. Prognosis. Retrospective Studies. Treatment Outcome

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  • (PMID = 20049928.001).
  • [ISSN] 1545-5017
  • [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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48. Tröbs RB, Hänsel M, Friedrich T, Bennek J: A 23-year experience with malignant renal tumors in infancy and childhood. Eur J Pediatr Surg; 2001 Apr;11(2):92-8
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  • A retrospective analysis of 77 children treated between 1974 and 1996 was undertaken to evaluate morbidity and the evolution of therapy.
  • We observed 3 children of school age with renal carcinoma and one patient with an intrarenal neuroblastoma.
  • Comparing relapse-free survival of stages I, II and III, respectively, there was a reduced survival rate for stage III (p=0.019).
  • According to the SIOP/GPOH protocol in 1989, the regimen was switched from primary surgery to preoperative chemotherapy without biopsy in 1989 (11 pats.).
  • During preoperative chemotherapy a venous occlusive disease of the liver occurred in 2 patients.
  • Preoperative chemotherapy led to an impressive tumor shrinkage in the majority of patients.
  • In our experience, reduction of tumor volume due to preoperative chemotherapy facilitates tumor removal by surgery and may prevent tumor spillage and the deleterious effects of radiation in young children.
  • Surgery without delay is necessary if the diagnosis is unclear or the tumor fails to respond to preoperative chemotherapy.
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Chemotherapy, Adjuvant. Child. Child, Preschool. Disease-Free Survival. Female. Humans. Infant. Male. Neoplasm Recurrence, Local / epidemiology. Neoplasm Staging. Prognosis. Retrospective Studies

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  • (PMID = 11371043.001).
  • [ISSN] 0939-7248
  • [Journal-full-title] European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift für Kinderchirurgie
  • [ISO-abbreviation] Eur J Pediatr Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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49. Costa C, Rocha G, Grilo M, Bianchi R, Sotto Mayor T, Monteiro J, Guimarães H: [Neonatal tumors]. Acta Med Port; 2010 May-Jun;23(3):405-12

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • INTRODUCTION: Tumors affecting the fetus and newborn differ from those found in older children and adults, leading to new diagnostic and therapeutic challenges.
  • RESULTS: Total = 32 cases, 16M/16F, birth weight: 3146 g (965-4590), gestational age 38 weeks (28-41), seven (22%) preterm, C-section rate 75% (n = 24), two with EXIT procedure.
  • DIAGNOSIS: Teratoma (n = 8); lymphangioma (n = 7), neuroblastoma (n = 6), haemangioma (n = 5), other solid tumors (n = 6); acute lymphoblastic leukemia (n = 1).
  • NEUROBLASTOMA: abdominal location (n = 5); cervical location (n = 1); deletion 1p (n = 0); oncogene n-myc amplification (n = 0); stage I (n = 1); IIB (n = 1); III (n = 3); IV (n = 1).
  • Chemotherapy (n = 5), according to the <<European Infant Neuroblastoma Study>> (n = 2), surgical resection (n = 4).

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  • (PMID = 20654259.001).
  • [ISSN] 1646-0758
  • [Journal-full-title] Acta médica portuguesa
  • [ISO-abbreviation] Acta Med Port
  • [Language] por
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Portugal
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50. Hu WH, Xie FY, Fang SH, Jiao JJ, Yan C, Peng WJ, Fu XY, Zhang F: [Cancer of the nasal cavity]. Zhonghua Zhong Liu Za Zhi; 2005 Feb;27(2):117-21
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  • The 5-year survival rate was 55.8% in squamous-cell carcinoma, 44.0% in adenocarcinoma, 59.7% in undifferentiated carcinoma, 76.3% in adenoid cystic carcinoma, 71.4% in mucoepidermoid carcinoma, 25.0% in rhabdomyosarcoma, 26.7% in malignant melanoma, 50.0% in neuroblastoma (P > 0.05).
  • The 5-year survival rate was 73.8% in patients whose cancer completely disappeared after treatment.
  • The 5-year survival was 78.3% in stage I disease, 56.4% in stage II disease, 54.2% in stage III and 35.9% in stage IV (P < 0.05).
  • That with chemotherapy only was 25.0% whereas that of patients treated with combination treatment was 61.8% (P > 0.05).
  • CONCLUSION: Clinical stage, immediate therapeutic response and involvement of sphenoidal or maxillary sinus; but not the pathologic type, the presence of cervical metastasis nor the method of treatment, are the factors affecting the prognosis of patients with nasal carcinoma.
  • [MeSH-major] Nasal Cavity. Nose Neoplasms / mortality. Nose Neoplasms / therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Combined Modality Therapy. Female. Humans. Male. Middle Aged. Multivariate Analysis. Prognosis. Survival Rate

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  • (PMID = 15946555.001).
  • [ISSN] 0253-3766
  • [Journal-full-title] Zhonghua zhong liu za zhi [Chinese journal of oncology]
  • [ISO-abbreviation] Zhonghua Zhong Liu Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] China
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51. Perek D, Brozyna A, Dembowska-Baginska B, Stypinska M, Sojka M, Bacewicz L, Polnik D, Kalicinski P: [Tumours in newborns and infants up to three months of life. One institution experience]. Med Wieku Rozwoj; 2006 Jul-Sep;10(3 Pt 1):711-23
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • INTRODUCTION: Newborns and infants up to three months of life are a specific group of population in paediatric oncology due to immaturity of tissues and organs and rarity of neoplastic diseases in this group of patients (pts).
  • There are no strict therapeutic procedures established for these children.
  • THE AIM of our study was to examine distribution of tumours in newborns and infants up to 3 months of age treated in our institution and to present our own experience in the treatment of these patients.
  • Distribution of tumour types in newborns and babies from 1 to 3 months of age was analyzed separately.
  • Due to similar growth pattern, response to treatment and it's side effects in newborns and small infants, treatment results were evaluated for the whole group.
  • The second most common was neuroblastoma (NBL) 22%.
  • There were also 3 cases of soft tissue sarcomas (STS), 2 central nervous system tumours (CNS), 2 retinoblastoma (RB), 2 hepatoblastoma (HB).
  • It concerned pts with MT--28, IT--3 pts, yolk sac tumour (YST)--1 pt and malignant tumours (stage I and II): 8-NBL, 2-CNS tumours, 2 STS, 3-HB, 1-WT.
  • Eleven pts underwent combined treatment of chemotherapy and surgery: 5 with stage III and IV NBL, 6 with other tumours.
  • Chemotherapy alone was administered to 7 pts in whom local advancement of disease enabled surgery and to pts with RBL.
  • Four pts with NBL (2 stage IV and 2 stage IVS) were treated with irradiation to the liver only.
  • One pt, critically ill, died before any treatment.
  • GCT and neuroblastoma are the most common tumours in newborns and infants up to 3 months of age.
  • 2. Newborns and small infants with advanced neoplastic disease, similarly to older children can be cured with chemotherapy.
  • 3. Individual approach is warranted in newborns and small infants and treatment should be carried out in specialized centres.
  • 4. All patients who completed treatment of any tumour type should be followed up by a pediatric oncologist.
  • [MeSH-major] Infant Welfare / statistics & numerical data. Neoplasms / epidemiology. Neoplasms / therapy

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  • (PMID = 17317902.001).
  • [Journal-full-title] Medycyna wieku rozwojowego
  • [ISO-abbreviation] Med Wieku Rozwoj
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
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52. Thomas SE, Hutchinson RJ, DebRoy M, Magee JC: Successful renal transplantation following prior bone marrow transplantation in pediatric patients. Pediatr Transplant; 2004 Oct;8(5):507-12
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Improving survival rates following pediatric bone marrow transplantation (BMT) will likely result in greater numbers of children progressing to end-stage renal disease (ESRD) because of prior chemotherapy, irradiation, sepsis, and exposure to nephrotoxic agents.
  • Renal transplantation remains the treatment of choice for ESRD; however, the safety of renal transplantation in this unique population is not well established.
  • Two patients with neuroblastoma and ESRD because of BMT nephropathy, and one patient with Schimke immuno-osseous dysplasia and ESRD because of immune complex mediated glomerulonephritis and nephrotic syndrome.
  • Age at time of BMT ranged from 2 to 7 yr.
  • One patient with a history of grade III skin and grade IV gastrointestinal-graft-vs.-host disease (GI-GVHD) prior to transplantation, had a mild flare of GI-GVHD (grade I) post-renal transplant and is currently asymptomatic.
  • Renal transplantation is an excellent option for the treatment of pediatric patients with ESRD following BMT.
  • [MeSH-major] Bone Marrow Transplantation / adverse effects. Kidney Failure, Chronic / therapy. Kidney Transplantation
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Graft vs Host Disease / etiology. Humans. Immunosuppression. Infant. Opportunistic Infections / etiology. Postoperative Care. Treatment Outcome






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