Key Points
• ES and PNET are part of the same spectrum of neoplastic disorders, irrespective of their localisation or cellular differentiation.
• ES/PNET occurs in young patients.
• On radiographs/CT, 37.5% are located in the axial skeleton and 62.5% in the peripheral skeleton.
• ES/PNET involving the bone is mostly mixed sclerotic-lytic. A spiculated periosteal reaction is most frequent.
• The most characteristic finding on MRI is the presence of a large soft tissue mass.
• At initial presentation, 20%–30% of patients have pulmonary and/or skeletal metastases.
• PET-CT is becoming standard of care for initial staging, in monitoring response to chemotherapy and detection of recurrence or new metastatic disease.
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References
Ambros IM, Ambros PF, Strehl S et al. (1991) MIC2 is a specific marker for Ewing’s sarcoma and peripheral primitive neuroectodermal tumors. Cancer 67:1886–1893
Angervall L, Enzinger FM (1975) Extraskeletal neoplasm resembling Ewing’s sarcoma. Cancer 36:240–251
Arush MW, Israel O, Postovsky S et al. (2007) Positron emission tomography/computed tomography with 18fluoro-deoxyglucose in the detection of local recurrence and distant metastases of pediatric sarcoma. Pediatr Blood Cancer 49:901–905
Askin FB, Rosai J, Sibley RK et al. (1979) Malignant small cell tumor of the thoracopulmonary region in childhood: a distinctive clinicopathologic entity of uncertain histogenenesis. Cancer 43:2438–2451
Bacci G, Balladelli A, Forni C et al. (2007) 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 109:780–786
Baker ND, Dorfman DM (1996) Ewing’s sacoma of the sacrum. Skeletal Radiol 25:302–304
Baldini EH, Demetri GD, Fletcher CD et al. (1999) Adults with Ewing’s sarcoma: primitive neuroectodermal tumor: adverse effect of older age and primary extraosseous disease on outcome. Ann Surg 230:79–86
Baraga JJ, Amrami KK, Swee RG (2001) Radiographic features of Ewing’s sarcoma of the bones of the hands and feet. Skeletal Radiol 30:121–126
Campanacci M (1999) Ewings’s sarcoma, primitive neuroectodermal tumor (PNET). In: Campanacci M (ed) Bone and soft tissue tumors, 2nd edn. Springer, Berlin Heidelberg New York, pp 653–682
Chow E, Mercant TE, Pappo A et al. (2000) Cutaneous and subcutaneous Ewing’s sarcoma: an indolent disease. Int J Radiat Oncol Biol Phys 46:433–438
Cotterill SJ, Ahrens S, Paulussen M et al. (2000) Prognostic factors in Ewing’s tumors of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing’s Sarcoma Study Group. J Clin Oncol 18:3108–3114
Craft A, Cotterill S, Malcolm A et al. (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 16:3628–3633
Daldrup-Link HE, Franzius C, Link TM et al. (2001) Whole-body MR imaging for detection of bone metastasis in children and young adults. AJR Am J Roentgenol 177:229–236
Delaney T, Hornicek FJ, Mankin HJ (2008) http://www.uptodate.com
Ferrari S, Bertoni F, Mercuri M et al. (2001) Ewing’s sarcoma of bone: relation between clinical characteristics and staging. Oncol Rep 8:553–556
Franzius C, Sciuk J, Daldrup-Link HE et al. (2000) FDG-PET for detection of osseous metastases from malignant primary bone tumours: comparison with bone scintigraphy. Eur J Nucl Med 27:1305–1311
Gyorke T, Zajic T, Lange A et al. (2006) Impact of FDG-PET for staging of Ewing sarcomas and primitive neuroectodermal tumours. Nucl Med Commun 27:17–24
Hanna SL, Fletcher BD, Kaste SC et al. (1994) Increased confidence of diagnosis of Ewing sarcoma using T2-weighted MR images. Magn Reson Imaging 12:559–568
Huang HY, Illei PB, Zhao Z et al. (2005) Ewing Sarcomas with p53 mutation or p16/p14ARF homozygous deletion: a highly lethal subset associated with poor chemoresponse. J Clin Oncol 23:548–558
Iagaru A, Chawla S, Menendez L et al. (2006) 18F-FDG PET and PET/CT for detection of pulmonary metastases from musculoskeletal sarcomas. Nucl Med Commun 27:785–802
Ilaslan H, Sundaram M, Unni KK et al. (2004) Primary Ewing’s sarcoma of the vertebral column. Skeletal Radiol 33:506–513
Jaffe R, Santamaria M, Yunis EJ et al. (1984) The neuroectoderal tumor of bone. Am J Surg Pathol 8:885–898
Jiya TU, Wuisman PI (2005) Long-term follow-up of 15 patients with nonmetastatic Ewing’s sarcoma and a skip lesion. Acta Orthop 76:899–903
Khong PL, Chan GC, Shek TW et al. (2002) Imaging of peripheral PNET: common and uncommon locations. Clin Radiol 57:272–277
Kransdorf MJ, Smith SE (2000) Lesions of unknown histogenesis: Langerhans cell histiocytosis and Ewing sarcoma. Semin Musculoskelet Radiol 4:113–125
Lee CS, Southey MC, Slater H et al. (1995) Primary cutaneous Ewing’s sarcoma/peripheral primitive neuroectodermal tumors in childhood. A molecular, cytogenetic, and immunohistochemical study. Diag Mol Pathol 4:174–181
Li W-Y, Brock P, Saunders DE (2005) Imaging characteristics of primary cranial Ewing sarcoma. Pediatr Radiol 35:612–618
Mar WA, Taljanovic MS, Bagatell R et al. (2008) Update on imaging and treatment of Ewing sarcoma family tumors: what the radiologist needs to know. J Comput Assist Tomogr 32:108–118
McCarville MB, Christie R, Daw NC et al. (2005) PET/CT in the evaluation of childhood sarcomas. AJR Am J Roentgenol 184:1293–1304
Miller SL, Hoffer FA, Reddick WE et al. (2001) Tumor volume or dynamic contrast-enhanced MRI for prediction of clinical outcome of Ewing sarcoma family of tumors. Pediatr Radiol 31:518–523
Moser RP Jr, Davis MJ, Gilkey FW et al. (1990) Primary Ewing sarcoma of rib. Radiographics 10:899–914
Oberlin O, Deley MC, Bui BN et al. (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 85:1646–1654
Parham DM, Hijazi Y, Steinberg SM et al. (1999) Neuroectodermal differentiation in Ewing’s sarcoma family of tumors does not predict tumor behavior. Human Pathol 30:911–918
Paulussen M, Fröhlich B, Jürgens H (2001) Ewing tumour: incidence, prognosis and treatment options. Paediatr Drugs 3:899–913
Peersman B, Vanhoenacker FM, Heyman S et al. (2007) Ewing’s sarcoma: imaging features. JBR-BTR 90:368–376
Reinus WR, Gilula LA (1984) Radiology of Ewing’s sarcoma: intergroup study (IESS). Radiographics 4:929–944
Resnick D, Kransdorf MJ (2005) Tumors and tumorlike diseases. In: Resnick D (ed) Bone and joint imaging, 3rd edn. Elsevier, Philadelphia, pp 1192–1198
Rud NP, Reiman HM, Pritchard DJ et al. (1989) Extraosseous Ewing’s sarcoma. A study of 42 cases. Cancer 64:1548–1553
Steens SC, Kroon HM, Taminiau AH et al. (2007) Nail-Patella syndrome associated with Ewing sarcoma. JBR-BTR 90:214–215
Szuhai K, Ijszenga M, Tanke HJ et al. (2006) Molecular cytogenetic characterization of four previously established and two newly established Ewing sarcoma cell lines. Cancer Genet Cytogenet 166:173–179
Van der Woude HJ, Bloem JL, Holscher HC et al. (1994) Monitoring the effect of chemotherapy in Ewing sarcoma of bone with MR imaging. Skeletal Radiol 23:493–500
Van der Woude HJ, Bloem JL, Hogendoorn PC et al. (1998) Preoperative evaluation and monitoring chemotherapy in patients with high-grade osteogenic and Ewing’s sarcoma: review of current imaging modalities. Skeletal Radiol 27:57–71
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Vanhoenacker, F., van Kerkhove, F., Peersman, B., Brys, P., de Schepper, A. (2009). Ewing Sarcoma/PNET Tumors. In: Davies, A., Sundaram, M., James, S. (eds) Imaging of Bone Tumors and Tumor-Like Lesions. Medical Radiology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-77984-1_17
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