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1. Gardner SL, Carreras J, Boudreau C, Camitta BM, Adams RH, Chen AR, Davies SM, Edwards JR, Grovas AC, Hale GA, Lazarus HM, Arora M, Stiff PJ, Eapen M: Myeloablative therapy with autologous stem cell rescue for patients with Ewing sarcoma. Bone Marrow Transplant; 2008 May;41(10):867-72
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  • [Title] Myeloablative therapy with autologous stem cell rescue for patients with Ewing sarcoma.
  • The aim of this study was to identify risk factors associated with PFS in patients with Ewing sarcoma undergoing ASCT; 116 patients underwent ASCT in 1989-2000 and reported to the Center for International Blood and Marrow Transplant Research.
  • Eighty patients (69%) received ASCT as first-line therapy and 36 (31%), for recurrent disease.
  • Five-year probabilities of PFS in patients with localized and metastatic disease at diagnosis who received ASCT as first-line therapy were 49% (95% CI 30-69) and 34% (95% CI 22-47) respectively.
  • PFS rates after ASCT are comparable to published rates in patients with similar disease characteristics treated with conventional chemotherapy, surgery and irradiation suggesting a limited role for ASCT in these patients.

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  • [CommentIn] Bone Marrow Transplant. 2008 Dec;42(11):761; author reply 763 [18679365.001]
  • (PMID = 18246113.001).
  • [ISSN] 0268-3369
  • [Journal-full-title] Bone marrow transplantation
  • [ISO-abbreviation] Bone Marrow Transplant.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / U24 CA076518; United States / NCI NIH HHS / CA / U24 CA076518-10; United States / NCI NIH HHS / CA / U24 CA 76518
  • [Publication-type] Journal Article; Multicenter Study; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Myeloablative Agonists
  • [Other-IDs] NLM/ NIHMS57143; NLM/ PMC3164955
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2. Jedlicka P: Ewing Sarcoma, an enigmatic malignancy of likely progenitor cell origin, driven by transcription factor oncogenic fusions. Int J Clin Exp Pathol; 2010;3(4):338-47
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  • [Title] Ewing Sarcoma, an enigmatic malignancy of likely progenitor cell origin, driven by transcription factor oncogenic fusions.
  • Since its first description by James Ewing in 1921, Ewing Sarcoma has been a cryptic malignancy.
  • A poorly differentiated tumor of uncertain histogenesis and aggressive biologic behavior, it is the second most common malignancy of bone and soft tissue affecting adolescents and young adults.
  • Some two decades ago, the understanding of Ewing Sarcoma biology took a leap forward with the identification of recurrent EWS/Ets fusions, which drive onco-genesis in this disease.
  • Improved understanding of EWS/Ets biology and relevant oncogenic pathways has in turn led to the development of targeted therapies, including, recently, small molecules targeting key complexes involving the oncogenic fusion itself.
  • In many respects still remaining an enigma, Ewing Sarcoma is an important model for cancers originating in progenitor-type cells or manifesting progenitor-type cell features, and cancers containing recurrent oncogenic fusions, the latter a surprisingly expanding number.
  • [MeSH-major] Oncogene Proteins, Fusion / genetics. Proto-Oncogene Proteins c-ets / genetics. RNA-Binding Protein EWS / genetics. Sarcoma, Ewing / genetics. Stem Cells / pathology
  • [MeSH-minor] Animals. Bone Neoplasms / drug therapy. Bone Neoplasms / genetics. Bone Neoplasms / pathology. Cell Transformation, Neoplastic / genetics. Cell Transformation, Neoplastic / pathology. Gene Expression. Humans. Soft Tissue Neoplasms / drug therapy. Soft Tissue Neoplasms / genetics. Soft Tissue Neoplasms / pathology

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  • (PMID = 20490326.001).
  • [ISSN] 1936-2625
  • [Journal-full-title] International journal of clinical and experimental pathology
  • [ISO-abbreviation] Int J Clin Exp Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Oncogene Proteins, Fusion; 0 / Proto-Oncogene Proteins c-ets; 0 / RNA-Binding Protein EWS
  • [Number-of-references] 73
  • [Other-IDs] NLM/ PMC2872742
  • [Keywords] NOTNLM ; Ewing Sarcoma / fusion oncogene / progenitor cell / transcription factor
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3. de Hooge AS, Berghuis D, Santos SJ, Mooiman E, Romeo S, Kummer JA, Egeler RM, van Tol MJ, Melief CJ, Hogendoorn PC, Lankester AC: Expression of cellular FLICE inhibitory protein, caspase-8, and protease inhibitor-9 in Ewing sarcoma and implications for susceptibility to cytotoxic pathways. Clin Cancer Res; 2007 Jan 1;13(1):206-14
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  • [Title] Expression of cellular FLICE inhibitory protein, caspase-8, and protease inhibitor-9 in Ewing sarcoma and implications for susceptibility to cytotoxic pathways.
  • PURPOSE: Ewing sarcoma is a common pediatric bone tumor with an unfavorable prognosis for metastatic or recurrent disease.
  • Cellular immunotherapy may provide new treatment options and depends on the cytolytic death receptor and perforin/granzyme pathways.
  • Expression of death receptor pathway inhibitor cellular FLICE inhibitory protein (cFLIP), initiator caspase-8, and granzyme B inhibitor protease inhibitor-9 (PI-9) have been reported to determine susceptibility to cell- and chemotherapy-mediated killing in several tumor types.
  • Here, we have studied their in vitro and in vivo expression in Ewing sarcoma and the implications for susceptibility to cytotoxicity.
  • EXPERIMENTAL DESIGN: Ewing sarcoma cell lines (n = 8) were tested for cFLIP, PI-9, and caspase-8 expression.
  • Functional significance was tested by anti-Fas antibody (death receptor pathway) or natural killer cell (perforin/granzyme pathway) treatment.
  • RESULTS: Although all tested Ewing sarcoma cell lines expressed cFLIP, resistance to CD95/Fas-mediated apoptosis was only observed in two cell lines lacking caspase-8 expression.
  • PI-9 was expressed at low levels in four of eight Ewing sarcoma cell lines, but positive cell lines remained susceptible to perforin/granzyme-mediated killing.
  • In primary Ewing sarcoma, including metastases, cFLIP was abundantly expressed in 18 of 18 patients.
  • CONCLUSIONS: The expression patterns of cFLIP, caspase-8, and the absence of PI-9 provide a rationale to preferentially exploit the perforin/granzyme pathway in cytotoxic therapies against Ewing sarcoma.
  • [MeSH-major] Biomarkers, Tumor. CASP8 and FADD-Like Apoptosis Regulating Protein / biosynthesis. Caspase 8 / biosynthesis. Gene Expression Regulation, Neoplastic. Genetic Predisposition to Disease. Sarcoma, Ewing / genetics. Sarcoma, Ewing / metabolism. Serpins / biosynthesis

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  • (PMID = 17200356.001).
  • [ISSN] 1078-0432
  • [Journal-full-title] Clinical cancer research : an official journal of the American Association for Cancer Research
  • [ISO-abbreviation] Clin. Cancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD95; 0 / Biomarkers, Tumor; 0 / CASP8 and FADD-Like Apoptosis Regulating Protein; 0 / SERPINB9 protein, human; 0 / Serpins; EC 3.4.22.- / Caspase 8
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4. Whelan JS, McTiernan A, Kakouri E, Kilby A, London Bone and Soft Tissue Tumour Service: Carboplatin-based chemotherapy for refractory and recurrent Ewing's tumours. Pediatr Blood Cancer; 2004 Sep;43(3):237-42
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  • [Title] Carboplatin-based chemotherapy for refractory and recurrent Ewing's tumours.
  • BACKGROUND: Failure of first line therapy for the Ewing's family of tumours (EFT) is associated with a very poor outlook.
  • Studies of second line chemotherapy are therefore necessary to identify active agents and drug combinations.
  • Cisplatin-based therapy is frequently used in these circumstances but there are few studies to clearly define activity and toxicity.
  • PROCEDURE: Between 1990 and 1998, 23 males and 16 females aged between 6 and 48 years (median 23) with relapsed or refractory EFT were treated with carboplatin-based chemotherapy.
  • Previous chemotherapy had included ifosfamide and doxorubicin in all but two patients.
  • Twenty patients were treated at the time of recurrence, and 19 after a poor response to initial chemotherapy.
  • Treatment comprised of carboplatin to give an area under the plasma carboplatin concentration versus time curve of (AUC) 6 mg/ml, etoposide 120 mg/m2 for 3 days, and cyclophosphamide 500-750 mg/m2 for 2 days, repeated every 21 days.
  • Median time to progression was 10 weeks (range 2-54).
  • Six patients proceeded to high dose consolidation treatment with bone marrow or peripheral stem cell rescue.
  • [MeSH-major] Carboplatin / therapeutic use. Drug Resistance, Neoplasm. Sarcoma, Ewing / drug therapy. Sarcoma, Ewing / pathology

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  • [Copyright] Copyright 2004 Wiley-Liss, Inc.
  • (PMID = 15266407.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] BG3F62OND5 / Carboplatin
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5. Maheshwari AV, Cheng EY: Ewing sarcoma family of tumors. J Am Acad Orthop Surg; 2010 Feb;18(2):94-107
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  • [Title] Ewing sarcoma family of tumors.
  • The Ewing sarcoma family of tumors (ESFT) consists of a group of tumors characterized by morphologically similar round-cell neoplasm and by the presence of a common chromosomal translocation.
  • Although rare, such tumors constitute the third most frequent primary sarcoma of bone after osteosarcoma and chondrosarcoma.
  • Because most patients with clinically apparent localized disease at diagnosis may also have occult metastatic (ie, systemic) disease, multidrug chemotherapy as well as local disease control with surgery and/or radiation therapy are indicated for all patients.
  • Despite marked improvements in survival during the past 40 years for patients with localized disease, lesser improvements have been seen in patients with metastatic or recurrent disease.
  • A better understanding of the complex biology of ESFT may lead to the successful development of biologically targeted therapies.
  • As the regulatory pathways responsible for transformation, growth, and metastasis of ESFT become more refined, the number of potential therapeutic targets will expand.
  • [MeSH-major] Bone Neoplasms / surgery. Sarcoma, Ewing / surgery
  • [MeSH-minor] Algorithms. Combined Modality Therapy. Humans. Neoplasm Recurrence, Local. Neoplasm Staging. Neoplasms, Second Primary. Prognosis. Translocation, Genetic

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  • (PMID = 20118326.001).
  • [ISSN] 1067-151X
  • [Journal-full-title] The Journal of the American Academy of Orthopaedic Surgeons
  • [ISO-abbreviation] J Am Acad Orthop Surg
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 74
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6. Hoffer FA: Primary skeletal neoplasms: osteosarcoma and ewing sarcoma. Top Magn Reson Imaging; 2002 Aug;13(4):231-9
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  • [Title] Primary skeletal neoplasms: osteosarcoma and ewing sarcoma.
  • The care for children and adolescents with malignant bone tumors such as osteosarcoma and Ewing sarcoma will be improved by the magnetic resonance imager that can recognize the tumor early in its course and can stage it correctly for proper chemotherapy and local control.
  • Determination of response on follow-up static and dynamic contrast-enhanced magnetic resonance imaging and knowledge of optimal methods to determine recurrent tumor will improve the outcome for these children.
  • [MeSH-major] Bone Neoplasms / diagnosis. Magnetic Resonance Imaging / methods. Osteosarcoma / diagnosis. Sarcoma, Ewing / diagnosis

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  • (PMID = 12409691.001).
  • [ISSN] 0899-3459
  • [Journal-full-title] Topics in magnetic resonance imaging : TMRI
  • [ISO-abbreviation] Top Magn Reson Imaging
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 20180; United States / NCI NIH HHS / CA / CA 21765; United States / NCI NIH HHS / CA / CA 23099
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Review
  • [Publication-country] United States
  • [Number-of-references] 20
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7. Lashkari A, Chow WA, Valdes F, Leong L, Phan V, Twardowski P, Kapoor N, Molina A, Al-Kadhimi Z, Frankel P, Somlo G: Tandem high-dose chemotherapy followed by autologous transplantation in patients with locally advanced or metastatic sarcoma. Anticancer Res; 2009 Aug;29(8):3281-8
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  • [Title] Tandem high-dose chemotherapy followed by autologous transplantation in patients with locally advanced or metastatic sarcoma.
  • BACKGROUND: Patients with locally advanced or metastatic/recurrent soft tissue and Ewing's sarcoma (EWS) have few treatment options.
  • The purpose of our phase II study was to assess the feasibility, safety and efficacy of tandem high-dose chemotherapy (HDCT) followed by autologous stem cell transplantation (ASCT) in such patients.
  • No treatment-related mortality occurred and grade 3 or 4 toxicity was clinically tolerable.
  • Out of the four patients still alive, two had EWS and measurable disease at the time of ASCT and achieved a complete remission, remaining progression free 126 and 155 months after ASCT.
  • CONCLUSION: Our study demonstrates the feasibility and safety of tandem HDCT in patients with high-risk or metastatic/recurrent sarcoma, with some patients achieving long-term PFS and OS.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / therapy. Hematopoietic Stem Cell Transplantation. Neoplasm Recurrence, Local / therapy. Sarcoma / therapy
  • [MeSH-minor] Adult. Cisplatin / administration & dosage. Combined Modality Therapy. Disease Progression. Doxorubicin / administration & dosage. Feasibility Studies. Female. Humans. Ifosfamide / administration & dosage. Immunoenzyme Techniques. Male. Melphalan / administration & dosage. Mesna / administration & dosage. Neoplasm Staging. Neuroectodermal Tumors, Primitive, Peripheral / pathology. Neuroectodermal Tumors, Primitive, Peripheral / therapy. Osteosarcoma / pathology. Osteosarcoma / therapy. Prognosis. Prospective Studies. Protective Agents / administration & dosage. Remission Induction. Rhabdomyosarcoma / pathology. Rhabdomyosarcoma / therapy. Safety. Sarcoma, Ewing / pathology. Sarcoma, Ewing / therapy. Survival Rate. Transplantation, Autologous. Treatment Outcome. Young Adult

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  • (PMID = 19661346.001).
  • [ISSN] 1791-7530
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase II; Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Protective Agents; 80168379AG / Doxorubicin; NR7O1405Q9 / Mesna; Q20Q21Q62J / Cisplatin; Q41OR9510P / Melphalan; UM20QQM95Y / Ifosfamide
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8. Avigad S, Cohen IJ, Zilberstein J, Liberzon E, Goshen Y, Ash S, Meller I, Kollender Y, Issakov J, Zaizov R, Yaniv I: The predictive potential of molecular detection in the nonmetastatic Ewing family of tumors. Cancer; 2004 Mar 1;100(5):1053-8
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  • [Title] The predictive potential of molecular detection in the nonmetastatic Ewing family of tumors.
  • BACKGROUND: Tumors in the Ewing family (EFTs) are the second most common bone tumors in children and adolescents.
  • Despite aggressive chemotherapy, one-third of patients with localized tumor still may develop recurrences.
  • METHODS: The authors report the prognostic potential of the positive chimeric transcript (EWS/FLI1) in bone marrow (BM) and/or peripheral blood (PBL) in 26 patients with EFT during a long follow-up period (median, 61 months).
  • In 10 of 11 patients who developed disease progression, BM and/or PBL samples were positive for the chimeric transcript before evidence of overt clinical recurrence.
  • CONCLUSIONS: Occult tumor cells in BM and/or PBL samples during long follow-up are strong predictors of recurrent disease in patients with nonmetastatic EFTs.
  • [MeSH-major] Bone Neoplasms / genetics. Genetic Predisposition to Disease. Recombinant Fusion Proteins / genetics. Reverse Transcriptase Polymerase Chain Reaction. Sarcoma, Ewing / genetics. Transcription, Genetic
  • [MeSH-minor] Adolescent. Adult. Age Distribution. Bone Marrow / pathology. Chi-Square Distribution. Child. Child, Preschool. DNA Primers. Disease-Free Survival. Female. Humans. Incidence. Male. Molecular Biology. Neoplastic Cells, Circulating. Predictive Value of Tests. Proportional Hazards Models. Prospective Studies. Risk Factors. Sampling Studies. Sex Distribution. Survival Analysis

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  • [Copyright] Copyright 2004 American Cancer Society.
  • (PMID = 14983502.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA Primers; 0 / Recombinant Fusion Proteins
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9. El Weshi A, Memon M, Raja M, Bazarbashi S, Rahal M, El Foudeh M, Pai C, Allam A, El Hassan I, Ezzat A: VIP (etoposide, ifosfamide, cisplatin) in adult patients with recurrent or refractory Ewing sarcoma family of tumors. Am J Clin Oncol; 2004 Oct;27(5):529-34
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] VIP (etoposide, ifosfamide, cisplatin) in adult patients with recurrent or refractory Ewing sarcoma family of tumors.
  • Despite the fact that Ewing sarcoma family of tumors (ET) is chemosensitive, long-term survival is extremely rare for patients with primary refractory or recurrent disease.
  • There is no standard salvage chemotherapy regimen available in this context.
  • In this study the authors reviewed their experience with the combination of etoposide, ifosfamide, and cisplatin in adult patients with recurrent or refractory disease.
  • From February 1997 through December 2001, they evaluated the efficacy of etoposide (75 mg/m2/day for 5 days), ifosfamide (1,200 mg/m2/day for 5 days), and cisplatin (20 mg/m2/day for 5 days) combination chemotherapy (VIP regimen), as second-line salvage therapy in 27 patients with recurrent or refractory ET.
  • All patients were evaluated for response, time to progression, and overall survival.
  • Twenty-one male and 6 female patients with recurrent (n = 14) and refractory (n = 13) disease were treated with the VIP regimen.
  • Sites of recurrent or progressive disease included local (n = 3), distant (n = 11), and both local and distant (n = 13).
  • Median time to progression and median overall survival were 6.6 months and 8.1 months respectively for all patients, and 12.8 months and 14.2 months respectively for responders.
  • The authors conclude that the VIP combination is active in patients with recurrent/refractory ET, with acceptable toxicity, and offers good palliation.
  • Cisplatin-based combination chemotherapy merits further investigation, possibly as first-line treatment in this disease.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / drug therapy. Cisplatin / therapeutic use. Etoposide / therapeutic use. Ifosfamide / therapeutic use. Sarcoma, Ewing / drug therapy
  • [MeSH-minor] Adolescent. Adult. Drug Resistance, Neoplasm. Female. Humans. Male. Neoplasm Recurrence, Local / drug therapy. Salvage Therapy. Survival Analysis

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  • (PMID = 15596925.001).
  • [ISSN] 1537-453X
  • [Journal-full-title] American journal of clinical oncology
  • [ISO-abbreviation] Am. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; Q20Q21Q62J / Cisplatin; UM20QQM95Y / Ifosfamide; ICE protocol 1
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10. Karosas AO: Ewing's sarcoma. Am J Health Syst Pharm; 2010 Oct 1;67(19):1599-605
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  • [Title] Ewing's sarcoma.
  • PURPOSE: The current treatments of and new therapeutic options for the management of Ewing's sarcoma (ES) are reviewed.
  • SUMMARY: ES is the second most common primary bone malignancy in pediatric patients and is numbered among the cancers that result in the greatest risk of mortality and morbidity in children and young adults.
  • Much progress has been made in the treatment of ES since the disease was first described in the 1920s.
  • With current multimodality treatment including chemotherapy, radiation, and surgery, patients with localized disease have a long-term survival rate of approximately 50%.
  • New combinations of cytotoxic agents such as cyclophosphamide, topotecan, irinotecan, and temozolomide have shown efficacy and tolerability in patients with relapsed or refractory disease.
  • To date, the role of high-dose chemotherapy supported by stem cell rescue as a consolidation therapy for high-risk ES tumors has yet to be conclusively determined.
  • Much effort is being invested in treating cancer with targeted therapies, and the EWS-ETS fusion gene would likely provide an important tumor-specific target.
  • Tyrosine kinases (TKs) are overexpressed in human sarcoma tumors, and cell lines may serve as potential targets for new therapies.
  • One TK receptor that is a promising therapeutic target is insulinlike growth factor-1 receptor.
  • CONCLUSION: Treatments for ES include surgery, radiation, and cytotoxic regimens, many of which include vincristine.
  • Treatment for recurrent ES has included topotecan, cyclophosphamide, temozolomide, and irinotecan.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / therapy. Sarcoma, Ewing / therapy
  • [MeSH-minor] Adult. Child. Combined Modality Therapy. Drug Delivery Systems. Humans. Neoplasm Recurrence, Local. Survival Rate. Young Adult

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  • (PMID = 20852160.001).
  • [ISSN] 1535-2900
  • [Journal-full-title] American journal of health-system pharmacy : AJHP : official journal of the American Society of Health-System Pharmacists
  • [ISO-abbreviation] Am J Health Syst Pharm
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
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11. Hunold A, Weddeling N, Paulussen M, Ranft A, Liebscher C, Jürgens H: Topotecan and cyclophosphamide in patients with refractory or relapsed Ewing tumors. Pediatr Blood Cancer; 2006 Nov;47(6):795-800
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  • [Title] Topotecan and cyclophosphamide in patients with refractory or relapsed Ewing tumors.
  • BACKGROUND: The prognosis of Ewing tumor (ET) patients has significantly improved to cure rates approximating 70%.
  • Promising response rates have recently been reported for the combination of topotecan (TOPO) and cyclophosphamide (CYC) encouraging wider application of this combination in patients with relapsed ETs.
  • This report summarizes the experience of patients treated with TOPO/CYC for recurrent or refractory disease within the German ET trials.
  • PROCEDURE: Fifty-four patients aged 3.2-49.5 (median: 17.4) years received TOPO (0.75 mg/m2/day, days 1-5) and CYC (250 mg/m2/day, days 1-5) following first (40) or second (6) relapse or progression under first-line therapy (8).
  • At completion of relapse therapy, 24/54 patients had entered complete (19) or partial (5) remission, 2 had SD, 26 showed progression, information was unavailable in 2 patients.
  • At the time of evaluation, after a median follow-up for survivors of 23.1 (range: 7.8-59.8) months from the event prompting TOPO/CYC treatment, 14/54 patients (25.9%) were in continuous complete (13) or partial (1) remission.
  • CONCLUSION: TOPO/CYC is active in relapsed ETs and warrants further evaluation.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / drug therapy. Cyclophosphamide / administration & dosage. Neoplasm Recurrence, Local / drug therapy. Sarcoma, Ewing / drug therapy. Topotecan / administration & dosage
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Disease Progression. Dose-Response Relationship, Drug. Female. Follow-Up Studies. Humans. Male. Middle Aged. Prognosis. Recurrence. Remission Induction. Salvage Therapy. Survival Rate. Treatment Outcome

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  • [Copyright] (c) 2006 Wiley-Liss, Inc.
  • (PMID = 16411206.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 7M7YKX2N15 / Topotecan; 8N3DW7272P / Cyclophosphamide
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12. Boehm R, Till H, Landes J, Schmid I, Joppich I: Ileoileal intussusception caused by a Ewing sarcoma tumour. An unusual case report. Eur J Pediatr Surg; 2003 Aug;13(4):272-5
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  • [Title] Ileoileal intussusception caused by a Ewing sarcoma tumour. An unusual case report.
  • The patient experienced increasing colics, recurrent vomiting, dehydration and weight loss.
  • Histopathology revealed a very rare, highly malignant mesenchymal Ewing sarcoma, infiltrating the complete bowel wall.
  • After the postoperative course, the patient was transferred to our oncological department for chemotherapy.
  • A Ewing sarcoma may be responsible for an intussusception.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Bone Neoplasms / therapy. Ileal Diseases. Ileal Neoplasms / therapy. Intussusception / surgery. Sarcoma, Ewing / therapy
  • [MeSH-minor] Adolescent. Cisplatin / administration & dosage. Combined Modality Therapy. Digestive System Surgical Procedures / methods. Doxorubicin / administration & dosage. Etoposide / administration & dosage. Humans. Ifosfamide / administration & dosage. Male. Stem Cell Transplantation. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 13680499.001).
  • [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] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; Q20Q21Q62J / Cisplatin; UM20QQM95Y / Ifosfamide
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13. Chow WA, Chu P, Chung V, Lawrence J, Garcia D, Doroshow JH: Imatinib mesylate therapy for recurrent Ewing's family of tumors (EFT). J Clin Oncol; 2004 Jul 15;22(14_suppl):9054

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Imatinib mesylate therapy for recurrent Ewing's family of tumors (EFT).
  • : 9054 Background: The prognosis for patients with recurrent Ewing's sarcoma (ES) and primitive neuroectodermal tumors (PNET) remains poor.
  • Novel therapies are urgently needed.
  • Based upon these findings, we treated a patient with recurrent EFT that expressed both c-Kit and PDGFR-α with imatinib mesylate.
  • Staging evaluation revealed bone and bone marrow involvement.
  • Chemotherapy with vincristine, doxorubicin and cyclophosphamide, altenating with ifosfamide and etoposide was initiated.
  • Progression was documented after 11 cycles of chemotherapy.
  • CONCLUSIONS: This case report demonstrates that imatinib mesylate was an effective alternative therapy for this patient with recurrent EFT whose tumor expressed the c-Kit and PDGFR-α TKRs.

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  • (PMID = 28014099.001).
  • [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. Juergens H, Ranft A, Paulussen M, Kontny U, Dilloo D, Dirksen U: Treosulfan-based high-dose chemotherapy with autologous stem cell transplantation in high-risk Ewing sarcoma. J Clin Oncol; 2009 May 20;27(15_suppl):10546

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treosulfan-based high-dose chemotherapy with autologous stem cell transplantation in high-risk Ewing sarcoma.
  • : 10546 Background: High-dose chemotherapy (HDC) is widely used in consolidation treatment of Ewing sarcoma patients with primary disseminated or recurrent disease.
  • Treosulfan is a newly-developed bifunctional alkylating agent that has been shown to be safe in current trials of the German Society for Pediatric Hematology and Oncology (GPOH) even in heavily pre-treated patients including axial radiation.
  • Relapse patients had received appropriate second line chemotherapy regimens prior to treosulfan-based HDC.
  • Metastatic sites were: bone (76%), bone marrow (22%), CNS (10%), liver (15%), lymphnode (29%), other (16%), and additionally lung metastases in 57%.
  • 1y-EFS was 0.70 (SE=.10), 2y-EFS was 0.27 (SE=0.10), and 3y-EFS was 0.16 (SE=.09).
  • 1y-OS was 0.84 (SE=.08), 2y-OS was 0.46 (SE=.13), and 3y-OS was 0.32 (SE=.12).
  • Survival after relapse at 3yrs was 0.70 (SE=.15).
  • CONCLUSIONS: Treosulfan-based HDC is an alternative approach in high-risk Ewing tumor patients especially after relapse to consolidate a second remission.

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  • (PMID = 27963953.001).
  • [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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15. Palmerini E, Brach Del Prever A, Fagioli F, Luksch R, Prete A, Tamburini A, Abate ME, Picci P, Ferrari S, Tienghi A: High-dose chemotherapy with autologous stem cell transplantation for relapsed Ewing's sarcoma. J Clin Oncol; 2009 May 20;27(15_suppl):10545

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] High-dose chemotherapy with autologous stem cell transplantation for relapsed Ewing's sarcoma.
  • : 10545 Background: Nearly 30-40% of patients with newly diagnosed, non-metastatic Ewing's Sarcoma (EWS) relapse.
  • The role of high-dose chemotherapy (HDCT) with autologous stem cell transplantation (ASCT) is under investigation in metastatic and high risk localized EWS patients.
  • METHODS: All non-metastatic EWS patients treated in Italian Sarcoma Group centers who relapsed between 1999 and June 2008 were offered HDCT (busulfan 4 mg/kg × 4 days orally and melphalan 140 mg/m<sup>2</sup>) with ASCT whenever possible (no previous HDCT; stable or responding disease after standard dose chemotherapy; adequate peripheral blood stem cells harvest).
  • Pattern of relapse was: lung metastases in 20 (28%) patients, bone metastases in 12 (16%), local recurrence in 11 (15%) and multiple sites in 29 (40%).
  • Treatment at 1<sup>st</sup> relapse was: standard dose chemotherapy in 31 (43%) patients; HDCT followed by ASCT in 24 (33%); palliative treatment in 12 (17%) and surgery only in 5 (7%).
  • Three patients died of treatment-related toxicity.
  • 3-year PRS was 33% (95%CI 13-54) for patients treated with HDCT and 22% (95%CI 6-39) for those receiving standard dose chemotherapy.
  • A significant (P 0.02) advantage was observed in the subgroup of patients with a shorter RFI treated with HDCT [3-year PRS 29% (95%CI 5-52)] compared to those treated with standard dose chemotherapy [3-years PRS 13%, (95%CI 2-29)].
  • The use of HDCT with ASCT in recurrent EWS is investigational.

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  • (PMID = 27963952.001).
  • [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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16. Hawkins D, Barnett T, Bensinger W, Gooley T, Sanders J: Busulfan, melphalan, and thiotepa with or without total marrow irradiation with hematopoietic stem cell rescue for poor-risk Ewing-Sarcoma-Family tumors. Med Pediatr Oncol; 2000 May;34(5):328-37
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  • [Title] Busulfan, melphalan, and thiotepa with or without total marrow irradiation with hematopoietic stem cell rescue for poor-risk Ewing-Sarcoma-Family tumors.
  • BACKGROUND: Survival following metastatic or recurrent Ewing sarcoma family tumors (ESFT) remains <25%.
  • Myeloablative therapy with hematopoietic stem cell transplantation (HSCT) may improve survival for poor-risk ESFT.
  • We describe the toxicity and efficacy of a myeloablative chemotherapy regimen, followed by a second myeloablative radiotherapy regimen as consolidation treatment for poor-risk ESFT.
  • PROCEDURE: Sixteen patients with poor-risk ESFT were treated with myeloablative therapy followed by HSCT.
  • All patients received busulfan, melphalan, and thiotepa (BuMelTT) as chemotherapy conditioning.
  • Nine patients received total marrow irradiation (TMI) as a second myeloablative therapy, also followed by HSCT.
  • Seven patients were excluded from TMI because of inadequate peripheral blood stem cell harvest, extensive prior radiation therapy, early disease progression, orpatient refusal.
  • The disease status prior to my eloablative therapy was first complete response (CR1) in three patients, CR2 in nine, second partial response (PR2) in one, CR3 in one, and progressive disease (PD) in two.
  • Eight developed recurrent disease (median time to progression 6.8 months).
  • CONCLUSIONS: Dual myeloablative therapy with BuMelTT and TMI was a feasible and promising treatment approach for patients with poor-risk ESFT.
  • Inability to collect sufficient PBSC and extensive previous radiation therapy limit the ability to deliver TMI as a second HSCT conditioning regimen.
  • [MeSH-major] Antineoplastic Agents, Alkylating / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Marrow / drug effects. Bone Marrow Purging / methods. Busulfan / administration & dosage. Hematopoietic Stem Cell Transplantation. Melphalan / administration & dosage. Sarcoma, Ewing / therapy. Thiotepa / administration & dosage
  • [MeSH-minor] Adolescent. Adult. Cause of Death. Child. Child, Preschool. Disease Progression. Disease-Free Survival. Feasibility Studies. Humans. Neoplasm Recurrence, Local. Prognosis. Remission Induction. Survival Rate. Treatment Outcome. Treatment Refusal

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  • [Copyright] Copyright 2000 Wiley-Liss, Inc.
  • (PMID = 10797354.001).
  • [ISSN] 0098-1532
  • [Journal-full-title] Medical and pediatric oncology
  • [ISO-abbreviation] Med. Pediatr. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA-15704; United States / NCI NIH HHS / CA / CA-18029; United States / NCI NIH HHS / CA / CA-47748; etc
  • [Publication-type] Clinical Trial; Clinical Trial, Phase I; Clinical Trial, Phase II; Journal Article; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 905Z5W3GKH / Thiotepa; G1LN9045DK / Busulfan; Q41OR9510P / Melphalan
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17. Desai KI, Nadkarni TD, Goel A, Muzumdar DP, Naresh KN, Nair CN: Primary Ewing's sarcoma of the cranium. Neurosurgery; 2000 Jan;46(1):62-8; discussion 68-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary Ewing's sarcoma of the cranium.
  • OBJECTIVE: We analyzed the data for a series of 14 patients with primary Ewing's sarcomas of the cranium who were treated since 1985.
  • Our aim was to assess the long-term outcomes and the selection of appropriate treatment methods.
  • METHODS: The patients were reviewed with respect to their clinical presentations, treatment, and outcomes.
  • Skeletal surveys with routine radiographs and technetium-99 bone scans to detect extracranial Ewing's sarcomas were performed for all patients.
  • All patients were then subjected to adjuvant multidrug chemotherapy and radiotherapy.
  • This recurrent tumor was completely excised, and additional chemotherapy was administered.
  • CONCLUSION: Although primary Ewing's sarcoma of the cranium is a malignant bone tumor, it is associated with a good prognosis when treated with radical surgery, aggressive multidrug chemotherapy, and radiotherapy.
  • [MeSH-major] Sarcoma, Ewing / therapy. Skull Neoplasms / therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Female. Humans. Infant. Male. Time Factors. Treatment Outcome

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  • (PMID = 10626936.001).
  • [ISSN] 0148-396X
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] UNITED STATES
  • [Number-of-references] 37
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18. Rodriguez-Galindo C, Billups CA, Kun LE, Rao BN, Pratt CB, Merchant TE, Santana VM, Pappo AS: Survival after recurrence of Ewing tumors: the St Jude Children's Research Hospital experience, 1979-1999. Cancer; 2002 Jan 15;94(2):561-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Survival after recurrence of Ewing tumors: the St Jude Children's Research Hospital experience, 1979-1999.
  • BACKGROUND: Despite improved therapies, 30-40% of patients with Ewing tumors (ET) experience recurrence and have a poor prognosis.
  • The authors analyzed factors prognostic of survival in patients with recurrent ET.
  • METHODS: The authors assessed the relation between postrecurrence survival (PRS) and demographic, disease, and treatment factors in 71 patients who experienced recurrent ET after treatment on one of three consecutive institutional protocols.
  • Patients who experience recurrence > or = 2 years after diagnosis and patients who have local recurrence that can be treated with radical surgery and intensive chemotherapy have the most favorable outcomes.
  • [MeSH-major] Bone Neoplasms / mortality. Neoplasm Recurrence, Local / mortality. Sarcoma, Ewing / mortality
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Female. Follow-Up Studies. Humans. Male. Prognosis. Radiotherapy. Retrospective Studies. Risk Factors. Salvage Therapy. Survival Rate. Treatment Outcome

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  • (PMID = 11900241.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Grant] United States / PHS HHS / / LA-23099; United States / NCI NIH HHS / CA / P30 CA 21765
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
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19. Anderson P, Kopp L, Anderson N, Cornelius K, Herzog C, Hughes D, Huh W: Novel bone cancer drugs: investigational agents and control paradigms for primary bone sarcomas (Ewing's sarcoma and osteosarcoma). Expert Opin Investig Drugs; 2008 Nov;17(11):1703-15
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Novel bone cancer drugs: investigational agents and control paradigms for primary bone sarcomas (Ewing's sarcoma and osteosarcoma).
  • BACKGROUND: New investigational agents and chemotherapy regimens including cyclophosphamide + topotecan, temozolomide + irinotecan, and anti-IGF-1R antibodies in Ewing's sarcoma (ES) and liposomal muramyltripeptide phosphatidylethanolamine (L-MTP-PE), aerosol therapy, and bone-specific agents in osteosarcoma (OS) may improve survival and/or quality of life on 'continuation' therapy.
  • OBJECTIVE: Review of investigational approaches and control paradigms for recurrent or metastatic primary bone tumors.
  • Review some current state-of-the-art approaches for OS including L-MTP-PE, anti-IGF-1R inhibition, aerosol therapies and bone specific agents.
  • RESULTS/CONCLUSION: L-MTP-PE with chemotherapy in OS has been shown to improve survival; compassionate access is available for recurrence and/or metastases.
  • Aerosol therapy (granulocyte-macrophage colony stimulating factor, cisplatin, gemcitabine) for lung metastases is a promising approach to reduce systemic toxicity.
  • The bone-specific agents including denosumab (anti-receptor activator of NF-kappaB ligand antibody) and bisphosphonates may have benefit against giant cell tumor, ES and OS.
  • Anti-IGF-1R antibody SCH717454 has preclinical activity in OS but best effectiveness will most likely be in combination with chemotherapy earlier in therapy.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Osteosarcoma / drug therapy. Sarcoma, Ewing / drug therapy
  • [MeSH-minor] Animals. Drug Evaluation, Preclinical. Humans. Immunotherapy. Neoplasm Metastasis / pathology


20. Rosenthal J, Bolotin E, Shakhnovits M, Pawlowska A, Falk P, Qian D, Oliver C, Sato J, Miser J, Forman S: High-dose therapy with hematopoietic stem cell rescue in patients with poor prognosis Ewing family tumors. Bone Marrow Transplant; 2008 Sep;42(5):311-8
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  • [Title] High-dose therapy with hematopoietic stem cell rescue in patients with poor prognosis Ewing family tumors.
  • The purpose of the study was to evaluate the feasibility and safety of two cycles of high-dose chemotherapy (HDT) followed by autologous hematopoietic SCT (HSCT) in patients with poor prognosis Ewing family of tumors (EFT).
  • Twenty patients with primary metastatic bulky disease or recurrent EFT were enrolled to a treatment protocol with two cycles of HDT and HSCT.
  • Dose intensification with two cycles of HDT and HSCT is feasible and safe, with low and acceptable treatment-related morbidity and mortality.
  • Adding a second course of therapy does not impair engraftment.
  • Further studies are required to define the optimal mode of delivery of HDT and HSCT in treatment of advanced EFT.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Bone Neoplasms / therapy. Hematopoietic Stem Cell Transplantation. Sarcoma, Ewing / therapy
  • [MeSH-minor] Adolescent. Adult. Child. Disease-Free Survival. Female. Graft Survival. Humans. Male. Survival Rate. Time Factors. Transplantation, Autologous

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  • (PMID = 18587438.001).
  • [ISSN] 0268-3369
  • [Journal-full-title] Bone marrow transplantation
  • [ISO-abbreviation] Bone Marrow Transplant.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] England
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21. McTiernan A, Driver D, Michelagnoli MP, Kilby AM, Whelan JS: High dose chemotherapy with bone marrow or peripheral stem cell rescue is an effective treatment option for patients with relapsed or progressive Ewing's sarcoma family of tumours. Ann Oncol; 2006 Aug;17(8):1301-5
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  • [Title] High dose chemotherapy with bone marrow or peripheral stem cell rescue is an effective treatment option for patients with relapsed or progressive Ewing's sarcoma family of tumours.
  • BACKGROUND: The outcome for patients with recurrent or progressive Ewing's sarcoma family of tumours (ESFT) is poor.
  • High dose therapy (HDT) has been used for a number of years in an attempt to improve survival; however, evidence for the efficacy of this treatment remains limited.
  • PATIENTS AND METHODS: Between 1992 and 2004, 33 patients with recurrent or progressive ESFT were treated with HDT with bone marrow (n=2), peripheral blood stem cell (n=30), or bone marrow and peripheral blood stem cell support (n=1), at a single institution.
  • There was one treatment related death from colitis, and grade 4 infection was observed in two patients.
  • CONCLUSIONS: Long-term survival can be attained in patients with recurrent or refractory ESFT treated with HDT.
  • However, this treatment is associated with severe toxicity.

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  • (PMID = 16782749.001).
  • [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] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; G1LN9045DK / Busulfan; Q41OR9510P / Melphalan
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22. Subbiah V, Anderson P, Lazar AJ, Burdett E, Raymond K, Ludwig JA: Ewing's sarcoma: standard and experimental treatment options. Curr Treat Options Oncol; 2009 Apr;10(1-2):126-40
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  • [Title] Ewing's sarcoma: standard and experimental treatment options.
  • OPINION STATEMENT: Ewing sarcoma family tumors (EWS), which include classic Ewing's sarcoma in addition to primitive neuroectodermal tumor and Askin tumor, are the second most common variety of primary bone cancer to afflict adolescents and young adults.
  • Multi-disciplinary care incorporating advances in diagnosis, surgery, chemotherapy, and radiation has substantially improved the survival rate of patients with localized Ewing sarcoma to nearly 70%.
  • Unfortunately, those advances have not significantly changed the long-term outcome for those with metastatic or recurrent disease; 5-year survival remains less than 25%.
  • This apparent therapeutic plateau exists despite extensive effort during the last four decades to optimize the efficacy of cytotoxic chemotherapy through combination of chemotherapies of mechanistically diverse action, dose-dense scheduling (provided as frequently as every 2 weeks), increased adjuvant treatment duration, and higher dosage per cycle (facilitated with parallel strides in supportive care incorporating growth factors).
  • As has already occurred for malignancies such as breast or colon cancer, the "-omics-based" revolution has enhanced our understanding of the molecular changes responsible for Ewing's tumor formation and identified a number of potential targets (such as IGF-1R or mTOR) amenable to biological therapy.
  • It has also created both a challenge and an opportunity to develop predictive biomarkers capable of selecting patients most likely to benefit from targeted therapy.
  • In this review, we discuss current standard-of-care for patients with Ewing's sarcoma and highlight the most promising experimental therapies in early-phase clinical trials.
  • [MeSH-major] Bone Neoplasms / surgery. Sarcoma, Ewing / surgery. Therapies, Investigational
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Agents / pharmacology. Antineoplastic Agents / therapeutic use. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Clinical Trials as Topic. Combined Modality Therapy. Drug Delivery Systems. Drug Screening Assays, Antitumor. Humans. Lung Neoplasms / drug therapy. Lung Neoplasms / mortality. Lung Neoplasms / secondary. Lung Neoplasms / surgery. Multicenter Studies as Topic. Oncogene Proteins, Fusion / antagonists & inhibitors. Oncogene Proteins, Fusion / genetics. Proto-Oncogene Protein c-fli-1. RNA-Binding Protein EWS. Receptor, IGF Type 1 / antagonists & inhibitors. Survival Rate. Transcription Factors / antagonists & inhibitors. Translocation, Genetic. Young Adult

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  • (PMID = 19533369.001).
  • [ISSN] 1534-6277
  • [Journal-full-title] Current treatment options in oncology
  • [ISO-abbreviation] Curr Treat Options Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / EWS-FLI fusion protein; 0 / Oncogene Proteins, Fusion; 0 / Proto-Oncogene Protein c-fli-1; 0 / RNA-Binding Protein EWS; 0 / Transcription Factors; EC 2.7.10.1 / Receptor, IGF Type 1
  • [Number-of-references] 94
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23. Navid F, Willert JR, McCarville MB, Furman W, Watkins A, Roberts W, Daw NC: Combination of gemcitabine and docetaxel in the treatment of children and young adults with refractory bone sarcoma. Cancer; 2008 Jul 15;113(2):419-25
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  • [Title] Combination of gemcitabine and docetaxel in the treatment of children and young adults with refractory bone sarcoma.
  • METHODS: A retrospective case review of 22 patients with recurrent or refractory bone or soft-tissue sarcomas who received gemcitabine (at a dose of 675 mg/m(2) intravenously on Days 1 and 8) and docetaxel (at a dose of 75-100 mg/m(2) intravenously on Day 8) was undertaken.
  • RESULTS: The patients (ages 8-23 years) received a total of 109 courses of chemotherapy (median, 4 courses; range, 1-13 courses).
  • Seventeen patients had osteosarcoma, 2 patients had Ewing sarcoma family of tumors (ESFT), 1 patient had a malignant fibrous histiocytoma (MFH), 1 patient had a chondrosarcoma, and 1 patient had an undifferentiated sarcoma.
  • CONCLUSIONS: In the current study, gemcitabine in combination with docetaxel was found to be well tolerated and demonstrated antitumor activity in children and adolescents with recurrent or refractory osteosarcoma and MFH.
  • Further evaluation of this drug combination is warranted in these patients.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Deoxycytidine / analogs & derivatives. Osteosarcoma / drug therapy. Taxoids / therapeutic use
  • [MeSH-minor] Adolescent. Adult. Child. Dose-Response Relationship, Drug. Female. Humans. Male. Tomography, X-Ray Computed. Treatment Outcome

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  • [Copyright] (c) 2008 American Cancer Society.
  • (PMID = 18484657.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA21765; United States / NCI NIH HHS / CA / CA23099
  • [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 / Taxoids; 0W860991D6 / Deoxycytidine; 15H5577CQD / docetaxel; B76N6SBZ8R / gemcitabine
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24. Bar-Sever Z, Cohen IJ, Connolly LP, Horev G, Perri T, Treves T, Hardoff R: Tc-99m MIBI to evaluate children with Ewing's sarcoma. Clin Nucl Med; 2000 Jun;25(6):410-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Tc-99m MIBI to evaluate children with Ewing's sarcoma.
  • PURPOSE: Tc-99m MIBI has been used increasingly to evaluate benign and malignant tumors because of its tumor-seeking properties and ability to provide an imaging assessment of multiple-drug resistance.
  • This study investigated the clinical utility of Tc-99m MIBI in the management of Ewing's sarcoma in children.
  • METHODS: Thirteen Tc-99m MIBI studies in nine (six male, three female) patients ages 6.5 to 20 years (mean, 13.4 years) with Ewing's sarcoma were reviewed.
  • All patients had imaging studies at diagnosis, and four had follow-up studies during or after therapy.
  • RESULTS: Tc-99m MIBI accumulated in 6 of 9 primary tumors and did not accumulate in one recurrent tumor.
  • The presence or absence of Tc-99m MIBI uptake at diagnosis or after therapy carried no prognostic significance.
  • CONCLUSION: Tc-99m MIBI imaging does not appear to be useful in Ewing's sarcoma.
  • [MeSH-major] Bone Neoplasms / radionuclide imaging. Radiopharmaceuticals. Sarcoma, Ewing / radionuclide imaging. Technetium Tc 99m Sestamibi
  • [MeSH-minor] Adolescent. Adult. Child. Drug Resistance, Multiple. Drug Resistance, Neoplasm. Female. Femoral Neoplasms / radionuclide imaging. Femoral Neoplasms / therapy. Follow-Up Studies. Humans. Lung Neoplasms / radionuclide imaging. Lung Neoplasms / secondary. Male. Neoplasm Recurrence, Local / radionuclide imaging. P-Glycoprotein / analysis. Pelvic Bones / radionuclide imaging. Prognosis. Retrospective Studies

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  • (PMID = 10836685.001).
  • [ISSN] 0363-9762
  • [Journal-full-title] Clinical nuclear medicine
  • [ISO-abbreviation] Clin Nucl Med
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / P-Glycoprotein; 0 / Radiopharmaceuticals; 971Z4W1S09 / Technetium Tc 99m Sestamibi
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25. Kołłataj B, Zajaczkowska M, Katski K, Sikora P, Pijanowska M, Majewski M, Kołłataj W: [Acquired Fanconi-de Toni-Debre syndrome due to therapy for Ewing's sarcoma in 5-years old boy]. Przegl Lek; 2006;63 Suppl 3:220-2
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Acquired Fanconi-de Toni-Debre syndrome due to therapy for Ewing's sarcoma in 5-years old boy].
  • We present a 5-years old boy with acquired Fanconi-de Toni-Debre syndrome being a effect of therapy for Ewing's sarcoma.
  • At the age of 3 years, this boy was diagnosed as suffering from Ewing sarcoma of his right femur.
  • The boy received a course of 8-month pre-surgery (6 VIDE--Vincristine, Ifosfamide, Doxorubicin, Etoposide cycles and 2 VAI--Vincristine, Actinomycin, Ifosfamide cycles) and 6-month post-surgery (6 VAI--Vincristine, Actinomycin, Ifosfamide cycles) cytostatic therapies according to EWING, EURO 99 protocol.
  • In forth month of post-surgery cytostatic therapy, progressive malaise, polyuria, polydypsia, and recurrent vomiting occurred.
  • Acquired Fanconi-de Toni-Debre syndrome due to toxic effect of cytostatic therapy on renal proximal tubules was diagnosed.
  • At present, two years after the time the diagnosis was made, despite constant substitution of potassium, phosphates and bicarbonates, deficit of body mass and height, and bone mineral density abnormalities are observed.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / adverse effects. Bone Neoplasms / drug therapy. Fanconi Syndrome / chemically induced. Femur / surgery. Sarcoma, Ewing / drug therapy

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  • (PMID = 16898536.001).
  • [ISSN] 0033-2240
  • [Journal-full-title] Przegla̧d lekarski
  • [ISO-abbreviation] Prz. Lek.
  • [Language] pol
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Poland
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; UM20QQM95Y / Ifosfamide
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26. Rutigliano DN, Meyers P, Ghossein RA, Carlson DL, Kayton ML, Kraus D, La Quaglia MP: Mucoepidermoid carcinoma as a secondary malignancy in pediatric sarcoma. J Pediatr Surg; 2007 Jul;42(7):E9-13
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  • [Title] Mucoepidermoid carcinoma as a secondary malignancy in pediatric sarcoma.
  • PURPOSE: Children diagnosed with osteosarcoma (OS) and Ewing sarcoma (ES) have greatly benefited from the addition of alkylator therapy.
  • Herein we report on 2 patients with sarcoma who developed a case of secondary mucoepidermoid carcinoma after chemotherapy treatment without associated radiation therapy.
  • To our knowledge, this is the first series of mucoepidermoid carcinomas arising in pediatric patients treated for sarcoma without radiotherapy.
  • Details of their initial evaluation, previous therapies, resection techniques, pathologic findings, and follow-up compose this report.
  • Both patients underwent primary resection and chemotherapy including alkylating agents, but neither received radiation.
  • The mucoepidermoid carcinomas developed 27 months and 132 months after completion of therapy, respectively, and were noted on routine yearly follow-up.
  • One patient is alive, without evidence of recurrent mucoepidermoid carcinoma after 4 years; the other recently completed radiotherapy and is disease-free after 12 months.
  • This is the first reported series of parotid mucoepidermoid carcinomas occurring after sarcoma treatment without radiotherapy.
  • A common link between the 2 patients may be the use of alkylating therapy.
  • [MeSH-major] Bone Neoplasms / pathology. Carcinoma, Mucoepidermoid / pathology. Neoplasms, Second Primary / pathology. Osteosarcoma / pathology. Parotid Neoplasms / pathology. Sarcoma, Ewing / pathology
  • [MeSH-minor] Adolescent. Combined Modality Therapy. Female. Humans. Male

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  • (PMID = 17618873.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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27. Mutafoğlu Uysal K, Olgun N, Sarialioğlu F, Kargi A, Cevik N: A case with extraosseous Ewing's sarcoma: a late effect related to bone marrow transplantation for thalassemia or a component of a familial cancer syndrome? Pediatr Hematol Oncol; 2000 Jul-Aug;17(5):415-9
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  • [Title] A case with extraosseous Ewing's sarcoma: a late effect related to bone marrow transplantation for thalassemia or a component of a familial cancer syndrome?
  • Allogeneic bone marrow transplantation has proved to be a radical form of cure in patients with beta-thalassemia major who have a human leukocyte antigen identical donor.
  • Although malignant neoplasms are serious late complications of bone marrow transplantation, very few reports describing the development of malignant tumors after allografting for thalassemia appeared in the literature.
  • A case is presented here of extraosseous Ewing's sarcoma that developed 8 years after allogeneic bone marrow transplantation performed for beta-thalassemia major.
  • The patient was treated with chemotherapy and radiotherapy and died with recurrent disease.
  • To the authors' knowledge, this is the first case of extraosseous Ewing's sarcoma after bone marrow transplantation for thalassemia.
  • The possible contribution of transplantation procedure and the genetic factors as well as the primary genetic hemoglobinopathy to the development of this malignant tumor are discussed.
  • [MeSH-major] Sarcoma, Ewing / etiology. Soft Tissue Neoplasms / etiology
  • [MeSH-minor] Adult. Bone Marrow Transplantation / adverse effects. Child. Family Health. Female. Genetic Predisposition to Disease. Humans. Male. Neoplastic Cells, Circulating. Pedigree. Transplantation, Homologous / adverse effects. beta-Thalassemia / complications. beta-Thalassemia / therapy

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  • (PMID = 10914053.001).
  • [ISSN] 0888-0018
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] ENGLAND
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28. Chao J, Budd GT, Chu P, Frankel P, Garcia D, Junqueira M, Loera S, Somlo G, Sato J, Chow WA: Phase II clinical trial of imatinib mesylate in therapy of KIT and/or PDGFRalpha-expressing Ewing sarcoma family of tumors and desmoplastic small round cell tumors. Anticancer Res; 2010 Feb;30(2):547-52
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  • [Title] Phase II clinical trial of imatinib mesylate in therapy of KIT and/or PDGFRalpha-expressing Ewing sarcoma family of tumors and desmoplastic small round cell tumors.
  • We conducted a Phase II trial to evaluate the effectiveness of imatinib for patients with recurrent ESFT or DSRCT expressing KIT and/or PDGFRalpha.
  • Two patients were not evaluable due to one early death and one refusing treatment.
  • Given the poor prognosis with recurrent ESFT, further studies with other novel KIT and PDGFRalpha inhibitors are needed.
  • [MeSH-major] Abdominal Neoplasms / drug therapy. Bone Neoplasms / drug therapy. Carcinoma, Small Cell / drug therapy. Piperazines / therapeutic use. Proto-Oncogene Proteins c-kit / metabolism. Pyrimidines / therapeutic use. Receptor, Platelet-Derived Growth Factor beta / metabolism. Sarcoma, Ewing / drug therapy
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Antineoplastic Agents / therapeutic use. Benzamides. Female. Humans. Imatinib Mesylate. Male. Middle Aged. Neuroectodermal Tumors, Primitive / drug therapy. Neuroectodermal Tumors, Primitive / metabolism. Neuroectodermal Tumors, Primitive / pathology. Survival Rate. Treatment Outcome

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  • (PMID = 20332468.001).
  • [ISSN] 1791-7530
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit; EC 2.7.10.1 / Receptor, Platelet-Derived Growth Factor beta
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29. Drabko K, Zawitkowska-Klaczynska J, Wojcik B, Choma M, Zaucha-Prazmo A, Kowalczyk J, Gorczynska E, Toporski J, Kałwak K, Turkiewicz D, Chybicka A: Megachemotherapy followed by autologous stem cell transplantation in children with Ewing's sarcoma. Pediatr Transplant; 2005 Oct;9(5):618-21
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  • [Title] Megachemotherapy followed by autologous stem cell transplantation in children with Ewing's sarcoma.
  • Twenty-one children with high-risk Ewing's tumor received high-dose chemotherapy with a PBSCT.
  • Aim of the study was evaluation of efficiency and safety of this procedure.
  • Megachemotherapy consisted of melphalan 140 mg/m2/busulfan 16 mg/kg in 12 patients, melphalan 140 mg2/treosulfan 10.0 g/m2 in two patients and melphalan with other drugs in seven patients.
  • Children transplanted without remission died: Two of them due to transplant related causes and eight had progression of disease in a median time 7 month after PBSCT.
  • Megachemotherapy with PBSCT is a safe procedure in children with Ewing's sarcoma in remission.
  • Autologos transplantation in children with metastatic Ewing's sarcoma seems to improve their outcome.
  • Patients with Ewing's sarcoma, resistant to conventional therapy and with recurrent disease did not benefit from megachemotherapy.
  • New approaches such as anti-tumor vaccination or using of imatinib are reasonable to introduce in patients with relapsed or resistant to therapy Ewing's tumor.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / therapy. Hematopoietic Stem Cell Transplantation. Sarcoma, Ewing / therapy
  • [MeSH-minor] Adolescent. Antineoplastic Agents, Alkylating / administration & dosage. Antineoplastic Agents, Alkylating / adverse effects. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Humans. Male. Melphalan / administration & dosage. Melphalan / adverse effects. Survival Rate. Transplantation, Autologous

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  • (PMID = 16176419.001).
  • [ISSN] 1397-3142
  • [Journal-full-title] Pediatric transplantation
  • [ISO-abbreviation] Pediatr Transplant
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; Q41OR9510P / Melphalan
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30. Saylors RL 3rd, Stine KC, Sullivan J, Kepner JL, Wall DA, Bernstein ML, Harris MB, Hayashi R, Vietti TJ, Pediatric Oncology Group: Cyclophosphamide plus topotecan in children with recurrent or refractory solid tumors: a Pediatric Oncology Group phase II study. J Clin Oncol; 2001 Aug 01;19(15):3463-9
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  • [Title] Cyclophosphamide plus topotecan in children with recurrent or refractory solid tumors: a Pediatric Oncology Group phase II study.
  • PURPOSE: To determine the response rate of the combination of cyclophosphamide and topotecan in pediatric patients with recurrent or refractory malignant solid tumors.
  • All patients received filgrastim (5 mcg/kg) daily until the absolute neutrophil count (ANC) was > or = 1,500 microL after the time of the expected ANC nadir.
  • RESULTS: A total of 307 treatment courses were given to the 83 fully assessable patients.
  • Responses (complete response plus partial response) were seen in rhabdomyosarcoma (10 of 15 patients), Ewing's sarcoma (six of 17 patients), and neuroblastoma (six of 13 patients).
  • CONCLUSION: The combination of cyclophosphamide and topotecan is active in rhabdomyosarcoma, neuroblastoma, and Ewing's sarcoma.
  • The therapy can be given with acceptable hematopoietic toxicity with the use of filgrastim support.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Neoplasm Recurrence, Local / drug therapy. Neoplasms / drug therapy
  • [MeSH-minor] Adolescent. Adult. Bone Neoplasms / drug therapy. Child. Child, Preschool. Cyclophosphamide / administration & dosage. Female. Humans. Infant. Infusions, Intravenous. Male. Neuroblastoma / drug therapy. Osteosarcoma / drug therapy. Rhabdomyosarcoma / drug therapy. Sarcoma, Ewing / drug therapy. Topotecan / administration & dosage

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  • (PMID = 11481351.001).
  • [ISSN] 0732-183X
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA 05587; United States / NCI NIH HHS / CA / CA 07431; United States / NCI NIH HHS / CA / CA 11233; United States / NCI NIH HHS / CA / CA 15089; United States / NCI NIH HHS / CA / CA 20549; United States / NCI NIH HHS / CA / CA 25408; United States / NCI NIH HHS / CA / CA 28476; United States / NCI NIH HHS / CA / CA 29139; United States / NCI NIH HHS / CA / CA 29293; United States / NCI NIH HHS / CA / CA 29691; United States / NCI NIH HHS / CA / CA 30969; United States / NCI NIH HHS / CA / CA 32053; United States / NCI NIH HHS / CA / CA 33603; United States / NCI NIH HHS / CA / CA 35587; United States / NCI NIH HHS / CA / CA 53128; United States / NCI NIH HHS / CA / CA 69428
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Journal Article; Multicenter Study; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 7M7YKX2N15 / Topotecan; 8N3DW7272P / Cyclophosphamide
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31. Maki RG, Kraft AS, Scheu K, Yamada J, Wadler S, Antonescu CR, Wright JJ, Schwartz GK: A multicenter Phase II study of bortezomib in recurrent or metastatic sarcomas. Cancer; 2005 Apr 1;103(7):1431-8
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  • [Title] A multicenter Phase II study of bortezomib in recurrent or metastatic sarcomas.
  • Arm A included patients with osteogenic sarcoma, Ewing sarcoma, and rhabdomyosarcoma.
  • Arm B accrued patients with other types of soft tissue sarcomas.
  • Patients were not allowed to have received previous chemotherapy for metastatic disease.
  • CONCLUSIONS: Bortezomib has minimal activity in soft tissue sarcoma as a single agent.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Bone Neoplasms / drug therapy. Boronic Acids / therapeutic use. Proteasome Inhibitors. Pyrazines / therapeutic use. Sarcoma / drug therapy. Soft Tissue Neoplasms / drug therapy
  • [MeSH-minor] Adult. Aged. Bortezomib. Drug Administration Schedule. Fatigue / chemically induced. Female. Humans. Male. Middle Aged. Neoplasm Metastasis / drug therapy. Neoplasm Recurrence, Local / drug therapy. Nervous System Diseases / chemically induced. Treatment Outcome

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  • [Copyright] Copyright 2005 American Cancer Society.
  • (PMID = 15739208.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CM / N01-CM17105; United States / NCI NIH HHS / CA / P01-CA47179
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Boronic Acids; 0 / Proteasome Inhibitors; 0 / Pyrazines; 69G8BD63PP / Bortezomib
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32. Niculescu M, Negrusoiu M: Deep recurrent infection of the hip after tumoral resection in an 18-years old male--a case report. J Med Life; 2008 Oct-Dec;1(4):439-42
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  • [Title] Deep recurrent infection of the hip after tumoral resection in an 18-years old male--a case report.
  • In 2002 he was diagnosed with Ewing sarcoma of the proximal two-thirds of his left femur.
  • Eight months after surgery a deep infection of the hip developed, and despite antibiotic treatment and two consecutive debridments and lavage the results were negative.
  • [MeSH-major] Arthroplasty, Replacement, Hip / methods. Bone Neoplasms / surgery. Femur / surgery. Rifampin / administration & dosage. Sarcoma, Ewing / surgery. Staphylococcal Infections / drug therapy. Surgical Wound Infection / surgery. Teicoplanin / administration & dosage
  • [MeSH-minor] Adolescent. Humans. Male. Prosthesis Failure. Recurrence. Sepsis / drug therapy

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  • (PMID = 20108525.001).
  • [ISSN] 1844-122X
  • [Journal-full-title] Journal of medicine and life
  • [ISO-abbreviation] J Med Life
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Romania
  • [Chemical-registry-number] 61036-62-2 / Teicoplanin; VJT6J7R4TR / Rifampin
  • [Other-IDs] NLM/ PMC3018973
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33. Somers GR, Shago M, Zielenska M, Chan HS, Ngan BY: Primary subcutaneous primitive neuroectodermal tumor with aggressive behavior and an unusual karyotype: case report. Pediatr Dev Pathol; 2004 Sep-Oct;7(5):538-45
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  • Primitive neuroectodermal tumor/Ewing sarcoma (PNET/ES) rarely occurs in the skin and subcutaneous tissues.
  • Despite wide local excision and chemotherapy, she rapidly developed cranial bone and brain metastases, followed by lung and skeletal metastases, and died shortly thereafter.
  • The recurrent tumor exhibited light microscopic features of a small, round, blue cell tumor with intracytoplasmic glycogen.
  • Cytogenetic analysis of the relapsed tumor showed a complex karyotype: 47,XX,i(1)(q10), der(4)t(4;19) (q33 approximately q35;q13.1), + 8,t(15;17)(q24;p11.2 approximately p12),der(19)t (19;20)(q13.1;p11.2),der(22)t(20;22)(q13;q13).
  • [MeSH-major] Neuroectodermal Tumors, Primitive, Peripheral / secondary. Sarcoma, Ewing / pathology. Soft Tissue Neoplasms / pathology. Subcutaneous Tissue / pathology
  • [MeSH-minor] Adolescent. Biomarkers, Tumor / analysis. Bone Neoplasms / secondary. Brain Neoplasms / metabolism. Brain Neoplasms / secondary. Chromosome Aberrations. Fatal Outcome. Female. Humans. Immunohistochemistry. Lung Neoplasms / secondary. Microscopy, Electron, Transmission. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 15547779.001).
  • [ISSN] 1093-5266
  • [Journal-full-title] Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
  • [ISO-abbreviation] Pediatr. Dev. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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34. Higman MA, Port JD, Beauchamp NJ Jr, Chen AR: Reversible leukoencephalopathy associated with re-infusion of DMSO preserved stem cells. Bone Marrow Transplant; 2000 Oct;26(7):797-800
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  • We report a case of posterior reversible leuko- encephalopathy (PRL) following the infusion of dimethylsulfoxide (DMSO) cryopreserved autologous stem cells in the setting of myeloablative chemotherapy in a patient with recurrent Ewing's sarcoma.
  • Bone Marrow Transplantation (2000) 26, 797-800.
  • [MeSH-minor] Adolescent. Female. Humans. Magnetic Resonance Imaging. Sarcoma, Ewing / therapy. Transplantation, Autologous

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  • (PMID = 11042664.001).
  • [ISSN] 0268-3369
  • [Journal-full-title] Bone marrow transplantation
  • [ISO-abbreviation] Bone Marrow Transplant.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] ENGLAND
  • [Chemical-registry-number] YOW8V9698H / Dimethyl Sulfoxide
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35. Bond M, Bernstein ML, Pappo A, Schultz KR, Krailo M, Blaney SM, Adamson PC: A phase II study of imatinib mesylate in children with refractory or relapsed solid tumors: a Children's Oncology Group study. Pediatr Blood Cancer; 2008 Feb;50(2):254-8
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  • [Title] A phase II study of imatinib mesylate in children with refractory or relapsed solid tumors: a Children's Oncology Group study.
  • Ewing sarcoma, osteosarcoma, neuroblastoma, desmoplastic small round cell, and synovial sarcomas often overexpress KIT or the PDGF receptor.
  • PROCEDURE: Patients less than 30 years of age with refractory or recurrent Ewing sarcoma, osteosarcoma, neuroblastoma, desmoplastic small round cell, synovial sarcomas or GIST were eligible.
  • Only one partial response was seen among 24 patients with Ewing sarcoma.
  • Hemorrhagic pleural effusions occurred in seven patients with pulmonary lesions, four of whom had progressive disease at the time of the hemorrhage.
  • CONCLUSION: Imatinib as a single agent at a dose of 440 mg/m(2)/day demonstrated little or no activity as a single agent in children with relapsed or refractory Ewing sarcoma, osteosarcoma, neuroblastoma, or desmoplastic small round cell tumors.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Neoplasm Recurrence, Local / drug therapy. Neoplasms / drug therapy. Piperazines / therapeutic use. Pyrimidines / therapeutic use
  • [MeSH-minor] Adolescent. Adult. Benzamides. Bone Neoplasms / drug therapy. Carcinoma, Small Cell / drug therapy. Child. Child, Preschool. Female. Gastrointestinal Stromal Tumors / drug therapy. Humans. Imatinib Mesylate. Male. Neuroblastoma / drug therapy. Osteosarcoma / drug therapy. Sarcoma, Ewing / drug therapy. Sarcoma, Synovial / drug therapy

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17262795.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / U10 CA 98543
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate
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36. Nemec SF, Krestan CR, Hojreh A, Hörmann M: [Radiological diagnostics of malignant tumors of the musculoskeletal system in childhood and adolescence]. Radiologe; 2008 Oct;48(10):962-8
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  • [Transliterated title] Radiologische Diagnostik maligner Tumoren des Muskuloskelettalsystems im Kindes- und Adoleszentenalter.
  • Rhabdomyosarcoma, osteosarcoma and Ewing's sarcoma are the most common malignant tumors of the musculoskeletal system in childhood and adolescence representing about 10% of newly diagnosed cancers in children and adolescents.In the last two decades the prognosis of patients with such malignancies improved significantly.
  • On the one hand because of the advances in chemotherapy and orthopedic surgery, on the other hand also because of the innovations in radiological diagnostics.
  • The precise pre-therapeutical staging of tumors of the musculoskeletal system provides important prognostic information and has impact on the entire therapy management.
  • During respectively after therapy, imaging is extremely important in the follow-up and in diagnosing a possible recurrent disease.Modern imaging diagnostics of musculoskeletal tumors basically consist of conventional X-ray, of computed tomography (CT) and magnetic resonance imaging (MRI), and of modalities of nuclear medicine such as szintigraphy, positron emission tomography (PET) and PET CT.
  • [MeSH-major] Bone Neoplasms / diagnostic imaging. Muscle Neoplasms / diagnostic imaging. Osteosarcoma / diagnostic imaging. Prostatic Neoplasms / diagnosis. Rhabdomyosarcoma / diagnostic imaging. Sarcoma, Ewing / diagnostic imaging. Tomography, X-Ray Computed / methods

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  • (PMID = 18461298.001).
  • [ISSN] 0033-832X
  • [Journal-full-title] Der Radiologe
  • [ISO-abbreviation] Radiologe
  • [Language] ger
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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37. Wasilewski-Masker K, Liu Q, Yasui Y, Leisenring W, Meacham LR, Hammond S, Meadows AT, Robison LL, Mertens AC: Late recurrence in pediatric cancer: a report from the Childhood Cancer Survivor Study. J Natl Cancer Inst; 2009 Dec 16;101(24):1709-20
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  • RESULTS: Overall, 5-year survivors of pediatric cancers experienced a cumulative incidence of recurrent disease of 4.4%, 5.6%, and 6.2% at 10, 15, and 20 years, respectively.
  • Cumulative incidence varied by diagnosis: Survivors of Ewing sarcoma and astrocytoma had the highest 20-year cumulative incidences at 13.0% (95% confidence interval [CI] = 9.4 to 16.5) and 14.4% (95% CI = 12.3 to 16.6), respectively.
  • In multivariable analysis, the greatest risk factors for late recurrence included diagnosis, combination treatment with chemotherapy and radiation, earlier treatment era, and fewer years since diagnosis (P < .001 for all).

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  • (PMID = 19966206.001).
  • [ISSN] 1460-2105
  • [Journal-full-title] Journal of the National Cancer Institute
  • [ISO-abbreviation] J. Natl. Cancer Inst.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / U24-CA 55727
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC2800799
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38. DeLaney TF, Liebsch NJ, Pedlow FX, Adams J, Dean S, Yeap BY, McManus P, Rosenberg AE, Nielsen GP, Harmon DC, Spiro IJ, Raskin KA, Suit HD, Yoon SS, Hornicek FJ: Phase II study of high-dose photon/proton radiotherapy in the management of spine sarcomas. Int J Radiat Oncol Biol Phys; 2009 Jul 1;74(3):732-9
NCI CPTC Antibody Characterization Program. NCI CPTC Antibody Characterization Program .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Negative surgical margins are uncommon; hence, doses of >or=66 Gy are recommended.
  • Treatment included pre- and/or postoperative photon/proton XRT with or without radical resection; patients with osteosarcoma and Ewing's sarcoma received chemotherapy.
  • Shrinking fields delivered 50.4 cobalt Gray equivalent (Gy RBE) to subclinical disease, 70.2 Gy RBE to microscopic disease in the tumor bed, and 77.4 Gy RBE to gross disease at 1.8 Gy RBE qd.
  • Spinal cord dose was limited to 63/54 Gy RBE to surface/center.
  • Intraoperative boost doses of 7.5 to 10 Gy could be given by dural plaque.
  • Two of 36 (5.6%) patients treated for primary versus 7/14 (50%) for recurrent tumor developed local recurrence (p < 0.001).
  • Five patients developed late radiation-associated complications; no myelopathy developed but three sacral neuropathies appeared after 77.12 to 77.4 Gy RBE.
  • CONCLUSIONS: Local control with this treatment is high in patients radiated at the time of primary presentation.
  • Sacral nerves receiving 77.12-77.4 Gy RBE are at risk for late toxicity.

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  • (PMID = 19095372.001).
  • [ISSN] 1879-355X
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P01 CA021239; United States / NCI NIH HHS / CA / CA021239-27; United States / NCI NIH HHS / CA / CA021239-28; United States / NCI NIH HHS / CA / P01CA021239; United States / NCI NIH HHS / CA / P01 CA021239-27; United States / NCI NIH HHS / CA / P01 CA021239-28
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Protons
  • [Other-IDs] NLM/ NIHMS121640; NLM/ PMC2734911
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39. Ruiz Tovar J, Reguero Callejas ME, Arano Bermejo JI, Capote Armas LF, González-Palacios Martínez F, Cabañas Navarro L: Malignant mixed Mullerian tumors. Clin Transl Oncol; 2006 Feb;8(2):129-32
Hazardous Substances Data Bank. CYCLOPHOSPHAMIDE .

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  • Treatment in 2 patients was hysterectomy with double ooforectomy, and resection of the pelvic mass was the treatment in the third case.
  • Adjuvant radio chemotherapy was administrated in 2 of the 3 cases.
  • Follow-up revealed recurrent pelvic tumour in 1 patient at 59 months, and breast metastases at 20 months in the second one.
  • [MeSH-minor] Adult. Aged. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / secondary. Breast Neoplasms / secondary. Chemotherapy, Adjuvant. Cisplatin / administration & dosage. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Fatal Outcome. Female. Femoral Neoplasms / secondary. Humans. Hysterectomy. Ifosfamide / administration & dosage. Ilium. Middle Aged. Neoplasms, Second Primary. Ovariectomy. Paclitaxel / administration & dosage. Palliative Care. Pelvic Neoplasms / secondary. Pelvic Neoplasms / surgery. Prognosis. Radiotherapy, Adjuvant. Sarcoma, Ewing. Spinal Neoplasms / secondary

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  • (PMID = 16632428.001).
  • [ISSN] 1699-048X
  • [Journal-full-title] Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico
  • [ISO-abbreviation] Clin Transl Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 8N3DW7272P / Cyclophosphamide; P88XT4IS4D / Paclitaxel; Q20Q21Q62J / Cisplatin; UM20QQM95Y / Ifosfamide
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40. Bomgaars L, Kerr J, Berg S, Kuttesch J, Klenke R, Blaney SM: A phase I study of irinotecan administered on a weekly schedule in pediatric patients. Pediatr Blood Cancer; 2006 Jan;46(1):50-5
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  • BACKGROUND: The objectives of this study were to determine the maximum tolerated dose (MTD), dose-limiting toxicities (DLTs), pharmacokinetics, and anti-tumor effect of irinotecan in pediatric patients with recurrent or refractory malignancies.
  • PROCEDURE: Twenty-three patients between 1 and 21 years of age, with a solid tumor refractory to standard therapy or for which there was no standard therapy were enrolled.
  • A MTD was defined in heavily-pretreated and less-heavily-pretreated (< or =2 prior chemotherapy regimens, no prior bone marrow transplantation, and no central axis radiation) patients.
  • Five patients had stable disease for two to four cycles including one patient each with rhabdomyosarcoma, Ewing sarcoma, neuroblastoma, and two patients with ependymoma.
  • [MeSH-major] Antineoplastic Agents, Phytogenic / pharmacology. Camptothecin / analogs & derivatives. Neoplasms / drug therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Drug Administration Schedule. Female. Humans. Male. Maximum Tolerated Dose

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  • (PMID = 15768380.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United States / NCRR NIH HHS / RR / M01 RR00188-37
  • [Publication-type] Clinical Trial, Phase I; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Phytogenic; 7673326042 / irinotecan; XT3Z54Z28A / Camptothecin
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41. Siddiqui T, Marsh Rde W, Allegra C, Whittaker D, Scarborough M, Gibbs P, Zlotecki R, Reith JD, Drane W: Effective salvage treatment of recurrent Ewing sarcoma utilizing chemotherapy and zoledronic acid. Clin Adv Hematol Oncol; 2010 Jul;8(7):499-504
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Effective salvage treatment of recurrent Ewing sarcoma utilizing chemotherapy and zoledronic acid.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Density Conservation Agents / therapeutic use. Bone Neoplasms / drug therapy. Diphosphonates / therapeutic use. Imidazoles / therapeutic use. Neoplasm Recurrence, Local / drug therapy. Salvage Therapy. Sarcoma, Ewing / drug therapy
  • [MeSH-minor] Adult. Drug Therapy, Combination. Humans. Male. Remission Induction. Treatment Outcome

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  • (PMID = 20864918.001).
  • [ISSN] 1543-0790
  • [Journal-full-title] Clinical advances in hematology & oncology : H&O
  • [ISO-abbreviation] Clin Adv Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Bone Density Conservation Agents; 0 / Diphosphonates; 0 / Imidazoles; 6XC1PAD3KF / zoledronic acid
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