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1. Marec-Bérard P, Chotel F, Claude L: [PNET/Ewing tumours: current treatments and future perspectives]. Bull Cancer; 2010 Jun;97(6):707-13
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  • [Title] [PNET/Ewing tumours: current treatments and future perspectives].
  • [Transliterated title] Tumeurs PNET/Ewing : prise en charge actuelle et perspectives.
  • Ewing tumours are characterised as tumours consisting of small, blue, round malignant cells that may exhibit varying degrees of neural differentiation.
  • Most of them arise in bony sites, and they represent the second commonest primary osseous malignancy in and adolescence and young adults.
  • During the past 30 years, chemotherapy has increased survival from less than 5% to 65-70% in localized tumours and to 25-30% in primary metastatic tumours.
  • Surgery is a major tool, whereas advances in imaging techniques have improved treatment indication and optimization.
  • Clinical and biological prognostic factors has been clearly identified and should guide the therapeutic choice for these patients.
  • The metastatic Ewing tumours are of extremely poor prognosis, and impose the development of new therapeutic agents.
  • This article is a review of the data available in 2009 concerning Ewing's sarcoma either as biologic aspects or as therapeutic aspects.
  • [MeSH-major] Bone Neoplasms / therapy. Sarcoma, Ewing / therapy
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Agents / therapeutic use. Forecasting. Humans. Neuroectodermal Tumors, Primitive, Peripheral / genetics. Neuroectodermal Tumors, Primitive, Peripheral / pathology. Neuroectodermal Tumors, Primitive, Peripheral / therapy. Prognosis. Radiotherapy Dosage. Young Adult

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  • (PMID = 20497910.001).
  • [ISSN] 1769-6917
  • [Journal-full-title] Bulletin du cancer
  • [ISO-abbreviation] Bull Cancer
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 42
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2. Vukasinović Z, Stevanović V, Spasovski D, Zivković Z: [Ewing sarcoma--current opinion]. Srp Arh Celok Lek; 2006 Jul-Aug;134(7-8):348-55
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  • [Title] [Ewing sarcoma--current opinion].
  • Ewing tumor family consists of Ewing tumor of bone, extraosseous Ewing tumor, primitive neurectodermal tumor and Askin tumor.
  • Ewing tumor is the most common form, found in 60% of cases.
  • It is the second primary malignant bone tumor.
  • Localized lesion is found in nearly 80% and metastatic disease in 20% of cases.
  • Ewing tumor can develop in virtually any bone of the body and in extraosseous localizations as well, while localization in the extremities occurs in 50% of patients.
  • Systemic symptoms are also present, commonly fever or weight loss, which often indicates the presence of metastatic disease with predominant invasion of lung, bone and bone marrow.
  • Multimodal chemotherapy with local radiation and/or surgical resection is the best way of modern treatment.
  • Distal parts of extremities and axial skeleton are good prognostic features, while proximal parts, pelvic girdle, metastatic disease and low index of postchemotherapeutic necrosis are associated with poor outcome.
  • [MeSH-major] Sarcoma, Ewing
  • [MeSH-minor] Bone Neoplasms / diagnosis. Bone Neoplasms / pathology. Bone Neoplasms / therapy. Humans

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  • (PMID = 17009618.001).
  • [ISSN] 0370-8179
  • [Journal-full-title] Srpski arhiv za celokupno lekarstvo
  • [ISO-abbreviation] Srp Arh Celok Lek
  • [Language] srp
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Serbia and Montenegro
  • [Number-of-references] 26
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3. Krasin MJ, Davidoff AM, Rodriguez-Galindo C, Billups CA, Fuller CE, Neel MD, Merchant TE: Definitive surgery and multiagent systemic therapy for patients with localized Ewing sarcoma family of tumors: local outcome and prognostic factors. Cancer; 2005 Jul 15;104(2):367-73
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  • [Title] Definitive surgery and multiagent systemic therapy for patients with localized Ewing sarcoma family of tumors: local outcome and prognostic factors.
  • BACKGROUND: The local management of Ewing sarcoma family of tumors (ESFT) often centers on the surgical resectability of the primary lesion and physician biases regarding differences in the morbidity between primary surgical and radiotherapeutic management.
  • METHODS: The authors retrospectively reviewed the records of 33 patients with localized ESFT who underwent surgery and received systemic chemotherapy at St. Jude Children's Research Hospital (Memphis, TN).
  • Two multiagent systemic chemotherapy regimens were used: 14 patients received vincristine, doxorubicin, cyclophosphamide, and actinomycin D (VACA), and 19 received VACA in combination with ifosphamide and etoposide.
  • The primary tumor was surgically resected via a wide, local excision (n = 32) or a marginal excision (n = 1)performed either at diagnosis or after 3-5 months of systemic chemotherapy.
  • The survival rate of patients whose tumors arose in bone was 78.6%, and the survival rate of patients whose tumors originated in soft tissue was 25.0% (P = 0.028).
  • CONCLUSIONS: Local disease control and overall outcome for patients with ESFT managed by multiagent systemic therapy and surgery was excellent.
  • Patients with extraosseous primary sites of disease may fare less well with this approach to therapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Sarcoma, Ewing / drug therapy. Sarcoma, Ewing / surgery
  • [MeSH-minor] Adolescent. Chemotherapy, Adjuvant / methods. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Dactinomycin / administration & dosage. Disease-Free Survival. Doxorubicin / administration & dosage. Etoposide / administration & dosage. Female. Humans. Ifosfamide / administration & dosage. Male. Neoplasm Recurrence, Local. Prognosis. Retrospective Studies. Survival Rate. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 15948159.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; UM20QQM95Y / Ifosfamide; VACA protocol
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4. 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.
  • The 5-year probability of PFS in patients with localized disease at diagnosis, and received ASCT after recurrence was 14% (95% CI 3-30).
  • 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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  • (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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5. Bacci G, Balladelli A, Forni C, Ferrari S, Longhi A, Bacchini P, Alberghini M, Fabbri N, Benassi M, Briccoli A, Picci P: Adjuvant and neoadjuvant chemotherapy for Ewing sarcoma family tumors in patients aged between 40 and 60: report of 35 cases and comparison of results with 586 younger patients treated with the same protocols in the same years. Cancer; 2007 Feb 15;109(4):780-6
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  • [Title] Adjuvant and neoadjuvant chemotherapy for Ewing sarcoma family tumors in patients aged between 40 and 60: report of 35 cases and comparison of results with 586 younger patients treated with the same protocols in the same years.
  • BACKGROUND: The clinical and pathologic features of 46 patients 40 to 60 years old with Ewing sarcoma family tumor (ESFT) diagnosed at the authors' institute between 1972 and 2000 were reviewed.
  • METHODS: Ten patients with metastatic tumors at presentation went elsewhere for treatment; 35 of 36 remaining cases with localized disease were treated at the authors' institution according to different chemotherapy protocols activated in successive years.
  • In patients with nonmetastatic tumors local treatment was surgery in 9 patients, radiotherapy in 16, and surgery followed by radiotherapy in 10.
  • RESULTS: At follow-up times ranging from 6 and 34 years (mean, 17.8 years), 15 patients (42.9%) remained continuously disease-free, 19 experienced recurrence, and 1 died of chemotherapy-related toxicity.
  • Comparing this group of patients with 586 cases of younger patients seen in the same period at Rizzoli, the only difference between the 2 groups was a significantly higher rate of tumors located in the soft tissues with a larger volume in the older group.
  • The results achieved were comparable in the 2 groups, although the older group had a lower chemotherapy dose-intensity and a higher rate of WHO grade 4 hematologic toxicity.
  • CONCLUSIONS: For patients with localized disease treated with adjuvant and neoadjuvant chemotherapy the results were essentially comparable in the 2 groups.
  • It is concluded that patients 40 years or older with ESFT should be treated in the same way as younger patients and included in treatment trials for these tumors.
  • [MeSH-major] Bone Neoplasms / drug therapy. Neoadjuvant Therapy. Sarcoma, Ewing / drug therapy
  • [MeSH-minor] Adult. Aged. Chemotherapy, Adjuvant. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local / drug therapy. Prognosis. Treatment Outcome

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  • (PMID = 17219445.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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6. Hamanoue S, Makimoto A: [Ewing sarcoma]. Gan To Kagaku Ryoho; 2007 Feb;34(2):175-80
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  • [Title] [Ewing sarcoma].
  • Ewing sarcoma is the second most frequent primary bone cancer affecting children or young adults.
  • Advances in molecular biology have revealed common chromosomal translocations such as EWS-FLI 1 among Ewing sarcoma and related diseases such as primitive neuroectodermal tumor (PNET), so these are considered as Ewing sarcoma family tumor (ESFT).
  • Although fewer than 10% of patients with ESFT survived before establishment of modern multiagent chemotherapy, the multimodal therapeutic regimens including combination chemotherapy, radiotherapy, and surgery can cure 60% of patients with localized disease, due to the collaborative research in European-American or the international trials.
  • The standard chemotherapy for localized ESFT now comprises vincristine, actinomycin D, cyclophosphamide and doxorubicin (VACD) in Europe or vincristine, doxorubicin, cyclophosphamide, ifosfamide and etoposide (VDC-IE) in North America.
  • New American-European collaborative trials such as EURO-E.W.I.N.G.99 are in progress for further improvement of the cure rate in localized and metastatic ESFT.
  • In Japan, Japan Ewing Sarcoma Study Group (JESS) phase II clinical trial for localized ESFT, and some clinical trials including new drugs are ongoing and waiting for results.
  • [MeSH-major] Antineoplastic Protocols. Bone Neoplasms. Sarcoma, Ewing
  • [MeSH-minor] Adolescent. Child. Combined Modality Therapy. Humans. Radiotherapy Dosage. Survival Rate

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  • (PMID = 17301523.001).
  • [ISSN] 0385-0684
  • [Journal-full-title] Gan to kagaku ryoho. Cancer & chemotherapy
  • [ISO-abbreviation] Gan To Kagaku Ryoho
  • [Language] jpn
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Japan
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7. Donaldson SS: Ewing sarcoma: radiation dose and target volume. Pediatr Blood Cancer; 2004 May;42(5):471-6
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  • [Title] Ewing sarcoma: radiation dose and target volume.
  • BACKGROUND: The outcome of children and adolescents with Ewing sarcoma is impacted by many prognostic factors and often measured by estimates of: event-free, relapse-free, disease-free, or overall survival.
  • However, the preferred assessment following radiation therapy is local control.
  • PROCEDURE: A review of large group experiences over the past several decades was undertaken to assess the optimal radiation dose and volume for patients with localized, osseous Ewing sarcoma.
  • RESULTS: With multidisciplinary therapy, 5-year overall local control rates range from 58 to 93%.
  • Following definitive irradiation, they are 53-86%.
  • Recommended radiation therapy doses are 55.8-60.0 Gy.
  • In the postoperative setting, gross disease requires 55.8 Gy; microscopic disease requires 45 Gy.
  • The appropriate irradiated volume is an involved field to the pretreatment tumor volume plus 2.0-2.5 cm margin, followed by a boost to the post-induction chemotherapy tumor volume with margin.
  • Techniques to improve local control include risk-adapted multidisciplinary therapy, intraoperative boost radiation, and high radiation doses as delivered by 3-dimensional conformal radiation.
  • CONCLUSIONS: Innovative uses of radiation in the multidisciplinary setting will continue to provide excellent local control, improved function, and quality of life for young patients with localized Ewing sarcoma of bone.
  • [MeSH-major] Radiotherapy Dosage. Sarcoma, Ewing / radiotherapy
  • [MeSH-minor] Child. Dose-Response Relationship, Radiation. Humans. Magnetic Resonance Imaging. Treatment Outcome

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  • [Copyright] Copyright 2004 Wiley-Liss, Inc.
  • (PMID = 15049023.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 15
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8. Marec-Bérard P, Philip T: Ewing sarcoma: the pediatrician's point of view. Pediatr Blood Cancer; 2004 May;42(5):477-80
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  • [Title] Ewing sarcoma: the pediatrician's point of view.
  • BACKGROUND: Integrated multimodal care is needed for patients with Ewing sarcoma, which is the second most common primary bone malignancy in children and adolescence.
  • Chemotherapy increases survival from less than 5% to 65-70% for patients with localized tumors and to 25-30% for those with metastases at diagnosis.
  • Surgery is a major tool, whereas advances in imaging techniques have improved the indications for and the optimization of treatment.
  • METHODS: The point of view of the pediatric oncologist was assessed as follows: the place of chemotherapy in Ewing tumor treatment, the place of radiotherapy in Ewing tumor treatment (including why avoid radiotherapy when technically possible in children), and how to proceed into the future?
  • The place of surgery as local treatment for Ewing tumors was also evaluated.
  • [MeSH-major] Pediatrics / methods. Sarcoma, Ewing / therapy
  • [MeSH-minor] Algorithms. Chemotherapy, Adjuvant. Child. Combined Modality Therapy. Humans. Orthopedic Procedures. Practice Guidelines as Topic. Radiotherapy, Adjuvant

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  • [Copyright] Copyright 2003 Wiley-Liss, Inc.
  • (PMID = 15049024.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 29
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9. Sokolov T, Stoianova A, Mumdjiev I, Mihova A: [Treatment of Ewing's sarcoma with 2 different protocols]. Ann Med Interne (Paris); 2001 Dec;152(8):497-501
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  • [Title] [Treatment of Ewing's sarcoma with 2 different protocols].
  • [Transliterated title] Traitement du sarcome d'Ewing par deux protocoles successifs.
  • Since 1985, 54 with localized Ewing's sarcoma of bone were treated at the Onco-Orthopedics Clinic of the Sofia University Hospital (Sofia, Bulgaria).
  • Thirty-two patients received the classical four-drug combination with doxorubicin, dactinomycin, vincristine, and cyclophosphamid.
  • The last 22 patients were treated with six-drug protocols where cisplatinium and ifosfamid were added to the standard four-drug regimens.
  • Local treatment was adapted to the age of the patient and the location and volume of the tumor.
  • Fourteen patients received radiotherapy alone, 24 postoperative radiotherapy and 16 surgery alone as local treatment.
  • Eleven patients had a good local response and 25 developed metastatic disease.
  • Statistical analysis confirmed the deleterious effect of pelvic localizations and large tumor volume on prognosis and demonstrated the dramatic improvement obtained with the six-drug protocol (5-year disease free survival 61% versus 23%) and surgery (61% versus 25%).
  • [MeSH-major] Bone Neoplasms / therapy. Sarcoma, Ewing / therapy
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Cisplatin / administration & dosage. Cyclophosphamide / administration & dosage. Dactinomycin / administration & dosage. Doxorubicin / administration & dosage. Female. Humans. Ifosfamide / administration & dosage. Male. Prognosis. Radiotherapy / adverse effects. Survival Rate. Vincristine / administration & dosage

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  • (PMID = 11937983.001).
  • [ISSN] 0003-410X
  • [Journal-full-title] Annales de médecine interne
  • [ISO-abbreviation] Ann Med Interne (Paris)
  • [Language] fre
  • [Publication-type] Comparative Study; English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; Q20Q21Q62J / Cisplatin; UM20QQM95Y / Ifosfamide
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10. Paulussen M, Ahrens S, Dunst J, Winkelmann W, Exner GU, Kotz R, Amann G, Dockhorn-Dworniczak B, Harms D, Müller-Weihrich S, Welte K, Kornhuber B, Janka-Schaub G, Göbel U, Treuner J, Voûte PA, Zoubek A, Gadner H, Jürgens H: Localized Ewing tumor of bone: final results of the cooperative Ewing's Sarcoma Study CESS 86. J Clin Oncol; 2001 Mar 15;19(6):1818-29
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  • [Title] Localized Ewing tumor of bone: final results of the cooperative Ewing's Sarcoma Study CESS 86.
  • PURPOSE: Cooperative Ewing's Sarcoma Study (CESS) 86 aimed at improving event-free survival (EFS) in patients with high-risk localized Ewing tumor of bone.
  • Local therapy was surgery (23%), surgery plus radiotherapy (49%), or radiotherapy alone (28%).
  • RESULTS: On May 1, 1999 (median time under study, 133 months), the 10-year EFS was 0.52.
  • In multivariate analyses, small tumor volumes of <200 mL, good histologic response, and VAIA chemotherapy augured for fair outcome.
  • Six of 301 patients (2%) died under treatment, and four patients (1.3%) developed second malignancies.
  • CONCLUSION: Fifty-two percent of CESS 86 patients survived after risk-adapted therapy.
  • High-risk patients seem to have benefited from intensified treatment that incorporated ifosfamide.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / drug therapy. Sarcoma, Ewing / drug therapy
  • [MeSH-minor] Adolescent. Adult. Chemotherapy, Adjuvant. Child. Child, Preschool. Cyclophosphamide / administration & dosage. Dactinomycin / administration & dosage. Doxorubicin / administration & dosage. Female. Humans. Ifosfamide / administration & dosage. Infant. Male. Middle Aged. Neoplasm Staging. Prognosis. Radiotherapy, Adjuvant. Risk Factors. Survival Analysis. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 11251014.001).
  • [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
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; UM20QQM95Y / Ifosfamide; VACA protocol; VAIA protocol
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11. 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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12. de Alava E, Antonescu CR, Panizo A, Leung D, Meyers PA, Huvos AG, Pardo-Mindán FJ, Healey JH, Ladanyi M: Prognostic impact of P53 status in Ewing sarcoma. Cancer; 2000 Aug 15;89(4):783-92
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  • [Title] Prognostic impact of P53 status in Ewing sarcoma.
  • BACKGROUND: Disease stage at the time of diagnosis and response to therapy are the main prognostic factors for patients with Ewing sarcoma or peripheral neuroectodermal tumor (ES/PNET).
  • METHODS: Prechemotherapy, paraffin embedded, nondecalcified, primary tumor material in a well-characterized series of 55 patients with ES/PNET with defined EWS-FLI1 fusion transcripts (32 patients with type 1 and 23 patients with other types) was studied retrospectively by immunohistochemical techniques for cell cycle regulators and proliferative markers, such as P53, P21(WAF1), and Ki-67, as well as by the terminal deoxynucleotidyl transferase-mediated dUTP nick-end labeling (TUNEL) technique for apoptosis.
  • Histologic response to neoadjuvant chemotherapy was assessed.
  • RESULTS: Aberrant P53 expression (in > 20% of tumor cells) was present in 6 patients (11%) but showed no statistically significant correlation with disease stage, tumor size, proliferation rate (Ki-67), apoptotic rate (TUNEL), or EWS-FLI1 fusion type.
  • By univariate analysis, the P53 > 20% group showed a significantly poorer overall survival among patients with localized disease (n = 43 patients) (P = 0.001) and in the entire study group (P = 0.01).
  • In multivariate Cox analyses of overall survival, P53 > 20% was the strongest negative factor among prognostic factors available at the time of diagnosis (P = 0.001; relative risk [RR] = 9) and when chemotherapy response was included in the analysis (P53 > 20%: P = 0.01; RR = 10).
  • [MeSH-major] Biomarkers, Tumor / metabolism. Bone Neoplasms / metabolism. Sarcoma, Ewing / metabolism. Tumor Suppressor Protein p53 / metabolism
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Agents / therapeutic use. Child. Child, Preschool. Female. Humans. Immunohistochemistry. Male. Neoplasm Staging. Oncogene Proteins, Fusion / genetics. Oncogene Proteins, Fusion / metabolism. Point Mutation. Prognosis. Prospective Studies. Proto-Oncogene Protein c-fli-1. RNA-Binding Protein EWS. Transcription Factors / genetics. Transcription Factors / metabolism. Treatment Outcome

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  • [Copyright] Copyright 2000 American Cancer Society.
  • (PMID = 10951341.001).
  • [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 / Antineoplastic Agents; 0 / Biomarkers, Tumor; 0 / EWS-FLI fusion protein; 0 / Oncogene Proteins, Fusion; 0 / Proto-Oncogene Protein c-fli-1; 0 / RNA-Binding Protein EWS; 0 / Transcription Factors; 0 / Tumor Suppressor Protein p53
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13. Khoury JD: Ewing sarcoma family of tumors. Adv Anat Pathol; 2005 Jul;12(4):212-20
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  • [Title] Ewing sarcoma family of tumors.
  • The Ewing sarcoma family of tumors (ESFT) comprises morphologically heterogeneous tumors that are characterized by nonrandom chromosomal translocations involving the EWS gene and one of several members of the ETS family of transcription factors.
  • Current therapy for patients with ESFT includes chemotherapy and surgery with or without radiation therapy.
  • At present, the most significant prognostic factor for patients with ESFT is whether the disease is localized or metastatic.
  • [MeSH-major] Antigens, CD / analysis. Biomarkers, Tumor / analysis. Bone Neoplasms / genetics. Cell Adhesion Molecules / analysis. Gene Expression Regulation, Neoplastic. Sarcoma, Ewing / genetics

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  • (PMID = 16096383.001).
  • [ISSN] 1072-4109
  • [Journal-full-title] Advances in anatomic pathology
  • [ISO-abbreviation] Adv Anat Pathol
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA-023099
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Biomarkers, Tumor; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules; 0 / EWS-FLI fusion protein; 0 / FLII protein, human; 0 / Gelsolin; 0 / Microfilament Proteins; 0 / Oncogene Proteins, Fusion; 0 / Proto-Oncogene Protein c-fli-1; 0 / RNA-Binding Protein EWS; 0 / Receptors, Cytoplasmic and Nuclear; 0 / Transcription Factors
  • [Number-of-references] 113
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14. Dileo P, Bertulli R, Piovesan C, Marrari A, Puma E, Spreafico C, Gronchi A, Olmi P, Gandola L, Casali PG: Long-term results with combined treatment in adult localized extraskeletal Ewing's sarcoma (ES): A retrospective analysis of 51 patients (pts) from a single referral center. J Clin Oncol; 2009 May 20;27(15_suppl):10548

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Long-term results with combined treatment in adult localized extraskeletal Ewing's sarcoma (ES): A retrospective analysis of 51 patients (pts) from a single referral center.
  • METHODS: We retrospectively reviewed adult pts with extraskeletal, localized ES, undergoing medical therapy between 1988 and 2004, who were included in the dataset of the current Adult Sarcoma Medical Unit at Istituto Nazionale Tumori, Milano, Italy, which serves as a reference center for adult soft tissue sarcoma.
  • Fifty-one pts were treated with a combined approach, including chemotherapy, surgery when appropriate, and radiation therapy.
  • Chemotherapy regimens included: vincristine (V), ifosfamide (I), epirubicin (E)/adriamycin (A), dacarbazine (D), actinomycinD (ActD), cisplatin (C), etoposide (Et).
  • 35/51 pts (68%) underwent preoperative chemotherapy: 30 pts received IVE × 4-7 cycles; 5 pts received VAI alternating with IEt × 9 cycles.
  • 42/51 pts underwent surgery; 38/51 pts received radiation therapy.
  • RESULTS: Response rate to induction chemotherapy including antracicline, ifosfamide + etoposide was 83%.
  • CONCLUSIONS: In this series of adult pts with extraskeletal ES, response rate and DFS were comparable to those of ES of bone in conventional series.
  • The primary site may identify a subset of pts with worse outcome and prognosis, for whom more intensive treatments could be investigated.

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  • (PMID = 27963955.001).
  • [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

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  • [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)].

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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. Krasin MJ, Rodriguez-Galindo C, Billups CA, Davidoff AM, Neel MD, Merchant TE, Kun LE: Definitive irradiation in multidisciplinary management of localized Ewing sarcoma family of tumors in pediatric patients: outcome and prognostic factors. Int J Radiat Oncol Biol Phys; 2004 Nov 1;60(3):830-8
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  • [Title] Definitive irradiation in multidisciplinary management of localized Ewing sarcoma family of tumors in pediatric patients: outcome and prognostic factors.
  • PURPOSE: To assess the effect of radiation dose on local tumor control of the Ewing sarcoma family of tumors in 79 patients with localized disease treated at a single institution.
  • METHODS AND MATERIALS: Thirty-seven patients received vincristine, actinomycin D, cyclophosphamide, and doxorubicin, and 42 received vincristine, actinomycin D, and cyclophosphamide, with alternating cycles of ifosfamide and etoposide; all underwent definitive radiotherapy (median dose, 37.5 Gy) with either low-dose (<40 Gy) or standard dose (> or =40 Gy) radiation delivered according to the protocol.
  • We calculated the cumulative incidence of local treatment failure, disease recurrence, and overall survival and analyzed the effect of known prognostic factors and radiation dose.
  • RESULTS: The cumulative incidence of local treatment failure at 10 years was 30.4% and that of disease recurrence was 40%.
  • Patient age > or =14 years and tumor size > or =8 cm were adverse prognostic factors for local treatment failure; patient age > or =14 years was also associated with worse survival.
  • Although the radiation dose alone did not predict for local treatment failure, the cumulative incidence of local failure at 10 years was 19% when tumors <8 cm were treated with <40 Gy, and no patient treated with standard doses (> or =40 Gy) developed local recurrence (p = 0.084).
  • CONCLUSION: Tumor size and patient age predict for local tumor control in patients with Ewing sarcoma family of tumors treated with systemic therapy and definitive radiotherapy.
  • [MeSH-major] Bone Neoplasms / radiotherapy. Sarcoma, Ewing / radiotherapy
  • [MeSH-minor] Adolescent. Adult. Age Factors. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Dactinomycin / administration & dosage. Etoposide / administration & dosage. Female. Humans. Ifosfamide / administration & dosage. Infant. Male. Neoplasm Recurrence, Local. Neoplasms, Second Primary. Neuroectodermal Tumors, Primitive, Peripheral / drug therapy. Neuroectodermal Tumors, Primitive, Peripheral / radiotherapy. Prognosis. Radiotherapy Dosage. Retrospective Studies. Treatment Failure. Vincristine / administration & dosage

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  • (PMID = 15465200.001).
  • [ISSN] 0360-3016
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; UM20QQM95Y / Ifosfamide
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17. Bacci G, Ferrari S, Mercuri M, Longhi A, Giacomini S, Forni C, Bertoni F, Manfrini M, Barbieri E, Lari S, Donati D: Multimodal therapy for the treatment of nonmetastatic Ewing sarcoma of pelvis. J Pediatr Hematol Oncol; 2003 Feb;25(2):118-24
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  • [Title] Multimodal therapy for the treatment of nonmetastatic Ewing sarcoma of pelvis.
  • The purpose of this study was to evaluate the outcome of patients with Ewing sarcoma (ES) of the pelvis, attempting to identify prognostic factors to select patients for more aggressive treatment.
  • Four different protocols of chemotherapy were used successively.
  • Local treatment consisted of surgery in 5 patients, radiotherapy in 60, and surgery followed by radiotherapy in 12.
  • Thirty-three patients remained continuously free of disease; 43 relapsed (24 due to metastases and 19 to local recurrence and metastases); 1 died of treatment-related complications.
  • These results are significantly worse than the ones achieved in 329 contemporary patients with extrapelvic lesions treated with the same protocols of chemotherapy (5- and 10-year event-free survival = 46% vs. 64% and 44% vs. 69%).
  • Thus, despite associated chemotherapy, the outcome of ES localized in the pelvis remains poor, and new innovative methods for the treatment of this tumor are needed.
  • [MeSH-major] Bone Neoplasms / therapy. Pelvic Bones. Sarcoma, Ewing / therapy
  • [MeSH-minor] Adolescent. Adult. Chemotherapy, Adjuvant. Child. Disease-Free Survival. Female. Humans. Male. Neoplasm Recurrence, Local / prevention & control. Radiotherapy, Adjuvant. Retrospective Studies. Risk Factors. Survival Analysis. Treatment Outcome

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  • (PMID = 12571462.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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18. Hawkins DS, Schuetze SM, Butrynski JE, Rajendran JG, Vernon CB, Conrad EU 3rd, Eary JF: [18F]Fluorodeoxyglucose positron emission tomography predicts outcome for Ewing sarcoma family of tumors. J Clin Oncol; 2005 Dec 1;23(34):8828-34
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [18F]Fluorodeoxyglucose positron emission tomography predicts outcome for Ewing sarcoma family of tumors.
  • PURPOSE: Response to neoadjuvant chemotherapy is a significant prognostic factor for the Ewing sarcoma family of tumors (ESFTs).
  • [18F]fluorodeoxyglucose (FDG) positron emission tomography (PET) is a noninvasive imaging modality that accurately predicts histopathologic response in several malignancies.
  • All patients received neoadjuvant and adjuvant chemotherapy.
  • FDG PET standard uptake values before (SUV1) and after (SUV2) chemotherapy were analyzed and correlated with chemotherapy response, as assessed by histopathology in surgically excised tumors.
  • SUV2 was associated with outcome (4-year PFS 72% for SUV2 < 2.5 v 27% for SUV2 > or = 2.5, P = .01 for all patients; 80% for SUV2 < 2.5 v 33% for SUV2 > or = 2.5, P = .036 for localized at diagnosis patients).
  • CONCLUSION: FDG PET imaging of ESFTs correlates with histologic response to neoadjuvant chemotherapy.
  • [MeSH-major] Bone Neoplasms / diagnosis. Fluorodeoxyglucose F18. Positron-Emission Tomography / methods. Sarcoma, Ewing / diagnosis
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Disease-Free Survival. Follow-Up Studies. Humans. Middle Aged. Neoplasm Recurrence, Local. Prognosis. Prospective Studies. Radiotherapy, Adjuvant. Reproducibility of Results. Sensitivity and Specificity. Surgical Procedures, Operative. Treatment Outcome

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  • (PMID = 16314643.001).
  • [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 / CA65537; United States / NCI NIH HHS / CA / CA87721
  • [Publication-type] Comparative Study; Evaluation Studies; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0Z5B2CJX4D / Fluorodeoxyglucose F18
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19. 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%.
  • 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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20. Paulino AC, Nguyen TX, Mai WY, Teh BS, Wen BC: Dose response and local control using radiotherapy in non-metastatic Ewing sarcoma. Pediatr Blood Cancer; 2007 Aug;49(2):145-8
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  • [Title] Dose response and local control using radiotherapy in non-metastatic Ewing sarcoma.
  • BACKGROUND: To determine prognostic factors for local control in the radiotherapeutic management of non-metastatic Ewing sarcoma.
  • PROCEDURE: Forty patients with localized Ewing sarcoma (ES) were treated with primary site RT at one institution.
  • Median RT dose was 55.8 Gy (range, 25.5-76 Gy).
  • Chemotherapy was given to 34 patients (85%) with the most common regimen being vincristine, dactinomycin, cyclophosphamide, doxorubicin alternating with ifosfamide and etoposide (VACA + IE) in 10.
  • The 5- and 10-year local control rate was 88.7% for RT dose >or=49 Gy and was 37.5% for <49 Gy (P = 0.0002, log-rank test).
  • For tumors <or=8 cm, the 5- and 10-year local control rate was 94.1% for RT dose >or=49 Gy and 50.0% for RT dose <49 Gy (P = 0.01, log-rank test).
  • For tumors >8 cm, the 5- and 10-year local control rate was 85.7% for RT dose >or=54 Gy and 26.7% for RT dose <54 Gy (P = 0.006, log-rank test).
  • In particular, patients who received RT doses >or=49 Gy for tumor size <or=8 cm and >or=54 Gy for tumor size >8 cm had improved local control.
  • [MeSH-major] Bone Neoplasms / radiotherapy. Radiotherapy, High-Energy. Sarcoma, Ewing / radiotherapy
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Adjuvant. Child. Child, Preschool. Cyclophosphamide / administration & dosage. Dactinomycin / administration & dosage. Disease-Free Survival. Dose Fractionation. Dose-Response Relationship, Radiation. Doxorubicin / administration & dosage. Etoposide / administration & dosage. Female. Humans. Ifosfamide / administration & dosage. Kaplan-Meier Estimate. Male. Middle Aged. Radiotherapy Dosage. Retrospective Studies. Treatment Outcome. United States / epidemiology. Vincristine / administration & dosage

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  • (PMID = 16732580.001).
  • [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] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; UM20QQM95Y / Ifosfamide; IE protocol; VAC protocol; VACA protocol
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21. Rodriguez-Galindo C, Spunt SL, Pappo AS: Treatment of Ewing sarcoma family of tumors: current status and outlook for the future. Med Pediatr Oncol; 2003 May;40(5):276-87
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment of Ewing sarcoma family of tumors: current status and outlook for the future.
  • BACKGROUND: The Ewing sarcoma family of tumors (ESFT) comprises a group of well-characterized neoplasms with aggressive behavior.
  • Despite significant progress with the use of intensive multiagent chemotherapy and local control measures, a significant proportion of patients die of disease progression.
  • Chemotherapy dose intensification and autologous hematopoietic stem cell transplant (HSCT) have been explored by many institutions without obvious benefit in high-risk patients.
  • Our current understanding in the biology and treatment of ESFT suggests that a more rational approach to the development of risk-adapted therapy should be undertaken.
  • PROCEDURE: We performed a review of the most relevant data regarding the current status in the treatment of ESFT.
  • The results of the major American and European cooperative groups were analyzed, including the treatment strategies used and the prognostic factors identified for both localized and metastatic ESFT.
  • This benefit seems to be restricted to patients with localized disease, and a proportion of survivors are at risk of developing treatment-related hematologic malignancies.
  • Nevertheless, these advances have resulted in a re-definition of prognostic factors, which may help to define risk groups based on tumor load parameters as well as biologic factors (type of fusion transcript and histologic response to chemotherapy).
  • New strategies such as immunotherapy and the use of biologic modifiers may have a role in the treatment of ESFT.
  • CONCLUSIONS: Future treatment for ESFT should consider risk-adapted strategies and the inclusion of newer therapies such as biologic modifiers for the minimal residual disease.
  • A modified risk-adapted therapy is proposed.
  • [MeSH-major] Antineoplastic Protocols. Bone Neoplasms / therapy. Sarcoma, Ewing / therapy
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Combined Modality Therapy. Hematopoietic Stem Cell Transplantation. Humans. Neoplasm Metastasis. Prognosis. Risk

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  • [Copyright] Copyright 2003 Wiley-Liss, Inc.
  • [ErratumIn] Med Pediatr Oncol. 2003 Dec;41(6):594
  • (PMID = 12652615.001).
  • [ISSN] 0098-1532
  • [Journal-full-title] Medical and pediatric oncology
  • [ISO-abbreviation] Med. Pediatr. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA23099; United States / NCI NIH HHS / CA / CA76379; United States / NCI NIH HHS / CA / P30 CA21765
  • [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] 108
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22. Worcester HD, Vasef MA: Therapy-related acute myeloid leukemia with 11q23 abnormality coexisting with refractory metastatic Ewing sarcoma: report of a case and review of the literature. Pediatr Dev Pathol; 2010 Jan-Feb;13(1):50-4
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  • [Title] Therapy-related acute myeloid leukemia with 11q23 abnormality coexisting with refractory metastatic Ewing sarcoma: report of a case and review of the literature.
  • Intensified chemotherapy is one of the strategies currently used in the treatment of children with metastatic Ewing sarcoma.
  • We report a patient who initially presented with localized Ewing sarcoma and later developed metastatic disease that required dose-intensified chemotherapy.
  • The patient's Ewing sarcoma remained refractory to treatment despite continuous intensified chemotherapy and was complicated by a therapy-related acute myeloid leukemia with 11q23 abnormality.
  • Examination of bone marrow at the last clinical follow up demonstrated both acute myeloid leukemia and residual metastatic Ewing sarcoma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / adverse effects. Bone Neoplasms / drug therapy. Chromosomes, Human, Pair 11. Leukemia, Myeloid, Acute / chemically induced. Lung Neoplasms / drug therapy. Sarcoma, Ewing / drug therapy. Translocation, Genetic
  • [MeSH-minor] Adolescent. Biomarkers, Tumor / metabolism. Bone Marrow / pathology. Humans. Lymph Nodes / pathology. Lymphatic Metastasis. Male. Neoplasm, Residual / pathology. Toes / pathology. Toes / surgery


23. Obata H, Ueda T, Kawai A, Ishii T, Ozaki T, Abe S, Tanaka K, Tsuchiya H, Matsumine A, Yabe H, Japanese Musculoskeletal Oncology Group: Clinical outcome of patients with Ewing sarcoma family of tumors of bone in Japan: the Japanese Musculoskeletal Oncology Group cooperative study. Cancer; 2007 Feb 15;109(4):767-75
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  • [Title] Clinical outcome of patients with Ewing sarcoma family of tumors of bone in Japan: the Japanese Musculoskeletal Oncology Group cooperative study.
  • BACKGROUND: Ewing sarcoma family of tumors (ESFT) of bone is extremely rare in Japan.
  • The objectives of the current study were to assess the clinical outcome and prognostic factors of patients with ESFT of bone in Japan and to compare them between Euro-American and Japanese populations.
  • METHODS: The authors conducted a retrospective analysis of 243 patients who were treated for ESFT of bone in Japan between 1981 and 2003.
  • Local therapy was surgery in 35% of patients, surgery combined with radiotherapy in 40% of patients, radiotherapy alone in 22% of patients, and no local treatment in 3% of patients.
  • All but 3 patients received various regimens of multidrug chemotherapy.
  • A univariate survival analysis demonstrated that patients who had metastases at presentation, primary site in the trunk, age >or=16 years, tumor size >or=10 cm, tumor that responded poorly to induction chemotherapy, and local treatment with radiotherapy alone had a significantly worse event-free survival (EFS).
  • Of 201 patients with localized disease, 45 patients who received current chemotherapy regimens that included ifosfamide and etoposide had a significantly better 5-year EFS rate (67.6%) compared with other patients.
  • CONCLUSIONS: The clinical outcome of patients with localized ESFT of bone in Japan has improved markedly with the use of current chemotherapy regimens that include ifosfamide and etoposide and has become comparable to the outcomes observed in other major series of Euro-American patients.
  • [MeSH-major] Bone Neoplasms / therapy. Sarcoma, Ewing / therapy
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Asian Continental Ancestry Group / ethnology. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Etoposide / administration & dosage. Female. Humans. Ifosfamide / administration & dosage. Infant. Infant, Newborn. Japan / epidemiology. Male. Middle Aged. Retrospective Studies. Survival Rate. Treatment Outcome

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  • (PMID = 17238190.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; UM20QQM95Y / Ifosfamide
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24. Paulino AC, Nguyen TX, Mai WY: An analysis of primary site control and late effects according to local control modality in non-metastatic Ewing sarcoma. Pediatr Blood Cancer; 2007 Apr;48(4):423-9
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  • [Title] An analysis of primary site control and late effects according to local control modality in non-metastatic Ewing sarcoma.
  • PURPOSE: To examine prognostic factors for primary site control and analyze late effects according to local treatment modality in non-metastatic Ewing sarcoma (ES).
  • MATERIALS AND METHODS: From 1976 to 2001, 76 patients with localized ES and a median age of 14.5 years were seen and treated at one institution.
  • Local therapy included radiotherapy (RT) alone in 40, surgery (S) alone in 27, and surgery followed by postoperative radiotherapy (S + RT) in 9.
  • Chemotherapy (CT) was delivered to 65 patients (86%).
  • Scoliosis and decrease range of motion of an extremity were seen regardless of local treatment modality.
  • [MeSH-major] Bone Neoplasms / therapy. Sarcoma, Ewing / therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Amputation. Antineoplastic Combined Chemotherapy Protocols / adverse effects. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Adjuvant / adverse effects. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Kaplan-Meier Estimate. Male. Middle Aged. Neoplasm Recurrence, Local / epidemiology. Neoplasms, Radiation-Induced / epidemiology. Neoplasms, Radiation-Induced / etiology. Neoplasms, Second Primary / epidemiology. Neoplasms, Second Primary / etiology. Prognosis. Proportional Hazards Models. Radiotherapy, Adjuvant / adverse effects. Survival Analysis. Treatment Outcome

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  • (PMID = 16421909.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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25. Schuetze SM: Chemotherapy in the management of osteosarcoma and Ewing's sarcoma. J Natl Compr Canc Netw; 2007 Apr;5(4):449-55
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  • [Title] Chemotherapy in the management of osteosarcoma and Ewing's sarcoma.
  • Sarcomas of bone are rare malignancies diagnosed in fewer than 3000 individuals yearly in the United States.
  • Ewing's sarcoma and most osteosarcoma are high-grade neoplasms and account for approximately one half of bone sarcoma cases.
  • Fewer than 20% of patients presenting with localized Ewing's sarcoma or osteosarcoma are cured with surgery alone.
  • Current management typically involves collaboration among orthopedic oncologists, medical oncologists, musculoskeletal radiologists, sarcoma pathologists, and radiation oncologists.
  • Modern multidisciplinary management of Ewing's sarcoma and osteosarcoma has improved the cure rate of patients with localized disease to more than 50%.
  • Primary chemotherapy for high-grade bone sarcomas often involves intensive, multiagent regimens, and few secondary chemotherapy options are available to treat refractory or relapsed disease.
  • Patient participation in clinical trials of novel therapies for Ewing's sarcoma and osteosarcoma should be strongly encouraged.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Bone Neoplasms / drug therapy. Osteosarcoma / drug therapy. Sarcoma, Ewing / drug therapy

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  • (PMID = 17442235.001).
  • [ISSN] 1540-1405
  • [Journal-full-title] Journal of the National Comprehensive Cancer Network : JNCCN
  • [ISO-abbreviation] J Natl Compr Canc Netw
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 60
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26. Misao T, Yoshikawa T, Aoe M, Furukawa M, Nakamura S, Kawakami K: Localized extraosseous Ewing's sarcoma of the chest wall resected by video-assisted thoracoscopic surgery. Gen Thorac Cardiovasc Surg; 2010 Dec;58(12):647-50
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  • [Title] Localized extraosseous Ewing's sarcoma of the chest wall resected by video-assisted thoracoscopic surgery.
  • Extraosseous Ewing's sarcoma (ES) is a small round cell tumor arising in soft tissue that was undifferentiated histologically from classic ES of bone.
  • Herein, we report a case of localized extraosseous ES of the chest wall resected by video-assisted thoracoscopic surgery (VATS).
  • Chest computed tomography showed a 1.5-cm solid tumor on the chest wall.
  • As adjuvant therapy, the patient was treated with radiotherapy, followed by multiagent chemotherapy.
  • [MeSH-major] Sarcoma, Ewing / surgery. Thoracic Neoplasms / surgery. Thoracic Surgery, Video-Assisted. Thoracic Wall / surgery
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Adjuvant. Cytogenetic Analysis. Humans. Magnetic Resonance Imaging. Male. Radiotherapy, Adjuvant. Tomography, X-Ray Computed. Treatment Outcome

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  • [Cites] Cancer. 1975 Jul;36(1):240-51 [1203852.001]
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  • (PMID = 21170637.001).
  • [ISSN] 1863-6713
  • [Journal-full-title] General thoracic and cardiovascular surgery
  • [ISO-abbreviation] Gen Thorac Cardiovasc Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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27. 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.
  • [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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28. Laurence V, Pierga JY, Barthier S, Babinet A, Alapetite C, Palangié T, de Pinieux G, Anract P, Pouillart P: Long-term follow up of high-dose chemotherapy with autologous stem cell rescue in adults with Ewing tumor. Am J Clin Oncol; 2005 Jun;28(3):301-9
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  • [Title] Long-term follow up of high-dose chemotherapy with autologous stem cell rescue in adults with Ewing tumor.
  • Ewing tumors remain of poor prognosis, with 5-year overall survival of 55% to 65% in localized patients and not exceeding 25% in primarily metastatic disease.
  • Several reports, mainly in children, have reported that some patients with poor-risk Ewing tumors may benefit from high-dose chemotherapy (HDCT) with autologous stem cell rescue.
  • This retrospective study analyzed 46 patients treated in our institution between 1987 and 2000 for localized or primary metastatic Ewing tumors by HDCT followed by stem cell rescue.
  • The treatment regimen consisted of induction chemotherapy, local treatment, maintenance chemotherapy, and consolidation HDCT based on alkylating agents.
  • No toxic death was observed in the intensive therapy phase.
  • This retrospective study shows a high long-term survival rate with high-dose chemotherapy in adults.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / therapy. Hematopoietic Stem Cell Transplantation. Sarcoma, Ewing / therapy
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Agents, Alkylating / administration & dosage. Busulfan / administration & dosage. Carmustine / administration & dosage. Cisplatin / administration & dosage. Cohort Studies. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Disease-Free Survival. Doxorubicin / administration & dosage. Etoposide / administration & dosage. Female. Follow-Up Studies. Humans. Ifosfamide / administration & dosage. Life Tables. Male. Melphalan / administration & dosage. Middle Aged. Peripheral Blood Stem Cell Transplantation. Prognosis. Retrospective Studies. Risk Factors. Survival Analysis. Transplantation Conditioning. Transplantation, Autologous. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 15923805.001).
  • [ISSN] 1537-453X
  • [Journal-full-title] American journal of clinical oncology
  • [ISO-abbreviation] Am. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; G1LN9045DK / Busulfan; Q20Q21Q62J / Cisplatin; Q41OR9510P / Melphalan; U68WG3173Y / Carmustine; UM20QQM95Y / Ifosfamide; CAV protocol
  • [Number-of-references] 46
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29. Schultze-Mosgau S, Thorwarth M, Wehrhan F, Holter W, Stachel KD, Grabenbauer G, Amann K, Beck JD: Ewing sarcoma of the mandible in a child: interdisciplinary treatment concepts and surgical reconstruction. J Craniofac Surg; 2005 Nov;16(6):1140-6
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  • [Title] Ewing sarcoma of the mandible in a child: interdisciplinary treatment concepts and surgical reconstruction.
  • Ewing's sarcoma is the second most common primary bone malignancy in childhood and adolescence.
  • We present a standardized interdisciplinary treatment protocol according to the EURO-E.W.I.N.G.
  • 99 study, applied in the treatment of a 7-year-old patient with localized Ewing's sarcoma of the left mandible.
  • After six blocks of VIDE (vincristine/ifosfamide/doxorubicin/etoposide) chemotherapy and stem cells rescue, intensity modulated external radiation with 48.6 Gy and subsequent high dose therapy with busulphan-melphalan were administered.
  • Because of the effective neoadjuvant treatment, no extensive soft tissue resection was necessary.
  • The presented case underlines the requirement for multidisciplinary protocols involving radiologists, pathologists, oncologists, radiation oncologists, and surgeons for accurate diagnosis and appropriate therapy.
  • [MeSH-major] Mandibular Neoplasms / surgery. Sarcoma, Ewing / surgery
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Transplantation. Child. Esthetics. Follow-Up Studies. Humans. Male. Microsurgery. Neoadjuvant Therapy. Peripheral Blood Stem Cell Transplantation. Radiotherapy Dosage. Reconstructive Surgical Procedures. Skin Transplantation. Surgical Flaps. Treatment Outcome. Wound Healing / physiology

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  • (PMID = 16327573.001).
  • [ISSN] 1049-2275
  • [Journal-full-title] The Journal of craniofacial surgery
  • [ISO-abbreviation] J Craniofac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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30. Sharma RR, Netalkar A, Lad SD: Primary Ewing's sarcoma of the greater wing of the sphenoid bone. Br J Neurosurg; 2000 Feb;14(1):53-6
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  • [Title] Primary Ewing's sarcoma of the greater wing of the sphenoid bone.
  • Primary Ewing's sarcoma is an uncommon lethal tumour of the long bones and pelvic girdle mainly affecting children and young adults.
  • We report a unique case of primary involvement of the greater wing of sphenoid bone in a 16-year-old patient.
  • Aggressive management using microsurgical resection, radiotherapy and chemotherapy was curative.
  • Localized, primary Ewing's sarcoma of the cranial bones should be considered as a distinct clinicopathological entity with an extremely low rate of dural penetration and metastases, and with a relatively better prognosis as compared with those of long bones and pelvic girdle.
  • In neurosurgical practice, primary Ewing's sarcoma of the cranial bones requires early aggressive management to achieve adequate long-term prognosis and cure.
  • [MeSH-major] Sarcoma, Ewing / surgery. Skull Neoplasms / surgery. Sphenoid Bone / surgery
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Female. Humans. Microsurgery / methods. Prognosis

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  • (PMID = 10884887.001).
  • [ISSN] 0268-8697
  • [Journal-full-title] British journal of neurosurgery
  • [ISO-abbreviation] Br J Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] ENGLAND
  • [Number-of-references] 19
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31. Thacker MM, Temple HT, Scully SP: Current treatment for Ewing's sarcoma. Expert Rev Anticancer Ther; 2005 Apr;5(2):319-31
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  • [Title] Current treatment for Ewing's sarcoma.
  • Ewing's sarcoma is the second most common primary bone tumor seen in children and adolescents, and was described by James Ewing in 1921 as a diffuse endothelioma of bone.
  • This is not a single condition, but a group of morphologically and clinically closely related disorders with similar molecular biology -- expression of tumor-specific chimeric oncoproteins through balanced chromosomal translocations involving the EWS gene -- often referred to as the Ewing family of tumors.
  • This includes Ewing's sarcoma of bone, extra-osseous Ewing's sarcoma, Askin tumor and peripheral neuroectodermal tumor.
  • Ewing's sarcoma has therefore been considered as a systemic disease necessitating local as well as systemic treatment.
  • Despite aggressive treatment, 20-40% of patients with localized disease and almost 80% of patients with metastatic disease at presentation succumb to the illness.
  • Advances in understanding the molecular biology of these tumors will hopefully result in the development of novel treatment approaches.
  • The aim of this article is to review the existing treatment methods and to highlight the more recent approaches to the treatment of this condition.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / drug therapy. Sarcoma, Ewing / drug therapy

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  • (PMID = 15877528.001).
  • [ISSN] 1744-8328
  • [Journal-full-title] Expert review of anticancer therapy
  • [ISO-abbreviation] Expert Rev Anticancer Ther
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 123
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32. McAllister NR, Lessnick SL: The potential for molecular therapeutic targets in Ewing's sarcoma. Curr Treat Options Oncol; 2005 Nov;6(6):461-71
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The potential for molecular therapeutic targets in Ewing's sarcoma.
  • Ewing's sarcoma is an uncompromising tumor of children and young adults.
  • Before the introduction of chemotherapy for Ewing's sarcoma, nearly all patients succumbed to their disease, even with highly aggressive approaches to local control.
  • Modern therapy for Ewing's sarcoma combines high-dose chemotherapy for systemic control of disease, with advanced surgical and/or radiation therapeutic approaches for local control.
  • Current therapy remains imperfect.
  • Despite optimal management, the cure rate for localized disease is only approximately 70%, whereas the cure rate for metastatic disease at presentation is less than 30%.
  • Patients who experience long-term disease-free survival are at risk for significant side effects of therapy, including infertility, limb dysfunction, and an increased risk for second malignancies.
  • More effective and less toxic therapies are needed.
  • This report presents an overview of dysregulated molecular pathways in Ewing's sarcoma and highlights the possibility that they may serve as therapeutic targets for the disease.
  • Although a great deal of additional investigation is required before most of these approaches can be assessed in the clinic, we think that these potential new targets offer a great deal of hope for patients with Ewing's sarcoma.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Bone Neoplasms / therapy. Oncogene Proteins, Fusion / antagonists & inhibitors. Proto-Oncogene Protein c-fli-1 / antagonists & inhibitors. Sarcoma, Ewing / therapy
  • [MeSH-minor] Adolescent. Cell Transformation, Neoplastic. Child. DNA-Binding Proteins / therapeutic use. Humans. Neovascularization, Pathologic / drug therapy. RNA-Binding Protein EWS. Receptors, Somatomedin / antagonists & inhibitors. Signal Transduction. Translocation, Genetic. Vascular Endothelial Growth Factor A / antagonists & inhibitors

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  • (PMID = 16242051.001).
  • [ISSN] 1527-2729
  • [Journal-full-title] Current treatment options in oncology
  • [ISO-abbreviation] Curr Treat Options Oncol
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / K08 CA096755; United States / NCI NIH HHS / CA / K08 CA096755-04; United States / NCI NIH HHS / CA / CA93247; United States / NCI NIH HHS / CA / CA96755
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / DNA-Binding Proteins; 0 / EWS-FLI fusion protein; 0 / Oncogene Proteins, Fusion; 0 / Proto-Oncogene Protein c-fli-1; 0 / RNA-Binding Protein EWS; 0 / Receptors, Somatomedin; 0 / Vascular Endothelial Growth Factor A
  • [Number-of-references] 60
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33. Huang HY, Illei PB, Zhao Z, Mazumdar M, Huvos AG, Healey JH, Wexler LH, Gorlick R, Meyers P, Ladanyi M: Ewing sarcomas with p53 mutation or p16/p14ARF homozygous deletion: a highly lethal subset associated with poor chemoresponse. J Clin Oncol; 2005 Jan 20;23(3):548-58
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  • [Title] Ewing sarcomas with p53 mutation or p16/p14ARF homozygous deletion: a highly lethal subset associated with poor chemoresponse.
  • PURPOSE: EWS-FLI1 fusion type, p53 mutation, and homozygous deletion of p16/p14ARF have each been shown to be prognostically significant in Ewing sarcoma (ES).
  • PATIENTS AND METHODS: We studied 60 patients with ES (stage: localized in 54, metastatic in six).
  • All cases were confirmed to contain the EWS-FLI1 (29 type 1, 12 type 2, 14 other types) or EWS-ERG fusions (five cases).
  • [MeSH-major] Bone Neoplasms / drug therapy. Bone Neoplasms / genetics. Genes, p16. Genes, p53. Oncogene Proteins, Fusion / genetics. Sarcoma, Ewing / drug therapy. Sarcoma, Ewing / genetics. Transcription Factors / genetics. Tumor Suppressor Protein p14ARF / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. DNA Mutational Analysis. Female. Follow-Up Studies. Humans. In Situ Hybridization, Fluorescence. Infant. Male. Middle Aged. Neoplasm Staging. Prognosis. Proto-Oncogene Protein c-fli-1. RNA-Binding Protein EWS. Sequence Deletion. Survival Analysis. Treatment Outcome

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  • (PMID = 15659501.001).
  • [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
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / EWS-FLI fusion protein; 0 / Oncogene Proteins, Fusion; 0 / Proto-Oncogene Protein c-fli-1; 0 / RNA-Binding Protein EWS; 0 / Transcription Factors; 0 / Tumor Suppressor Protein p14ARF
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34. Casadei R, Magnani M, Biagini R, Mercuri M: Prognostic factors in Ewing's sarcoma of the foot. Clin Orthop Relat Res; 2004 Mar;(420):230-8
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  • [Title] Prognostic factors in Ewing's sarcoma of the foot.
  • The outcome of treatment and prognostic factors were reviewed in 36 patients who had Ewing's sarcoma of the foot.
  • Age, levels of lactate dehydrogenase, degree of anemia, tumor volume, type of surgery, and radiotherapy were not related to prognosis.
  • The only observed prognostic factors are tumor site and treatment.
  • Patients treated with four-drug neoadjuvant chemotherapy had the best survival.
  • Fourteen of 32 patients (44%) with localized Ewing's sarcoma were continuously disease-free at an average followup of 7 years.
  • [MeSH-major] Bone Neoplasms / pathology. Metatarsal Bones / pathology. Sarcoma, Ewing / pathology. Tarsal Bones / pathology

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  • (PMID = 15057103.001).
  • [ISSN] 0009-921X
  • [Journal-full-title] Clinical orthopaedics and related research
  • [ISO-abbreviation] Clin. Orthop. Relat. Res.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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35. 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%.
  • 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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36. Cormier JN, Patel SR, Herzog CE, Ballo MT, Burgess MA, Feig BW, Hunt KK, Raney RB, Zagars GK, Benjamin RS, Pisters PW: Concurrent ifosfamide-based chemotherapy and irradiation. Analysis of treatment-related toxicity in 43 patients with sarcoma. Cancer; 2001 Sep 15;92(6):1550-5
Hazardous Substances Data Bank. IFOSFAMIDE .

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  • [Title] Concurrent ifosfamide-based chemotherapy and irradiation. Analysis of treatment-related toxicity in 43 patients with sarcoma.
  • BACKGROUND: The current study was performed to evaluate the toxicity profile of therapeutic doses of ifosfamide (IFX) given concurrently with full-dose external beam radiotherapy (EBRT) in patients with soft tissue and bone sarcomas.
  • METHODS: The medical records of 43 consecutive patients with soft tissue or bone sarcomas who were treated with concurrent IFX and EBRT were reviewed.
  • Histologies were rhabdomyosarcoma (n = 16 patients), Ewing sarcoma (n = 10 patients), malignant fibrous histiocytoma (n = 9 patients), and other soft tissue sarcomas (n = 8 patients).
  • Thirty-one patients (72%) had localized disease, and 12 patients (28%) had synchronous local and distant disease.
  • Treatment consisted of EBRT (median dose, 50.4 gray [Gy]) with concomitant IFX (median dose per cycle, 10.2 g/m(2)).
  • All patients with Ewing sarcoma or rhabdomyosarcoma received additional concurrent chemotherapy.
  • Confluent moist desquamation (Grade 3) occurred in nine patients in the treatment field; no patient experienced Grade 4 local toxicity.
  • CONCLUSIONS: Local and systemic toxicities after the administration of therapeutic doses of IFX with concomitant EBRT appear comparable to those observed with either treatment alone.
  • These results support the design of prospective studies evaluating concurrent ifosfamide and radiation therapy for patients with sarcomas.
  • [MeSH-major] Antineoplastic Agents, Alkylating / administration & dosage. Bone Neoplasms / therapy. Ifosfamide / administration & dosage. Sarcoma / therapy. Soft Tissue Neoplasms / therapy
  • [MeSH-minor] Adult. Aged. Child. Child, Preschool. Combined Modality Therapy. Female. Histiocytoma, Benign Fibrous / therapy. Humans. Infant. Male. Middle Aged. Rhabdomyosarcoma / therapy. Sarcoma, Ewing / therapy. Treatment Outcome

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  • [Copyright] Copyright 2001 American Cancer Society.
  • (PMID = 11745234.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; UM20QQM95Y / Ifosfamide
  • [Number-of-references] 27
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37. Yoshida K, Kusuzaki K, Matsubara T, Matsumine A, Kumamoto T, Komada Y, Naka N, Uchida A: Periosteal Ewing's sarcoma treated by photodynamic therapy with acridine orange. Oncol Rep; 2005 Feb;13(2):279-82
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  • [Title] Periosteal Ewing's sarcoma treated by photodynamic therapy with acridine orange.
  • We recently encountered a very rare case of periosteal Ewing's sarcoma (PES), which was treated by surgery followed by photodynamic therapy using acridine orange with radiodynamic therapy.
  • In our case, MRI revealed the tumor to be localized on the cortical surface of the proximal humerus.
  • Histopathological examination of biopsy specimens revealed a small round cell sarcoma suggestive of Ewing's sarcoma or PNET, and immunohistochemistry showed positive staining for MIC2.
  • Therefore, we administered preoperative chemotherapy, as a result of which the tumor shrank to 48% of its original volume.
  • With a view to preserve excellent shoulder and upper limb function, we attempted intralesional tumor resection supported by photodynamic therapy using acridine orange with radiodynamic therapy.
  • After surgery followed by postoperative chemotherapy, the patient has, until the time of writing, had no local tumor recurrence and no evidence of metastatic disease, and can move his shoulder fully and throw a ball well.
  • Since it has been reported that PES has a better prognosis and responsiveness to chemotherapy than intramedullary Ewing's sarcoma, we believe that such reduction surgery with photodynamic therapy might be the strategy of choice to obtain satisfactory limb function in cases of PES.
  • [MeSH-major] Bone Neoplasms / drug therapy. Humerus. Periosteum. Photochemotherapy / methods. Sarcoma, Ewing / drug therapy

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  • (PMID = 15643511.001).
  • [ISSN] 1021-335X
  • [Journal-full-title] Oncology reports
  • [ISO-abbreviation] Oncol. Rep.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] F30N4O6XVV / Acridine Orange
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38. Kilicaslan I, Karayigit E, Bulut F, Basaran M, Dizdar Y, Aslay I, Uysal V: Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) of the penis. Int Urol Nephrol; 2008;40(1):113-5
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  • [Title] Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) of the penis.
  • Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) of the penis has been very rarely defined.
  • We report a case of a 19-year-old patient with a tumor, localized in the dorsal side of penis, composed of small round cells with diffuse membranous mic-2 (CD99) immunopositivity.
  • The patient was treated with multiagent chemotherapy and radiotherapy.
  • [MeSH-major] Bone Neoplasms / pathology. Neuroectodermal Tumors / pathology. Penis / pathology. Sarcoma, Ewing / pathology
  • [MeSH-minor] Adult. Humans. Male. Treatment Outcome

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  • (PMID = 17952624.001).
  • [ISSN] 0301-1623
  • [Journal-full-title] International urology and nephrology
  • [ISO-abbreviation] Int Urol Nephrol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
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39. Tkachev SI, Nazarenko AV, Siniukov PA, Machak FN, Ivanov SM: [Radiotherapy as a component of treatment for Ewing's sarcoma]. Vopr Onkol; 2005;51(3):355-60
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  • [Title] [Radiotherapy as a component of treatment for Ewing's sarcoma].
  • The main stages of development of such conservative modalities as radio- and chemoradiotherapy for localized Ewing's sarcoma are discussed.
  • [MeSH-major] Bone Neoplasms / drug therapy. Bone Neoplasms / radiotherapy. Sarcoma, Ewing / drug therapy. Sarcoma, Ewing / radiotherapy
  • [MeSH-minor] Adult. Chemotherapy, Adjuvant. Disease-Free Survival. Female. Humans. Male. Radiotherapy, Adjuvant. Survival Analysis. Treatment Outcome

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  • (PMID = 16279102.001).
  • [ISSN] 0507-3758
  • [Journal-full-title] Voprosy onkologii
  • [ISO-abbreviation] Vopr Onkol
  • [Language] rus
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Russia (Federation)
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40. Stevanović V, Vukasinović Z, Spasovski D: [Ewing's sarcoma in children: prognosis in relation to the method of treatment]. Srp Arh Celok Lek; 2006 Sep-Oct;134(9-10):420-6
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  • [Title] [Ewing's sarcoma in children: prognosis in relation to the method of treatment].
  • Ewing's tumor of bone is the second primary malignant bone tumor.
  • Localized lesion is found in nearly 80% of cases and metastatic lesions are present in 20% at the time of diagnosis.
  • Treatment protocols were analyzed, prognostic parameters were evaluated, and overall survival as well as survival until relapse of disease was studied.
  • Prognostic features included age and sex, localization of tumor, type of applied surgical resection and treatment protocol, and presence of necrosis after neoadjuvant therapy as well as morphological characteristics of the tumor.
  • Multimodal chemotherapy with local radiation and/or surgical resection is the best mode of modern treatment.
  • Follow-up included the period from the completion of therapy to final control, with remission period defined by development of recurrence or metastatic lesions.
  • Localization on distal parts of the extremities and axial skeleton is good prognostic feature, while localization on proximal parts of the extremities and pelvic girdle, presence of metastatic disease and low index of postchemotherapeutic necrosis, are associated with poor outcome.
  • In Ewing's sarcoma, prognosis of good outcome is definitely very delicate process.
  • [MeSH-major] Bone Neoplasms / therapy. Sarcoma, Ewing / therapy
  • [MeSH-minor] Child. Combined Modality Therapy. Humans. Prognosis. Survival Rate

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  • (PMID = 17252910.001).
  • [ISSN] 0370-8179
  • [Journal-full-title] Srpski arhiv za celokupno lekarstvo
  • [ISO-abbreviation] Srp Arh Celok Lek
  • [Language] srp
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Serbia and Montenegro
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41. Bernstein M, Kovar H, Paulussen M, Randall RL, Schuck A, Teot LA, Juergens H: Ewing's sarcoma family of tumors: current management. Oncologist; 2006 May;11(5):503-19
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  • [Title] Ewing's sarcoma family of tumors: current management.
  • Ewing's sarcoma is the second most frequent primary bone cancer, with approximately 225 new cases diagnosed each year in patients less than 20 years of age in North America.
  • Pathognomonic translocations involving the ews gene on chromosome 22 and an ets-type gene, most commonly the fli1 gene on chromosome 11, are implicated in the great majority of cases.
  • Clinical presentation is usually dominated by local bone pain and a mass.
  • Approximately three quarters of patients have initially localized disease.
  • Management, preferably at a specialist center with a multi-disciplinary team, includes both local control-either surgery, radiation or a combination-and systemic chemotherapy.
  • Chemotherapy includes cyclic combinations, incorporating vincristine, doxorubicin, cyclophosphamide, etoposide, ifosfamide and occasionally actinomycin D.
  • Studies incorporating intensive therapy followed by stem cell infusion show no clear benefit.
  • New approaches include anti-angiogenic therapy, particularly since vascular endothelial growth factor is an apparent downstream target of the ews-fli1 oncogene.
  • [MeSH-major] Bone Neoplasms / therapy. Sarcoma, Ewing / therapy. Soft Tissue Neoplasms / therapy
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Humans. Oncogene Proteins, Fusion / genetics. Oncogene Proteins, Fusion / metabolism. Proto-Oncogene Protein c-fli-1 / genetics. Proto-Oncogene Protein c-fli-1 / metabolism. RNA-Binding Protein EWS. Radiotherapy Dosage

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  • (PMID = 16720851.001).
  • [ISSN] 1083-7159
  • [Journal-full-title] The oncologist
  • [ISO-abbreviation] Oncologist
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / EWS-FLI fusion protein; 0 / Oncogene Proteins, Fusion; 0 / Proto-Oncogene Protein c-fli-1; 0 / RNA-Binding Protein EWS
  • [Number-of-references] 157
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42. Daw NC, Mahmoud HH, Meyer WH, Jenkins JJ, Kaste SC, Poquette CA, Kun LE, Pratt CB, Rao BN: Bone sarcomas of the head and neck in children: the St Jude Children's Research Hospital experience. Cancer; 2000 May 1;88(9):2172-80
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  • [Title] Bone sarcomas of the head and neck in children: the St Jude Children's Research Hospital experience.
  • BACKGROUND: Bone sarcomas of the head and neck are difficult to resect.
  • METHODS: The records of the 28 patients with bone sarcomas originating in the head and neck treated at St. Jude Children's Research Hospital between March 1962 and January 1998 were reviewed.
  • RESULTS: There were 10 males and 18 females (median age, 12.6 years) each with a single sarcoma: osteosarcoma (18), Ewing sarcoma (7), malignant fibrous histiocytoma (MFH) (2), and fibrosarcoma (1).
  • All but one patient with Ewing sarcoma had localized disease at the time of diagnosis.
  • All patients underwent surgery: complete resection, 8; gross total resection, 4; incomplete resection, 14; and biopsy only, 2; 22 also received chemotherapy.
  • Radiotherapy was given to all patients with Ewing sarcoma and to four patients with primary osteosarcoma.
  • Local disease progression was evident in 12 patients (9 with osteosarcoma, 2 with MFH, and 1 with Ewing sarcoma) who died of disease, 9 of whom had the initial treatment of biopsy alone or incomplete resection.
  • Patients with osteosarcoma who had the initial treatment of incomplete resection or biopsy alone were more likely to experience local failure (P = 0.001) and had poorer survival (P = 0.014) than those who underwent complete or gross total resection.
  • CONCLUSIONS: Bone sarcomas of the head and neck are rare among children and most often are localized at the time of diagnosis.
  • Although aggressive surgery is essential for the cure of osteosarcoma, its necessity in the treatment of Ewing sarcomas remains controversial.
  • [MeSH-major] Sarcoma / surgery. Skull Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Cause of Death. Chemotherapy, Adjuvant. Child. Child, Preschool. Disease Progression. Female. Fibrosarcoma / surgery. Histiocytoma, Benign Fibrous / surgery. Hospitals, Pediatric. Humans. Infant. Male. Mandibular Neoplasms / surgery. Maxillary Neoplasms / surgery. Neoplasm Recurrence, Local / pathology. Osteosarcoma / surgery. Radiotherapy, Adjuvant. Retrospective Studies. Sarcoma, Ewing / surgery. Survival Rate. Tennessee. Treatment Outcome

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  • (PMID = 10813731.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Grant] 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.
  • [Publication-country] UNITED STATES
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43. Laws HJ, van Kaick B, Pape H, Paulussen M, Göbel U: Relapse after high-dose therapy in relapsed Ewing's tumor patients: effects of maintenance chemotherapy in two selected patients? Onkologie; 2003 Dec;26(6):573-7
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  • [Title] Relapse after high-dose therapy in relapsed Ewing's tumor patients: effects of maintenance chemotherapy in two selected patients?
  • BACKGROUND: The prognosis for patients with high-risk Ewing's tumor, i.e. primarily multifocal or early relapsed disease, is extremely poor with conventional relapse therapy.
  • High-dose radio/chemotherapy (HDC) with subsequent stem cell transplantation seems to improve outcome in this patient cohort.
  • In spite of this intensified therapy however, relapse remains the most frequent cause of death.
  • In the majority of patients the time to second relapse after HDC is shorter than the time to first relapse after conventional therapy (3.4 vs. 18 months).
  • Event-free survival in Ewing's tumor patients who suffer a second relapse after first-line therapy (EICESS 92) is 2% after 18 months.
  • The patient with very late multifocal relapse after first-line treatment and a second localized relapse 30 months after HDC remains disease-free for 5 years after the last relapse.
  • However, the other patient with 2 early relapses after first-line treatment and HDC, respectively, did not benefit from this regime.
  • CONCLUSION: We propose that low-dose maintenance therapy may be beneficial in the subgroup of Ewing's tumor patients with late relapse after HDC.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / drug therapy. Cyclophosphamide / analogs & derivatives. Neoplasm Recurrence, Local / drug therapy. Neoplasms, Multiple Primary / drug therapy. Sarcoma, Ewing / drug therapy
  • [MeSH-minor] Adolescent. Bone Marrow Transplantation. Child. Combined Modality Therapy. Disease-Free Survival. Dose-Response Relationship, Drug. Etoposide / administration & dosage. Etoposide / adverse effects. Female. Follow-Up Studies. Hematopoietic Stem Cell Transplantation. Humans. Radiotherapy Dosage. Radiotherapy, Adjuvant. Retreatment

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  • [Copyright] Copyright 2003 S. Karger GmbH, Freiburg
  • (PMID = 14709933.001).
  • [ISSN] 0378-584X
  • [Journal-full-title] Onkologie
  • [ISO-abbreviation] Onkologie
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; H64JRU6GJ0 / trofosfamide
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44. Paulussen M, Craft AW, Lewis I, Hackshaw A, Douglas C, Dunst J, Schuck A, Winkelmann W, Köhler G, Poremba C, Zoubek A, Ladenstein R, van den Berg H, Hunold A, Cassoni A, Spooner D, Grimer R, Whelan J, McTiernan A, Jürgens H, European Intergroup Cooperative Ewing's Sarcoma Study-92: Results of the EICESS-92 Study: two randomized trials of Ewing's sarcoma treatment--cyclophosphamide compared with ifosfamide in standard-risk patients and assessment of benefit of etoposide added to standard treatment in high-risk patients. J Clin Oncol; 2008 Sep 20;26(27):4385-93
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  • [Title] Results of the EICESS-92 Study: two randomized trials of Ewing's sarcoma treatment--cyclophosphamide compared with ifosfamide in standard-risk patients and assessment of benefit of etoposide added to standard treatment in high-risk patients.
  • PURPOSE: The European Intergroup Cooperative Ewing's Sarcoma Study investigated whether cyclophosphamide has a similar efficacy as ifosfamide in standard-risk (SR) patients and whether the addition of etoposide improves survival in high-risk (HR) patients.
  • PATIENTS AND METHODS: SR patients (localized tumors, volume <100 mL) were randomly assigned to receive four courses of vincristine, dactinomycin, ifosfamide, and doxorubicin (VAIA) induction therapy followed by 10 courses of either VAIA or vincristine, dactinomycin, cyclophosphamide, and doxorubicin (VACA; cyclophosphamide replacing ifosfamide).
  • Outcome measures were event-free survival (EFS; defined as the time to first recurrence, progression, second malignancy, or death) and overall survival (OS).
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / drug therapy. Etoposide / administration & dosage. Ifosfamide / administration & dosage. Sarcoma, Ewing / drug therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Dactinomycin / administration & dosage. Disease-Free Survival. Doxorubicin / administration & dosage. Female. Follow-Up Studies. Hematologic Diseases / chemically induced. Humans. Infant. Lung Neoplasms / secondary. Male. Neoplasm Staging. Prospective Studies. Vincristine / administration & dosage


45. Taylor M, Guillon M, Champion V, Marcu M, Arnoux JB, Hartmann O: [Ewing's tumor]. Arch Pediatr; 2005 Sep;12(9):1383-91
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  • [Title] [Ewing's tumor].
  • [Transliterated title] La tumeur d'Ewing.
  • Ewing's tumor (ET) is a malignant bone tumor occurring in children and young adults.
  • ET affects mainly bones of the central axis, and almost always involves soft tissue infiltration.
  • Multiagent chemotherapy in association with local treatment (surgery and/or radiation) has clearly improved outcome.
  • The introduction of systemic treatment was justified by the frequent sub-clinical diffusion of apparently localized ET.
  • Intensified therapeutic strategies have for the first time cured some metastatic ET patients, but at the cost of major side effects.
  • Treatment is currently adapted as a result of a better definition of prognostic factors as well as a better assessment of its adverse effects.
  • ET is a fascinating example of the progress made not only in the diagnostic and therapeutic approach to cancer but also in the comprehension of the mechanisms behind carcinogenesis, and consequently reflects the revolution of medicine over the last century.
  • [MeSH-major] Bone Neoplasms / therapy. Sarcoma, Ewing / therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Humans. Infant. Neoadjuvant Therapy. Prognosis. Quality of Life. Treatment Outcome

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  • (PMID = 16046110.001).
  • [ISSN] 0929-693X
  • [Journal-full-title] Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie
  • [ISO-abbreviation] Arch Pediatr
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Number-of-references] 39
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