Ewing's Sarcoma Family of Tumors: Current Management

Oncologist - Tập 11 Số 5 - Trang 503-519 - 2006
Mark L. Bernstein1, Heinrich Kovar2, Michael Paulussen3, R. Lor Randall4, Andreas Schuck5, Lisa A. Teot6, Herbert Juergensg7
1aSte-Justine Hospital, University of Montreal, Montreal, Canada
2bChildren's Cancer Research Institute, Vienna, Austria
3cUniversity Children's Hospital Basel, Basel, Switzerland
4dHuntsman Cancer Institute & Primary Children's Medical Center, University of Utah, Salt Lake City, Utah, USA
5e Department of Radiotherapy, University Hospital Muenster, Münster, Germany
6fDepartment of Pathology, University of Pittsburgh School of Medicine and Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, USA
7gUniversity of Muenster, Children's Hospital, Paediatric Haematology and Oncology, Muenster, Germany

Tóm tắt

Abstract Learning Objectives

After completing this course, the reader will be able to: Describe the presentation, differential diagnosis, and prognosis for patients with Ewing's sarcoma.Explain the principles of multidisciplinary management of Ewing's sarcoma.Discuss the late effects of the therapy for Ewing's sarcoma.

Access and take the CME test online and receive 1 AMA PRA category 1 credit at CME.TheOncologist.com

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. It is one of the pediatric small round blue cell tumors, characterized by strong membrane expression of CD99 in a chain-mail pattern and negativity for lymphoid (CD45), rhabdomyosarcoma (myogenin, desmin, actin) and neuroblastoma (neurofilament protein) markers. 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. Imaging reveals a technetium pyrophosphate avid lesion that, on plain radiograph, is destructive, diaphyseal and classically causes layered periosteal calcification. Magnetic resonance best defines the extent of the lesion. Biopsy should be undertaken by an experienced orthopedic oncologist. Approximately three quarters of patients have initially localized disease. About two thirds survive disease-free. 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. Topotecan in combination with cyclophosphamide has shown preliminary activity. Patients with initially metastatic disease fare less well, with about one quarter surviving. 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.

Từ khóa


Tài liệu tham khảo

Gurney, 1999, Cancer Incidence and Survival Among Children and Adolescents: United States SEER Program 1975–1995, 99

Hense, 1999, Klin Padiatr, 211, 271, 10.1055/s-2008-1043799

Widhe, 2000, Initial symptoms and clinical features in osteosarcoma and Ewing sarcoma, J Bone Joint Surg Am, 82, 667, 10.2106/00004623-200005000-00007

Sneppen, 1984, Presenting symptoms and treatment delay in osteosarcoma and Ewing's sarcoma, Acta Radiol Oncol, 23, 159, 10.3109/02841868409136005

Craft, 1997, Long-term results from the first UKCCSG Ewing's Tumour Study (ET-1). United Kingdom Children's Cancer Study Group (UKCCSG) and the Medical Research Council Bone Sarcoma Working Party, Eur J Cancer, 33, 1061, 10.1016/S0959-8049(97)00043-9

Ferrari, 2001, Ewing's sarcoma of bone: relation between clinical characteristics and staging, Oncol Rep, 8, 553

Henk, 1998, et al. [Ewing sarcoma. Diagnostic imaging], Radiologe, 38, 509, 10.1007/s001170050386

Tateishi, 2003, Chest wall tumors: radiologic findings and pathologic correlation: part 2. Malignant tumors, Radiographics, 23, 1491, 10.1148/rg.236015527

Frouge, 1988, The role of magnetic resonance imaging in the evaluation of Ewing sarcoma. A report of 27 cases, Skeletal Radiol, 17, 387, 10.1007/BF00361656

Cohen, 1986, Efficacy of magnetic resonance imaging in 139 children with tumors, Arch Surg, 121, 522, 10.1001/archsurg.1986.01400050032004

Mankin, 1996, The hazards of the biopsy, revisited. Members of the Musculoskeletal Tumor Society, J Bone Joint Surg Am, 78, 656, 10.2106/00004623-199605000-00004

Ewing, 1921, Diffuse endothelioma of bone, Proc N Y Pathol Soc, 21, 17

Horowitz, 1992, Ewing's sarcoma, CA Cancer J Clin, 42, 300, 10.3322/canjclin.42.5.300

Tsokos, 1992, Peripheral primitive neuroectodermal tumors. Diagnosis, classification, and prognosis, Perspect Pediatr Pathol, 16, 27

Dehner, 1993, Primitive neuroectodermal tumor and Ewing's sarcoma, Am J Surg Pathol, 17, 1, 10.1097/00000478-199301000-00001

Fellinger, 1991, Biochemical and genetic characterization of the HBA71 Ewing's sarcoma cell surface antigen, Cancer Res, 51, 336

Ramani, 1993, Immunocytochemical study of 12E7 in small round-cell tumours of childhood: an assessment of its sensitivity and specificity, Histopathology, 23, 557, 10.1111/j.1365-2559.1993.tb01243.x

Weidner, 1994, Immunohistochemical profile of monoclonal antibody O13: antibody that recognizes glycoprotein p30/32MIC2 and is useful in diagnosing Ewing's sarcoma and peripheral neuroepithelioma, Am J Surg Pathol, 18, 486, 10.1097/00000478-199405000-00008

Navarro, 1994, Comparison of Ewing's sarcoma of bone and peripheral neuroepithelioma. An immunocytochemical and ultrastructural analysis of two primitive neuroectodermal neoplasms, Arch Pathol Lab Med, 118, 608

Lizard-Nacol, 1989, Immunologic characterization of Ewing's sarcoma using mesenchymal and neural markers, Am J Pathol, 135, 847

Dierick, 1993, The immunophenotype of Ewing's sarcoma. An immunohistochemical analysis, Pathol Res Pract, 189, 26, 10.1016/S0344-0338(11)80113-5

Shanfield, 1997, Immunohistochemical analysis of neural markers in peripheral primitive neuroectodermal tumor (pPNET) without light microscopic evidence of neural differentiation, Appl Immunohistochem Mol Morphol, 5, 78, 10.1097/00022744-199706000-00002

Gu, 2000, Cytokeratin immunoreactivity in Ewing's sarcoma: prevalence in 50 cases confirmed by molecular diagnostic studies, Am J Surg Pathol, 24, 410, 10.1097/00000478-200003000-00010

Ladanyi, 2000, Contribution of molecular genetic data to the classification of sarcomas, Hum Pathol, 31, 532, 10.1053/hp.2000.6706

Hill, 2002, Practical application of molecular genetic testing as an aid to the surgical pathologic diagnosis of sarcomas: a prospective study, Am J Surg Pathol, 26, 965, 10.1097/00000478-200208000-00001

Aurias, 1983, et al. [Translocation of chromosome 22 in Ewing's sarcoma], C R Seances Acad Sci III, 296, 1105

Turc-Carel, 1988, Chromosomes in Ewing's sarcoma. I. An evaluation of 85 cases of remarkable consistency of t(11;22)(q24;q12), Cancer Genet Cytogenet, 32, 229, 10.1016/0165-4608(88)90285-3

Hattinger, 1999, Prognostic impact of deletions at 1p36 and numerical aberrations in Ewing tumors, Genes Chromosomes Cancer, 24, 243, 10.1002/(SICI)1098-2264(199903)24:3<243::AID-GCC10>3.0.CO;2-A

Hattinger, 2002, Prognostic impact of chromosomal aberrations in Ewing tumours, Br J Cancer, 86, 1763, 10.1038/sj.bjc.6600332

Maurici, 1998, Frequency and implications of chromosome 8 and 12 gains in Ewing sarcoma, Cancer Genet Cytogenet, 100, 106, 10.1016/S0165-4608(97)00028-9

Kovar, 1997, Among genes involved in the RB dependent cell cycle regulatory cascade, the p16 tumor suppressor gene is frequently lost in the Ewing family of tumors, Oncogene, 15, 2225, 10.1038/sj.onc.1201397

Wei, 2000, Prognostic impact of INK4A deletion in Ewing sarcoma, Cancer, 89, 793, 10.1002/1097-0142(20000815)89:4<793::AID-CNCR11>3.0.CO;2-M

Huang, 2005, Ewing sarcomas with p53 mutation or p16/p14ARF homozygous deletion: a highly lethal subset associated with poor chemoresponse, J Clin Oncol, 23, 548, 10.1200/JCO.2005.02.081

Delattre, 1992, Gene fusion with an ETS DNA-binding domain caused by chromosome translocation in human tumours, Nature, 359, 162, 10.1038/359162a0

Zucman, 1992, Cloning and characterization of the Ewing's sarcoma and peripheral neuroepithelioma t(11;22) translocation breakpoints, Genes Chromosomes Cancer, 5, 271, 10.1002/gcc.2870050402

Ambros, 1991, MIC2 is a specific marker for Ewing's sarcoma and peripheral primitive neuroectodermal tumors. Evidence for a common histogenesis of Ewing's sarcoma and peripheral primitive neuroectodermal tumors from MIC2 expression and specific chromosome aberration, Cancer, 67, 1886, 10.1002/1097-0142(19910401)67:7<1886::AID-CNCR2820670712>3.0.CO;2-U

Ladanyi, 1993, EWS rearrangement in Ewing's sarcoma and peripheral neuroectodermal tumor. Molecular detection and correlation with cytogenetic analysis and MIC2 expression, Diagn Mol Pathol, 2, 141

Desmaze, 1994, Interphase molecular cytogenetics of Ewing's sarcoma and peripheral neuroepithelioma t(11;22) with flanking and overlapping cosmid probes, Cancer Genet Cytogenet, 74, 13, 10.1016/0165-4608(94)90022-1

Shing, 2003, FUS/ERG gene fusions in Ewing's tumors, Cancer Res, 63, 4568

Marley, 1997, Primitive neuroectodermal tumor of the kidney--another enigma: a pathologic, immunohistochemical, and molecular diagnostic study, Am J Surg Pathol, 21, 354, 10.1097/00000478-199703000-00013

Sezer, 1999, CD99 positivity and EWS-FLI1 gene rearrangement identify a breast tumor in a 60-year-old patient with attributes of the Ewing family of neoplasms, Diagn Mol Pathol, 8, 120, 10.1097/00019606-199909000-00003

Sheaff, 1997, Primitive neuroectodermal tumor of the kidney confirmed by fluorescence in situ hybridization, Am J Surg Pathol, 21, 461, 10.1097/00000478-199704000-00013

Kuroda, 2000, Primary primitive neuroectodermal tumor of the kidney, Pathol Int, 50, 967, 10.1046/j.1440-1827.2000.01147.x

Mikami, 2001, Primary pulmonary primitive neuroectodermal tumor (PNET). A case report, Pathol Res Pract, 197, 113

Kie, 2003, Primary Ewing's sarcoma of the suodenum: a case report, Int J Surg Pathol, 11, 331, 10.1177/106689690301100416

Tokudome, 2002, Primitive neuroectodermal tumor of the transverse colonic mesentery defined by the presence of EWS-FLI1 chimeric mRNA in a Japanese woman, J Gastroenterol, 37, 543, 10.1007/s005350200084

Shek, 2001, Ewing sarcoma of the small intestine, J Pediatr Hematol Oncol, 23, 530, 10.1097/00043426-200111000-00013

Maesawa, 2002, Esophageal extraskeletal Ewing's sarcoma, Hum Pathol, 33, 130, 10.1053/hupa.2002.30219

Colecchia, 2003, Primary primitive peripheral neuroectodermal tumor of the prostate. Immunophenotypic and molecular study of a case, Arch Pathol Lab Med, 127, e190, 10.5858/2003-127-e190-PPPNTO

Sinkre, 2000, Endometrial endometrioid carcinomas associated with Ewing sarcoma/peripheral primitive neuroectodermal tumor, Int J Gynecol Pathol, 19, 127, 10.1097/00004347-200004000-00005

Kato, 2001, Ewing's sarcoma family of tumor arising in the adrenal gland--possible diagnostic pitfall in pediatric pathology: histologic, immunohistochemical, ultrastructural, and molecular study, Hum Pathol, 32, 1012, 10.1053/hupa.2001.27121

Dedeurwaerdere, 2002, Primary peripheral PNET/Ewing's sarcoma of the dura: a clinicopathologic entity distinct from central PNET, Mod Pathol, 15, 673, 10.1038/modpathol.3880585

Cheung, 2001, Extraskeletal Ewing sarcoma in a 77-year-old woman, Arch Pathol Lab Med, 125, 1358, 10.5858/2001-125-1358-EESIAY

Daldrup-Link, 2001, Whole-body MR imaging for detection of bone metastases in children and young adults: comparison with skeletal scintigraphy and FDG PET, AJR Am J Roentgenol, 177, 229, 10.2214/ajr.177.1.1770229

Hawkins, 2005, J Clin Oncol, 23, 8828, 10.1200/JCO.2005.01.7079

Paulussen, 1998, Primary metastatic (stage IV) Ewing tumor: survival analysis of 171 patients from the EICESS studies. European Intergroup Cooperative Ewing Sarcoma Studies, Ann Oncol, 9, 275, 10.1023/A:1008208511815

Zoubek, 1998, Predictive potential of testing for bone marrow involvement in Ewing tumor patients by RT-PCR: a preliminary evaluation, Int J Cancer, 79, 56, 10.1002/(SICI)1097-0215(19980220)79:1<56::AID-IJC11>3.0.CO;2-F

Schleiermacher, 2003, Increased risk of systemic relapses associated with bone marrow micrometastasis and circulating tumor cells in localized Ewing tumor, J Clin Oncol, 21, 85, 10.1200/JCO.2003.03.006

Avigad, 2004, The predictive potential of molecular detection in the nonmetastatic Ewing family of tumors, Cancer, 100, 1053, 10.1002/cncr.20059

Ewing, 1924, Further report of endothelial myeloma of bone, Proc N Y Pathol Soc, 24, 93

Jenkin, 1966, Ewing's sarcoma a study of treatment methods, Clin Radiol, 17, 97, 10.1016/S0009-9260(66)80064-8

Phillips, 1967, The curability of Ewing's endothelioma of bone in children, J Pediatr, 70, 391, 10.1016/S0022-3476(67)80136-7

Hustu, 1968, Treatment of Ewing's sarcoma with concurrent radiotherapy and chemotherapy, J Pediatr, 73, 249, 10.1016/S0022-3476(68)80078-2

Sutow, 1971, Evaluation of chemotherapy in children with metastatic Ewing's sarcoma and osteogenic sarcoma, Cancer Chemother Rep, 55, 67

Rosen, 1974, Proceedings: disease-free survival in children with Ewing's sarcoma treated with radiation therapy and adjuvant four-drug sequential chemotherapy, Cancer, 33, 384, 10.1002/1097-0142(197402)33:2<384::AID-CNCR2820330213>3.0.CO;2-T

Gasparini, 1977, Ten years experience with Ewing's sarcoma, Tumori, 63, 77, 10.1177/030089167706300110

Jurgens, 1988, Multidisciplinary treatment of primary Ewing's sarcoma of bone. A 6-year experience of a European Cooperative Trial, Cancer, 61, 23, 10.1002/1097-0142(19880101)61:1<23::AID-CNCR2820610106>3.0.CO;2-M

Nesbit, 1990, et al. Multimodal therapy for the management of primary, nonmetastatic Ewing's sarcoma of bone: a long-term follow-up of the First Intergroup study, J Clin Oncol, 8, 1664, 10.1200/JCO.1990.8.10.1664

Burgert, 1990, Multimodal therapy for the management of nonpelvic, localized Ewing's sarcoma of bone: intergroup study IESS-II, J Clin Oncol, 8, 1514, 10.1200/JCO.1990.8.9.1514

Cotterill, 2000, Prognostic factors in Ewing's tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing's Sarcoma Study Group, J Clin Oncol, 18, 3108, 10.1200/JCO.2000.18.17.3108

Craft, 1998, Ifosfamide-containing chemotherapy in Ewing's sarcoma: The Second United Kingdom Children's Cancer Study Group and the Medical Research Council Ewing's Tumor Study, J Clin Oncol, 16, 3628, 10.1200/JCO.1998.16.11.3628

Oberlin, 2001, Prognostic factors in localized Ewing's tumours and peripheral neuroectodermal tumours: the third study of the French Society of Paediatric Oncology (EW88 study), Br J Cancer, 85, 1646, 10.1054/bjoc.2001.2150

Paulussen, 2001, Localized Ewing tumor of bone: final results of the cooperative Ewing's Sarcoma Study CESS 86, J Clin Oncol, 19, 1818, 10.1200/JCO.2001.19.6.1818

Kolb, 2003, Long-term event-free survival after intensive chemotherapy for Ewing's family of tumors in children and young adults, J Clin Oncol, 21, 3423, 10.1200/JCO.2003.10.033

Grier, 2003, Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone, N Engl J Med, 348, 694, 10.1056/NEJMoa020890

Bacci, 2004, Long-term outcome for patients with non-metastatic Ewing's sarcoma treated with adjuvant and neoadjuvant chemotherapies. 402 patients treated at Rizzoli between 1972 and 1992, Eur J Cancer, 40, 73, 10.1016/j.ejca.2003.08.022

Meyers, 2001, High-dose melphalan, etoposide, total-body irradiation, and autologous stem-cell reconstitution as consolidation therapy for high-risk Ewing's sarcoma does not improve prognosis, J Clin Oncol, 19, 2812, 10.1200/JCO.2001.19.11.2812

Aparicio, 1998, Long-term follow-up and prognostic factors in Ewing's sarcoma. A multivariate analysis of 116 patients from a single institution, Oncology, 55, 20, 10.1159/000011841

Carrie, 1999, Nonmetastatic pelvic Ewing sarcoma: report of the French society of pediatric oncology, Med Pediatr Oncol, 33, 444, 10.1002/(SICI)1096-911X(199911)33:5<444::AID-MPO2>3.0.CO;2-D

Bacci, 2000, Prognostic factors in nonmetastatic Ewing's sarcoma of bone treated with adjuvant chemotherapy: analysis of 359 patients at the Istituto Ortopedico Rizzoli, J Clin Oncol, 18, 4, 10.1200/JCO.2000.18.1.4

Schuck, 2003, Local therapy in localized Ewing tumors: results of 1058 patients treated in the CESS 81, CESS 86, and EICESS 92 trials, Int J Radiat Oncol Biol Phys, 55, 168, 10.1016/S0360-3016(02)03797-5

Dunst, 1991, Radiation therapy as local treatment in Ewing's sarcoma. Results of the Cooperative Ewing's Sarcoma Studies CESS 81 and CESS 86, Cancer, 67, 2818, 10.1002/1097-0142(19910601)67:11<2818::AID-CNCR2820671118>3.0.CO;2-Y

Gebhardt, 1991, Limb Salvage—Major Reconstructions in Oncologic and Nontumoral Conditions, 561

Shapiro, 1993, Treatment of tibial defects and nonunions using ipsilateral vascularized fibular transposition, Clin Orthop, 207

Ozaki, 1997, Reconstruction of tibia by ipsilateral vascularized fibula and allograft. 12 cases with malignant bone tumors, Acta Orthop Scand, 68, 298, 10.3109/17453679708996706

Schulte, 1999, Evaluation of neoadjuvant therapy response of osteogenic sarcoma using FDG PET, J Nucl Med, 40, 1637

Woude, 1998, Preoperative evaluation and monitoring chemotherapy in patients with high-grade osteogenic and Ewing's sarcoma: review of current imaging modalities, Skeletal Radiol, 27, 57, 10.1007/s002560050339

Sato, 1998, Value of thallium-201 scintigraphy in bone and soft tissue tumors, J Orthop Sci, 3, 297, 10.1007/s007760050056

Reddick, 1995, Dynamic contrast-enhanced MR imaging evaluation of osteosarcoma response to neoadjuvant chemotherapy, J Magn Reson Imaging, 5, 689, 10.1002/jmri.1880050612

Imbriaco, 1997, Thallium-201 scintigraphy for the evaluation of tumor response to preoperative chemotherapy in patients with osteosarcoma, Cancer, 80, 1507, 10.1002/(SICI)1097-0142(19971015)80:8<1507::AID-CNCR19>3.0.CO;2-9

Schuck, 1998, Radiotherapy in Ewing's sarcoma and PNET of the chest wall: results of the trials CESS 81, CESS 86 and EICESS 92, Int J Radiat Oncol Biol Phys, 42, 1001, 10.1016/S0360-3016(98)00294-6

Sailer, 1988, Ewing's sarcoma: surgical resection as a prognostic factor, Int J Radiat Oncol Biol Phys, 15, 43, 10.1016/0360-3016(88)90345-8

Arai, 1991, Ewing's sarcoma: local tumor control and patterns of failure following limited-volume radiation therapy, Int J Radiat Oncol Biol Phys, 21, 1501, 10.1016/0360-3016(91)90325-X

Donaldson, 1998, A multidisciplinary study investigating radiotherapy in Ewing's sarcoma: end results of POG #8346. Pediatric Oncology Group, Int J Radiat Oncol Biol Phys, 42, 125, 10.1016/S0360-3016(98)00191-6

Dunst, 1995, Radiation therapy in Ewing's sarcoma: an update of the CESS 86 trial, Int J Radiat Oncol Biol Phys, 32, 919, 10.1016/0360-3016(95)00016-R

Sutow, 1962, Cyclophosphamide therapy in children with Ewing's sarcoma, Cancer Chemother Rep, 23, 55

Pinkel, 1962, Cyclophosphamide in children with cancer, Cancer, 15, 42, 10.1002/1097-0142(196201/02)15:1<42::AID-CNCR2820150107>3.0.CO;2-D

Granowetter, 2001, Comparison of dose intensified and standard dose chemotherapy for the treatment of non-metastatic Ewing's sarcoma (ES) and primitive neuroectodermal tumor (PNET) of bone and soft tissue: a Pediatric Oncology Group - Children's Cancer Group phase III trial, Med Pediatr Oncol, 37, 172

Rosen, 1978, Curability of Ewing's sarcoma and considerations for future therapeutic trials, Cancer, 41, 888, 10.1002/1097-0142(197803)41:3<888::AID-CNCR2820410316>3.0.CO;2-T

Kushner, 1995, Very-high-dose short-term chemotherapy for poor-risk peripheral primitive neuroectodermal tumors, including Ewing's sarcoma, in children and young adults, J Clin Oncol, 13, 2796, 10.1200/JCO.1995.13.11.2796

Hayes, 1989, Therapy for localized Ewing's sarcoma of bone, J Clin Oncol, 7, 208, 10.1200/JCO.1989.7.2.208

Meyer, 1992, Ifosfamide plus etoposide in newly diagnosed Ewing's sarcoma of bone, J Clin Oncol, 10, 1737, 10.1200/JCO.1992.10.11.1737

Marina, 1999, Chemotherapy dose-intensification for pediatric patients with Ewing's family of tumors and desmoplastic small round-cell tumors: a feasibility study at St. Jude Children's Research Hospital, J Clin Oncol, 17, 180, 10.1200/JCO.1999.17.1.180

Bacci, 2002, Neoadjuvant chemotherapy for Ewing's tumour of bone: recent experience at the Rizzoli Orthopaedic Institute, Eur J Cancer, 38, 2243, 10.1016/S0959-8049(02)00148-X

Elomaa, 2000, Five-year results in Ewing's sarcoma. The Scandinavian Sarcoma Group experience with the SSG IX protocol, Eur J Cancer, 36, 875, 10.1016/S0959-8049(00)00028-9

Kung, 1993, Ifosfamide/etoposide combination in the treatment of recurrent malignant solid tumors of childhood. A Pediatric Oncology Group Phase II study, Cancer, 71, 1898, 10.1002/1097-0142(19930301)71:5<1898::AID-CNCR2820710529>3.0.CO;2-Q

Miser, 1987, Ifosfamide with mesna uroprotection and etoposide: an effective regimen in the treatment of recurrent sarcomas and other tumors of children and young adults, J Clin Oncol, 5, 1191, 10.1200/JCO.1987.5.8.1191

Pratt, 1996, Response of pediatric malignant solid tumors following ifosfamide or ifosfamide/carboplatin/etoposide: a single hospital experience, Med Pediatr Oncol, 27, 145, 10.1002/(SICI)1096-911X(199609)27:3<145::AID-MPO2>3.0.CO;2-E

EURO-E.W.I.N.G., 2006, Study Committee. EURO-E.W.I.N.G. 99 Study Manual—EUROpean Ewing Tumor Initiative of National Groups Ewing Tumor Studies 1999

Womer, 2000, Granulocyte colony stimulating factor permits dose intensification by interval compression in the treatment of Ewing's sarcomas and soft tissue sarcomas in children, Eur J Cancer, 36, 87, 10.1016/S0959-8049(99)00236-1

Kushner, 2000, Pilot study of topotecan and high-dose cyclophosphamide for resistant pediatric solid tumors, Med Pediatr Oncol, 35, 468, 10.1002/1096-911X(20001101)35:5<468::AID-MPO5>3.0.CO;2-P

Saylors, 2001, Cyclophosphamide plus topotecan in children with recurrent or refractory solid tumors: a Pediatric Oncology Group phase II study, J Clin Oncol, 19, 3463, 10.1200/JCO.2001.19.15.3463

Ladenstein, 1995, Impact of megatherapy in children with high-risk Ewing's tumours in complete remission: a report from the EBMT Solid Tumour Registry, Bone Marrow Transplant, 15, 697

Kinsella, 1983, Intensive combined modality therapy including low-dose TBI in high-risk Ewing's sarcoma patients, Int J Radiat Oncol Biol Phys, 9, 1955, 10.1016/0360-3016(83)90368-1

Burdach, 1993, Myeloablative radiochemotherapy and hematopoietic stem-cell rescue in poor-prognosis Ewing's sarcoma, J Clin Oncol, 11, 1482, 10.1200/JCO.1993.11.8.1482

Kushner, 2001, How effective is dose-intensive/myeloablative therapy against Ewing's sarcoma/primitive neuroectodermal tumor metastatic to bone or bone marrow? The Memorial Sloan-Kettering experience and a literature review, J Clin Oncol, 19, 870, 10.1200/JCO.2001.19.3.870

Pinkerton, 1995, Intensive chemotherapy with stem cell support-experience in pediatric solid tumours, Bull Cancer, 82, 61s

Meyers, 2004, High-dose therapy with autologous stem cell rescue for pediatric sarcomas, Curr Opin Oncol, 16, 120, 10.1097/00001622-200403000-00006

Cangir, 1990, Ewing's sarcoma metastatic at diagnosis. Results and comparisons of two intergroup Ewing's sarcoma studies, Cancer, 66, 887, 10.1002/1097-0142(19900901)66:5<887::AID-CNCR2820660513>3.0.CO;2-R

Sandoval, 1996, Outcome in 43 children presenting with metastatic Ewing sarcoma: the St. Jude Children's Research Hospital experience, 1962 to 1992, Med Pediatr Oncol, 26, 180, 10.1002/(SICI)1096-911X(199603)26:3<180::AID-MPO6>3.0.CO;2-G

Bizer, 1983, [Programs for the radiation and drug treatment of metastases of Ewing's sarcoma to the lungs in children], Med Radiol (Mosk), 28, 8

Dunst, 1993, Lung irradiation for Ewing's sarcoma with pulmonary metastases at diagnosis: results of the CESS-studies, Strahlenther Onkol, 169, 621

Paulussen, 1998, Ewing's tumors with primary lung metastases: survival analysis of 114 (European Intergroup) Cooperative Ewing's Sarcoma Studies patients, J Clin Oncol, 16, 3044, 10.1200/JCO.1998.16.9.3044

Whelan, 2002, A systematic review of the role of pulmonary irradiation in the management of primary bone tumours, Ann Oncol, 13, 23, 10.1093/annonc/mdf047

Folkman, 1972, Anti-angiogenesis: new concept for therapy of solid tumors, Ann Surg, 175, 409, 10.1097/00000658-197203000-00014

Vacca, 1999, Antiangiogenesis is produced by non-toxic doses of vinblastine, Blood, 94, 4143, 10.1182/blood.V94.12.4143

Klement, 2002, Differences in therapeutic indexes of combination metronomic chemotherapy and an anti-VEGFR-2 antibody in multidrug-resistant human breast cancer xenografts, Clin Cancer Res, 8, 221

Fosslien, 2000, Molecular pathology of cyclooxygenase-2 in neoplasia, Ann Clin Lab Sci, 30, 3

Ahrens, 1999, Evaluation of prognostic factors in a tumor volume-adapted treatment strategy for localized Ewing sarcoma of bone: the CESS 86 experience. Cooperative Ewing Sarcoma Study, Med Pediatr Oncol, 32, 186, 10.1002/(SICI)1096-911X(199903)32:3<186::AID-MPO5>3.0.CO;2-D

Bacci, 1998, Neoadjuvant chemotherapy for Ewing's sarcoma of bone: no benefit observed after adding ifosfamide and etoposide to vincristine, actinomycin, cyclophosphamide, and doxorubicin in the maintenance phase--results of two sequential studies, Cancer, 82, 1174, 10.1002/(SICI)1097-0142(19980315)82:6<1174::AID-CNCR24>3.0.CO;2-2

Klingebiel, 1998, Treatment of children with relapsed soft tissue sarcoma: report of the German CESS/CWS REZ 91 trial, Med Pediatr Oncol, 30, 269, 10.1002/(SICI)1096-911X(199805)30:5<269::AID-MPO2>3.0.CO;2-D

Ozaki, 1996, Significance of surgical margin on the prognosis of patients with Ewing's sarcoma. A report from the Cooperative Ewing's Sarcoma Study, Cancer, 78, 892, 10.1002/(SICI)1097-0142(19960815)78:4<892::AID-CNCR29>3.0.CO;2-P

Rodriguez-Galindo, 2002, Survival after recurrence of Ewing tumors: the St Jude Children's Research Hospital experience, 1979–1999, Cancer, 94, 561, 10.1002/cncr.10192

Burdach, 2004, Treatment of advanced Ewing tumors by combined radiochemotherapy and engineered cellular transplants, Pediatr Transplant, 8, 67, 10.1111/j.1398-2265.2004.00186.x

McLean, 1999, Late events in pediatric patients with Ewing sarcoma/primitive neuroectodermal tumor of bone: the Dana-Farber Cancer Institute/Children's Hospital experience, J Pediatr Hematol Oncol, 21, 486, 10.1097/00043426-199911000-00008

Shankar, 1999, Local therapy and other factors influencing site of relapse in patients with localised Ewing's sarcoma. United Kingdom Children's Cancer Study Group (UKCCSG), Eur J Cancer, 35, 1698, 10.1016/S0959-8049(99)00144-6

Nesbit, 1981, Multimodal therapy for the management of primary, nonmetastatic Ewing's sarcoma of bone: an Intergroup Study, Natl Cancer Inst Monogr, 255

Leu, 2004, Laboratory and clinical evidence of synergistic cytotoxicity of sequential treatment with gemcitabine followed by docetaxel in the treatment of sarcoma, J Clin Oncol, 22, 1706, 10.1200/JCO.2004.08.043

Burdach, 2003, High-dose therapy for patients with primary multifocal and early relapsed Ewing's tumors: results of two consecutive regimens assessing the role of total-body irradiation, J Clin Oncol, 21, 3072, 10.1200/JCO.2003.12.039

Frohlich, 1999, et al. [High-dosage chemotherapy in primary metastasized and relapsed Ewing's sarcoma. (EI)CESS], Klin Padiatr, 211, 284

Hawkins, 2000, Busulfan, melphalan, and thiotepa with or without total marrow irradiation with hematopoietic stem cell rescue for poor-risk Ewing-Sarcoma-Family tumors, Med Pediatr Oncol, 34, 328, 10.1002/(SICI)1096-911X(200005)34:5<328::AID-MPO3>3.0.CO;2-4

Kovar, 2003, Potentials for RNAi in sarcoma research and therapy: Ewing's sarcoma as a model, Semin Cancer Biol, 13, 275, 10.1016/S1044-579X(03)00041-5

Kovar, 1996, EWS/FLI-1 antagonists induce growth inhibition of Ewing tumor cells in vitro, Cell Growth Differ, 7, 429

Lambert, 2000, EWS Fli-1 antisense nanocapsules inhibits Ewing sarcoma-related tumor in mice, Biochem Biophys Res Commun, 279, 401, 10.1006/bbrc.2000.3963

Ouchida, 1995, Loss of tumorigenicity of Ewing's sarcoma cells expressing antisense RNA to EWS-fusion transcripts, Oncogene, 11, 1049

Tanaka, 1997, EWS-Fli1 antisense oligodeoxynucleotide inhibits proliferation of human Ewing's sarcoma and primitive neuroectodermal tumor cells, J Clin Invest, 99, 239, 10.1172/JCI119152

Toretsky, 1997, Inhibition of EWS-FLI-1 fusion protein with antisense oligodeoxynucleotides, J Neurooncol, 31, 9, 10.1023/A:1005716926800

Dohjima, 2003, Small interfering RNAs expressed from a Pol III promoter suppress the EWS/Fli-1 transcript in an Ewing sarcoma cell line, Mol Ther, 7, 811, 10.1016/S1525-0016(03)00101-1

Maksimenko, 2003, Oligonucleotides targeted against a junction oncogene are made efficient by nanotechnologies, Pharm Res, 20, 1565, 10.1023/A:1026122914852

Scotlandi, 2000, CD99 engagement: an effective therapeutic strategy for Ewing tumors, Cancer Res, 60, 5134

Kuttesch, 1996, Second malignancies after Ewing's sarcoma: radiation dose-dependency of secondary sarcomas, J Clin Oncol, 14, 2818, 10.1200/JCO.1996.14.10.2818

Dunst, 1998, Second malignancies after treatment for Ewing's sarcoma: a report of the CESS-studies, Int J Radiat Oncol Biol Phys, 42, 379, 10.1016/S0360-3016(98)00228-4

Le Vu, 1998, Radiation dose, chemotherapy and risk of osteosarcoma after solid tumours during childhood, Int J Cancer, 77, 370, 10.1002/(SICI)1097-0215(19980729)77:3<370::AID-IJC11>3.0.CO;2-C

Paulussen, 2001, Second malignancies after Ewing tumor treatment in 690 patients from a cooperative German/Austrian/Dutch study, Ann Oncol, 12, 1619, 10.1023/A:1013148730966

Friedman, 2002, Late effects of childhood cancer therapy, Pediatr Clin North Am, 49, 1083, 10.1016/S0031-3955(02)00032-9

Nagarajan, 2003, Education, employment, insurance, and marital status among 694 survivors of pediatric lower extremity bone tumors: a report from the childhood cancer survivor study, Cancer, 97, 2554, 10.1002/cncr.11363

Fuchs, 2003, Complications in long-term survivors of Ewing sarcoma, Cancer, 98, 2687, 10.1002/cncr.11891