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Epidemiology, pathology, and molecular genetics of the Ewing sarcoma family of tumors

Thomas F DeLaney, MD
Francis J Hornicek, MD, PhD
Armita Bahrami, MD
Section Editors
Alberto S Pappo, MD
Robert Maki, MD, PhD
Raphael E Pollock, MD
Deputy Editor
Diane MF Savarese, MD


Ewing sarcoma (ES) and peripheral primitive neuroectodermal tumor (PNET) were originally described as distinct clinicopathologic entities:

In 1918, Stout described a tumor of the ulnar nerve with the gross features of a sarcoma but composed of small round cells focally arranged as rosettes; this entity was subsequently designated neuroepithelioma, and then PNET [1].

ES was described by James Ewing in 1921 as an undifferentiated tumor involving the diaphysis of long bones that, in contrast to osteosarcoma, was radiation sensitive. Although most often a primary bone tumor, ES was also reported to arise in soft tissue (extraosseous Ewing sarcoma [EES]) [2].

However, over the last several decades, it has become clear that these entities comprise the same spectrum of neoplastic diseases known as the Ewing sarcoma family of tumors (EFT), which also includes malignant small cell tumor of the chest wall (Askin tumor) [3] and atypical ES [4,5]. Because of their similar histologic and immunohistochemical characteristics and their shared nonrandom chromosomal translocations, these tumors are considered to be derived from a common cell of origin, although the histogenic origin of this cell is debated [6-13]. (See 'Histogenesis' below and "Diseases of the chest wall", section on 'Malignant neoplasms'.)

The EFT can develop in almost any bone or soft tissue, but the most common site is in a flat or long bone, and patients typically present with localized pain and swelling. Although overt metastatic disease is found in fewer than 25 percent at the time of diagnosis, subclinical metastatic disease is assumed to be present in nearly all patients because of the 80 to 90 percent relapse rate in patients undergoing local therapy alone. As a result, systemic chemotherapy has evolved as an important component of treatment.

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Literature review current through: Sep 2017. | This topic last updated: Jun 05, 2017.
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  1. Jaffe R, Santamaria M, Yunis EJ, et al. The neuroectodermal tumor of bone. Am J Surg Pathol 1984; 8:885.
  2. Angervall L, Enzinger FM. Extraskeletal neoplasm resembling Ewing's sarcoma. Cancer 1975; 36:240.
  3. Askin FB, Rosai J, Sibley RK, et al. Malignant small cell tumor of the thoracopulmonary region in childhood: a distinctive clinicopathologic entity of uncertain histogenesis. Cancer 1979; 43:2438.
  4. Llombart-Bosch A, Lacombe MJ, Contesso G, Peydro-Olaya A. Small round blue cell sarcoma of bone mimicking atypical Ewing's sarcoma with neuroectodermal features. An analysis of five cases with immunohistochemical and electron microscopic support. Cancer 1987; 60:1570.
  5. Grier HE. The Ewing family of tumors. Ewing's sarcoma and primitive neuroectodermal tumors. Pediatr Clin North Am 1997; 44:991.
  6. Ambros IM, Ambros PF, Strehl S, et al. 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 1991; 67:1886.
  7. Delattre O, Zucman J, Melot T, et al. The Ewing family of tumors--a subgroup of small-round-cell tumors defined by specific chimeric transcripts. N Engl J Med 1994; 331:294.
  8. Denny CT. Gene rearrangements in Ewing's sarcoma. Cancer Invest 1996; 14:83.
  9. Llombart-Bosch A, Carda C, Peydro-Olaya A, et al. Soft tissue Ewing's sarcoma. Characterization in established cultures and xenografts with evidence of a neuroectodermic phenotype. Cancer 1990; 66:2589.
  10. Thiele CJ. Biology of pediatric peripheral neuroectodermal tumors. Cancer Metastasis Rev 1991; 10:311.
  11. O'Regan S, Diebler MF, Meunier FM, Vyas S. A Ewing's sarcoma cell line showing some, but not all, of the traits of a cholinergic neuron. J Neurochem 1995; 64:69.
  12. Lawlor ER, Lim JF, Tao W, et al. The Ewing tumor family of peripheral primitive neuroectodermal tumors expresses human gastrin-releasing peptide. Cancer Res 1998; 58:2469.
  13. Tirode F, Laud-Duval K, Prieur A, et al. Mesenchymal stem cell features of Ewing tumors. Cancer Cell 2007; 11:421.
  14. Bleyer A, O’Leary M, Barr R, Ries LAG. Cancer Epidemiology in Older Adolescents and Young Adults 15 to 29 Years of Age, Including SEER Incidence and Survival: 1975-2000. NIH Pub. No. 06-5767, National Cancer Institute, Bethesda, MD 2006.
  15. Jürgens H, Exner U, Gadner H, et al. Multidisciplinary treatment of primary Ewing's sarcoma of bone. A 6-year experience of a European Cooperative Trial. Cancer 1988; 61:23.
  16. Grier H, Krailo M, Link M, et al. Improved outcome in non-metastatic Ewing's sarcoma (EWS) and PNET of bone with the addition of ifosfamide (I) and etoposide (E) to vincristine (V), Adriamycin (Ad), cyclophosphamide (C) and actinomycin (A): A Children's Cancer Group (CCG) and Pediatric Oncology Group (POG) report (abstract). Proc Am Soc Clin Oncol 1994; 13:421.
  17. http://seer.cancer.gov/publications/childhood/introduction.pdf (Accessed on May 31, 2011).
  18. Stiller CA, Bielack SS, Jundt G, Steliarova-Foucher E. Bone tumours in European children and adolescents, 1978-1997. Report from the Automated Childhood Cancer Information System project. Eur J Cancer 2006; 42:2124.
  19. Birch JM, Alston RD, Kelsey AM, et al. Classification and incidence of cancers in adolescents and young adults in England 1979-1997. Br J Cancer 2002; 87:1267.
  20. Gurney JG, Swensen AR, Bulterys M. Malignant Bone Tumors. In: Cancer Incidence and Survival among Children and Adolescents: United States SEER Program 1975-1995, Ries LA, Smith MA, Gurney JG, et al (Eds), NCI, SEER Program, Bethesda, MD 1999.
  21. Glass AG, Fraumeni JF Jr. Epidemiology of bone cancer in children. J Natl Cancer Inst 1970; 44:187.
  22. Miller RW. Contrasting epidemiology of childhood osteosarcoma, Ewing's tumor, and rhabdomyosarcoma. Natl Cancer Inst Monogr 1981; :9.
  23. Fraumeni JF Jr, Glass AG. Rarity of Ewing's sarcoma among U.S. Negro children. Lancet 1970; 1:366.
  24. Parkin DM, Stiller CA, Nectoux J. International variations in the incidence of childhood bone tumours. Int J Cancer 1993; 53:371.
  25. Jawad MU, Cheung MC, Min ES, et al. Ewing sarcoma demonstrates racial disparities in incidence-related and sex-related differences in outcome: an analysis of 1631 cases from the SEER database, 1973-2005. Cancer 2009; 115:3526.
  26. Zucman-Rossi J, Batzer MA, Stoneking M, et al. Interethnic polymorphism of EWS intron 6: genome plasticity mediated by Alu retroposition and recombination. Hum Genet 1997; 99:357.
  27. Postel-Vinay S, Véron AS, Tirode F, et al. Common variants near TARDBP and EGR2 are associated with susceptibility to Ewing sarcoma. Nat Genet 2012; 44:323.
  28. Hartley AL, Birch JM, Blair V, et al. Cancer incidence in the families of children with Ewing's tumor. J Natl Cancer Inst 1991; 83:955.
  29. Buckley JD, Pendergrass TW, Buckley CM, et al. Epidemiology of osteosarcoma and Ewing's sarcoma in childhood: a study of 305 cases by the Children's Cancer Group. Cancer 1998; 83:1440.
  30. McKeen EA, Hanson MR, Mulvihill JJ, Glaubiger DL. Birth defects with Ewing's sarcoma. N Engl J Med 1983; 309:1522.
  31. Novakovic B, Goldstein AM, Wexler LH, Tucker MA. Increased risk of neuroectodermal tumors and stomach cancer in relatives of patients with Ewing's sarcoma family of tumors. J Natl Cancer Inst 1994; 86:1702.
  32. Zhang J, Walsh MF, Wu G, et al. Germline Mutations in Predisposition Genes in Pediatric Cancer. N Engl J Med 2015; 373:2336.
  33. Yamamoto T, Wakabayashi T. Bone tumors among the atomic bomb survivors of Hiroshima and Nagasaki. Acta Pathol Jpn 1969; 19:201.
  34. Tucker MA, D'Angio GJ, Boice JD Jr, et al. Bone sarcomas linked to radiotherapy and chemotherapy in children. N Engl J Med 1987; 317:588.
  35. Spunt SL, Rodriguez-Galindo C, Fuller CE, et al. Ewing sarcoma-family tumors that arise after treatment of primary childhood cancer. Cancer 2006; 107:201.
  36. Valery PC, Williams G, Sleigh AC, et al. Parental occupation and Ewing's sarcoma: pooled and meta-analysis. Int J Cancer 2005; 115:799.
  37. Cope JU, Tsokos M, Helman LJ, et al. Inguinal hernia in patients with Ewing sarcoma: a clue to etiology. Med Pediatr Oncol 2000; 34:195.
  38. Holly EA, Aston DA, Ahn DK, Kristiansen JJ. Ewing's bone sarcoma, paternal occupational exposure, and other factors. Am J Epidemiol 1992; 135:122.
  39. Winn DM, Li FP, Robison LL, et al. A case-control study of the etiology of Ewing's sarcoma. Cancer Epidemiol Biomarkers Prev 1992; 1:525.
  40. Valery PC, McWhirter W, Sleigh A, et al. A national case-control study of Ewing's sarcoma family of tumours in Australia. Int J Cancer 2003; 105:825.
  41. Lipinski M, Braham K, Philip I, et al. Neuroectoderm-associated antigens on Ewing's sarcoma cell lines. Cancer Res 1987; 47:183.
  42. McKeon C, Thiele CJ, Ross RA, et al. Indistinguishable patterns of protooncogene expression in two distinct but closely related tumors: Ewing's sarcoma and neuroepithelioma. Cancer Res 1988; 48:4307.
  43. Ginsberg, JP, Woo, SY, Hicks, MJ, Horowitz, ME. Ewing's sarcoma family of tumors: Ewing's sarcoma of bone and soft tissue and the peripheral primitive neuroectodermal tumors. In: Priniciples and Practice of Pediatric Oncology, 4th ed, Pizzo, PA, Poplack, DG (Eds), Lippincott, Williams and Wilkins, Philadelphia, 2002.
  44. Torchia EC, Jaishankar S, Baker SJ. Ewing tumor fusion proteins block the differentiation of pluripotent marrow stromal cells. Cancer Res 2003; 63:3464.
  45. de Alava E, Gerald WL. Molecular biology of the Ewing's sarcoma/primitive neuroectodermal tumor family. J Clin Oncol 2000; 18:204.
  46. Dorfman H, Czerniak B. Bone Tumors, CV Mosby, St. Louis 1997. p.607.
  47. Fellinger EJ, Garin-Chesa P, Triche TJ, et al. Immunohistochemical analysis of Ewing's sarcoma cell surface antigen p30/32MIC2. Am J Pathol 1991; 139:317.
  48. Weidner N, Tjoe J. 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 1994; 18:486.
  49. Yoshida A, Sekine S, Tsuta K, et al. NKX2.2 is a useful immunohistochemical marker for Ewing sarcoma. Am J Surg Pathol 2012; 36:993.
  50. Delattre O, Zucman J, Plougastel B, et al. Gene fusion with an ETS DNA-binding domain caused by chromosome translocation in human tumours. Nature 1992; 359:162.
  51. Zucman J, Delattre O, Desmaze C, et al. Cloning and characterization of the Ewing's sarcoma and peripheral neuroepithelioma t(11;22) translocation breakpoints. Genes Chromosomes Cancer 1992; 5:271.
  52. Meier VS, Kühne T, Jundt G, Gudat F. Molecular diagnosis of Ewing tumors: improved detection of EWS-FLI-1 and EWS-ERG chimeric transcripts and rapid determination of exon combinations. Diagn Mol Pathol 1998; 7:29.
  53. Dagher R, Pham TA, Sorbara L, et al. Molecular confirmation of Ewing sarcoma. J Pediatr Hematol Oncol 2001; 23:221.
  54. Ohno T, Ouchida M, Lee L, et al. The EWS gene, involved in Ewing family of tumors, malignant melanoma of soft parts and desmoplastic small round cell tumors, codes for an RNA binding protein with novel regulatory domains. Oncogene 1994; 9:3087.
  55. Burd CG, Dreyfuss G. Conserved structures and diversity of functions of RNA-binding proteins. Science 1994; 265:615.
  56. Hromas R, Klemsz M. The ETS oncogene family in development, proliferation and neoplasia. Int J Hematol 1994; 59:257.
  57. Wasylyk B, Hahn SL, Giovane A. The Ets family of transcription factors. Eur J Biochem 1993; 211:7.
  58. Jeon IS, Davis JN, Braun BS, et al. A variant Ewing's sarcoma translocation (7;22) fuses the EWS gene to the ETS gene ETV1. Oncogene 1995; 10:1229.
  59. Peter M, Couturier J, Pacquement H, et al. A new member of the ETS family fused to EWS in Ewing tumors. Oncogene 1997; 14:1159.
  60. Sorensen PH, Lessnick SL, Lopez-Terrada D, et al. A second Ewing's sarcoma translocation, t(21;22), fuses the EWS gene to another ETS-family transcription factor, ERG. Nat Genet 1994; 6:146.
  61. Urano F, Umezawa A, Yabe H, et al. Molecular analysis of Ewing's sarcoma: another fusion gene, EWS-E1AF, available for diagnosis. Jpn J Cancer Res 1998; 89:703.
  62. Bell RS, Wunder J, Andrulis I. Molecular alterations in bone and soft-tissue sarcoma. Can J Surg 1999; 42:259.
  63. Obata K, Hiraga H, Nojima T, et al. Molecular characterization of the genomic breakpoint junction in a t(11;22) translocation in Ewing sarcoma. Genes Chromosomes Cancer 1999; 25:6.
  64. Ng TL, O'Sullivan MJ, Pallen CJ, et al. Ewing sarcoma with novel translocation t(2;16) producing an in-frame fusion of FUS and FEV. J Mol Diagn 2007; 9:459.
  65. Shing DC, McMullan DJ, Roberts P, et al. FUS/ERG gene fusions in Ewing's tumors. Cancer Res 2003; 63:4568.
  66. Zucman J, Delattre O, Desmaze C, et al. EWS and ATF-1 gene fusion induced by t(12;22) translocation in malignant melanoma of soft parts. Nat Genet 1993; 4:341.
  67. Panagopoulos I, Höglund M, Mertens F, et al. Fusion of the EWS and CHOP genes in myxoid liposarcoma. Oncogene 1996; 12:489.
  68. Rabbitts TH, Forster A, Larson R, Nathan P. Fusion of the dominant negative transcription regulator CHOP with a novel gene FUS by translocation t(12;16) in malignant liposarcoma. Nat Genet 1993; 4:175.
  69. Hattinger CM, Rumpler S, Strehl S, et al. Prognostic impact of deletions at 1p36 and numerical aberrations in Ewing tumors. Genes Chromosomes Cancer 1999; 24:243.
  70. Armengol G, Tarkkanen M, Virolainen M, et al. Recurrent gains of 1q, 8 and 12 in the Ewing family of tumours by comparative genomic hybridization. Br J Cancer 1997; 75:1403.
  71. Savola S, Klami A, Tripathi A, et al. Combined use of expression and CGH arrays pinpoints novel candidate genes in Ewing sarcoma family of tumors. BMC Cancer 2009; 9:17.
  72. Kovar H, Auinger A, Jug G, et al. Narrow spectrum of infrequent p53 mutations and absence of MDM2 amplification in Ewing tumours. Oncogene 1993; 8:2683.
  73. Kovar H, Jug G, Aryee DN, et al. 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 1997; 15:2225.
  74. Huang HY, Illei PB, Zhao Z, et al. Ewing sarcomas with p53 mutation or p16/p14ARF homozygous deletion: a highly lethal subset associated with poor chemoresponse. J Clin Oncol 2005; 23:548.
  75. Tirode F, Surdez D, Ma X, et al. Genomic landscape of Ewing sarcoma defines an aggressive subtype with co-association of STAG2 and TP53 mutations. Cancer Discov 2014; 4:1342.
  76. Crompton BD, Stewart C, Taylor-Weiner A, et al. The genomic landscape of pediatric Ewing sarcoma. Cancer Discov 2014; 4:1326.
  77. Brohl AS, Solomon DA, Chang W, et al. The genomic landscape of the Ewing Sarcoma family of tumors reveals recurrent STAG2 mutation. PLoS Genet 2014; 10:e1004475.
  78. Solomon DA, Kim T, Diaz-Martinez LA, et al. Mutational inactivation of STAG2 causes aneuploidy in human cancer. Science 2011; 333:1039.
  79. Lerman DM, Monument MJ, McIlvaine E, et al. Tumoral TP53 and/or CDKN2A alterations are not reliable prognostic biomarkers in patients with localized Ewing sarcoma: a report from the Children's Oncology Group. Pediatr Blood Cancer 2015; 62:759.
  80. May WA, Lessnick SL, Braun BS, et al. The Ewing's sarcoma EWS/FLI-1 fusion gene encodes a more potent transcriptional activator and is a more powerful transforming gene than FLI-1. Mol Cell Biol 1993; 13:7393.
  81. Herrero-Martín D, Osuna D, Ordóñez JL, et al. Stable interference of EWS-FLI1 in an Ewing sarcoma cell line impairs IGF-1/IGF-1R signalling and reveals TOPK as a new target. Br J Cancer 2009; 101:80.
  82. Tirado OM, Mateo-Lozano S, Villar J, et al. Caveolin-1 (CAV1) is a target of EWS/FLI-1 and a key determinant of the oncogenic phenotype and tumorigenicity of Ewing's sarcoma cells. Cancer Res 2006; 66:9937.
  83. Arvand A, Denny CT. Biology of EWS/ETS fusions in Ewing's family tumors. Oncogene 2001; 20:5747.
  84. Ohno T, Rao VN, Reddy ES. EWS/Fli-1 chimeric protein is a transcriptional activator. Cancer Res 1993; 53:5859.
  85. Bailly RA, Bosselut R, Zucman J, et al. DNA-binding and transcriptional activation properties of the EWS-FLI-1 fusion protein resulting from the t(11;22) translocation in Ewing sarcoma. Mol Cell Biol 1994; 14:3230.
  86. Kinsey M, Smith R, Iyer AK, et al. EWS/FLI and its downstream target NR0B1 interact directly to modulate transcription and oncogenesis in Ewing's sarcoma. Cancer Res 2009; 69:9047.
  87. May WA, Gishizky ML, Lessnick SL, et al. Ewing sarcoma 11;22 translocation produces a chimeric transcription factor that requires the DNA-binding domain encoded by FLI1 for transformation. Proc Natl Acad Sci U S A 1993; 90:5752.
  88. Toretsky JA, Kalebic T, Blakesley V, et al. The insulin-like growth factor-I receptor is required for EWS/FLI-1 transformation of fibroblasts. J Biol Chem 1997; 272:30822.
  89. Toretsky JA, Steinberg SM, Thakar M, et al. Insulin-like growth factor type 1 (IGF-1) and IGF binding protein-3 in patients with Ewing sarcoma family of tumors. Cancer 2001; 92:2941.
  90. Rodon J, DeSantos V, Ferry RJ Jr, Kurzrock R. Early drug development of inhibitors of the insulin-like growth factor-I receptor pathway: lessons from the first clinical trials. Mol Cancer Ther 2008; 7:2575.
  91. Nakatani F, Tanaka K, Sakimura R, et al. Identification of p21WAF1/CIP1 as a direct target of EWS-Fli1 oncogenic fusion protein. J Biol Chem 2003; 278:15105.
  92. Tanaka K, Iwakuma T, Harimaya K, et al. EWS-Fli1 antisense oligodeoxynucleotide inhibits proliferation of human Ewing's sarcoma and primitive neuroectodermal tumor cells. J Clin Invest 1997; 99:239.
  93. Toretsky JA, Connell Y, Neckers L, Bhat NK. Inhibition of EWS-FLI-1 fusion protein with antisense oligodeoxynucleotides. J Neurooncol 1997; 31:9.
  94. Kinsey M, Smith R, Lessnick SL. NR0B1 is required for the oncogenic phenotype mediated by EWS/FLI in Ewing's sarcoma. Mol Cancer Res 2006; 4:851.
  95. Prieur A, Tirode F, Cohen P, Delattre O. EWS/FLI-1 silencing and gene profiling of Ewing cells reveal downstream oncogenic pathways and a crucial role for repression of insulin-like growth factor binding protein 3. Mol Cell Biol 2004; 24:7275.
  96. Smith R, Owen LA, Trem DJ, et al. Expression profiling of EWS/FLI identifies NKX2.2 as a critical target gene in Ewing's sarcoma. Cancer Cell 2006; 9:405.
  97. Erkizan HV, Kong Y, Merchant M, et al. A small molecule blocking oncogenic protein EWS-FLI1 interaction with RNA helicase A inhibits growth of Ewing's sarcoma. Nat Med 2009; 15:750.
  98. Kovar H, Aryee DN, Jug G, et al. EWS/FLI-1 antagonists induce growth inhibition of Ewing tumor cells in vitro. Cell Growth Differ 1996; 7:429.
  99. Hancock JD, Lessnick SL. A transcriptional profiling meta-analysis reveals a core EWS-FLI gene expression signature. Cell Cycle 2008; 7:250.
  100. Kauer M, Ban J, Kofler R, et al. A molecular function map of Ewing's sarcoma. PLoS One 2009; 4:e5415.
  101. Luo W, Gangwal K, Sankar S, et al. GSTM4 is a microsatellite-containing EWS/FLI target involved in Ewing's sarcoma oncogenesis and therapeutic resistance. Oncogene 2009; 28:4126.
  102. Owen LA, Kowalewski AA, Lessnick SL. EWS/FLI mediates transcriptional repression via NKX2.2 during oncogenic transformation in Ewing's sarcoma. PLoS One 2008; 3:e1965.
  103. Yi H, Fujimura Y, Ouchida M, et al. Inhibition of apoptosis by normal and aberrant Fli-1 and erg proteins involved in human solid tumors and leukemias. Oncogene 1997; 14:1259.
  104. Takahashi A, Higashino F, Aoyagi M, et al. EWS/ETS fusions activate telomerase in Ewing's tumors. Cancer Res 2003; 63:8338.
  105. Italiano A, Di Mauro I, Rapp J, et al. Clinical effect of molecular methods in sarcoma diagnosis (GENSARC): a prospective, multicentre, observational study. Lancet Oncol 2016; 17:532.
  106. Diffin F, Porter H, Mott MG, et al. Rapid and specific diagnosis of t(11;22) translocation in paediatric Ewing's sarcoma and primitive neuroectodermal tumours using RNA-PCR. J Clin Pathol 1994; 47:562.
  107. Downing JR, Head DR, Parham DM, et al. Detection of the (11;22)(q24;q12) translocation of Ewing's sarcoma and peripheral neuroectodermal tumor by reverse transcription polymerase chain reaction. Am J Pathol 1993; 143:1294.
  108. Taylor C, Patel K, Jones T, et al. Diagnosis of Ewing's sarcoma and peripheral neuroectodermal tumour based on the detection of t(11;22) using fluorescence in situ hybridisation. Br J Cancer 1993; 67:128.
  109. Ladanyi M. The emerging molecular genetics of sarcoma translocations. Diagn Mol Pathol 1995; 4:162.
  110. de Alava E, Kawai A, Healey JH, et al. EWS-FLI1 fusion transcript structure is an independent determinant of prognosis in Ewing's sarcoma. J Clin Oncol 1998; 16:1248.
  111. Zoubek A, Dockhorn-Dworniczak B, Delattre O, et al. Does expression of different EWS chimeric transcripts define clinically distinct risk groups of Ewing tumor patients? J Clin Oncol 1996; 14:1245.
  112. van Doorninck JA, Ji L, Schaub B, et al. Current treatment protocols have eliminated the prognostic advantage of type 1 fusions in Ewing sarcoma: a report from the Children's Oncology Group. J Clin Oncol 2010; 28:1989.
  113. Le Deley MC, Delattre O, Schaefer KL, et al. Impact of EWS-ETS fusion type on disease progression in Ewing's sarcoma/peripheral primitive neuroectodermal tumor: prospective results from the cooperative Euro-E.W.I.N.G. 99 trial. J Clin Oncol 2010; 28:1982.
  114. Athale UH, Shurtleff SA, Jenkins JJ, et al. Use of reverse transcriptase polymerase chain reaction for diagnosis and staging of alveolar rhabdomyosarcoma, Ewing sarcoma family of tumors, and desmoplastic small round cell tumor. J Pediatr Hematol Oncol 2001; 23:99.
  115. West DC, Grier HE, Swallow MM, et al. Detection of circulating tumor cells in patients with Ewing's sarcoma and peripheral primitive neuroectodermal tumor. J Clin Oncol 1997; 15:583.
  116. de Alava E, Lozano MD, Patiño A, et al. Ewing family tumors: potential prognostic value of reverse-transcriptase polymerase chain reaction detection of minimal residual disease in peripheral blood samples. Diagn Mol Pathol 1998; 7:152.
  117. Zoubek A, Ladenstein R, Windhager R, et al. Predictive potential of testing for bone marrow involvement in Ewing tumor patients by RT-PCR: a preliminary evaluation. Int J Cancer 1998; 79:56.
  118. Fagnou C, Michon J, Peter M, et al. Presence of tumor cells in bone marrow but not in blood is associated with adverse prognosis in patients with Ewing's tumor. Société Française d'Oncologie Pédiatrique. J Clin Oncol 1998; 16:1707.