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KEY FACTS
Imaging
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
- Extraskeletal Ewing sarcoma (EES)
- Small round cell sarcoma (SRCS)
Synonyms
- No longer recommended: peripheral neuroectodermal (PNET), peripheral neuroepithelioma, peripheral neuroblastoma, or Askin tumor
WHO Classification of Undifferentiated Small Round Cell Sarcomas
- Ewing sarcoma
- CIC-rearranged sarcoma
- Sarcoma with BCOR alterations
- Round cell sarcoma with EWSR1-non-ETS fusions: contains remainder of formerly Ewing-like undifferentiated sarcomas
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
Angiographic Findings
Nuclear Medicine Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
- Brady EJ et al: Imaging features and clinical course of undifferentiated round cell sarcomas with CIC-DUX4 and BCOR-CCNB3 translocations. Skeletal Radiol. 50(3):521-9, 2021
- Sirisena UDN et al: Imaging of bone and soft tissue BCOR-rearranged sarcoma. Skeletal Radiol. 50(7):1291-301, 2021
- Brčić I et al: Undifferentiated round cell sarcomas with CIC-DUX4 gene fusion: expanding the clinical spectrum. Pathology. 52(2):236-42, 2020
- Antonescu CR et al: Sarcomas with CIC-rearrangements are a distinct pathologic entity with aggressive outcome: a clinicopathologic and molecular study of 115 cases. Am J Surg Pathol. 41(7):941-9, 2017
- Huh J et al: Imaging features of primary tumors and metastatic patterns of the extraskeletal Ewing sarcoma family of tumors in adults: a 17-year experience at a single institution. Korean J Radiol. 16(4):783-90, 2015
- Murphey MD et al: From the radiologic pathology archives: Ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics. 33(3):803-31, 2013
- Javery O et al: A to Z of extraskeletal Ewing sarcoma family of tumors in adults: imaging features of primary disease, metastatic patterns, and treatment responses. AJR Am J Roentgenol. 197(6):W1015-22, 2011
- Bahk WJ et al: Primary cutaneous Ewing's sarcoma/primitive neuroectodermal tumor manifesting numerous small and huge ulcerated masses: its complete remission by chemotherapy and magnetic resonance imaging findings. Skeletal Radiol. 39(6):595-600, 2010
- Weiss SW et al: Ewing's sarcoma/PNET tumor family and related lesions. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 963-79, 2008
- Kransdorf MJ et al: Neurogenic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Lippincott Williams & Wilkins. 371-4, 2006
- Carvajal R et al: Ewing's sarcoma and primitive neuroectodermal family of tumors. Hematol Oncol Clin North Am. 19(3):501-25, vi-vii, 2005
- Christie DR et al: Diagnostic difficulties in extraosseous Ewing's sarcoma: a proposal for diagnostic criteria. Australas Radiol. 41(1):22-8, 1997
- Allam K et al: MR of primary extraosseous Ewing sarcoma. AJNR Am J Neuroradiol. 15(2):305-7, 1994
- Winer-Muram HT et al: Primitive neuroectodermal tumors of the chest wall (Askin tumors): CT and MR findings. AJR Am J Roentgenol. 161(2):265-8, 1993
- O'Keeffe F et al: Radiological features of extraskeletal Ewing sarcoma. Br J Radiol. 63(750):456-60, 1990
- Rose JS et al: Extraskeletal Ewing sarcoma with computed tomography correlation. Skeletal Radiol. 9(4):234-7, 1983
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References
Tables
Tables
KEY FACTS
Imaging
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
- Extraskeletal Ewing sarcoma (EES)
- Small round cell sarcoma (SRCS)
Synonyms
- No longer recommended: peripheral neuroectodermal (PNET), peripheral neuroepithelioma, peripheral neuroblastoma, or Askin tumor
WHO Classification of Undifferentiated Small Round Cell Sarcomas
- Ewing sarcoma
- CIC-rearranged sarcoma
- Sarcoma with BCOR alterations
- Round cell sarcoma with EWSR1-non-ETS fusions: contains remainder of formerly Ewing-like undifferentiated sarcomas
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
Angiographic Findings
Nuclear Medicine Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
- Brady EJ et al: Imaging features and clinical course of undifferentiated round cell sarcomas with CIC-DUX4 and BCOR-CCNB3 translocations. Skeletal Radiol. 50(3):521-9, 2021
- Sirisena UDN et al: Imaging of bone and soft tissue BCOR-rearranged sarcoma. Skeletal Radiol. 50(7):1291-301, 2021
- Brčić I et al: Undifferentiated round cell sarcomas with CIC-DUX4 gene fusion: expanding the clinical spectrum. Pathology. 52(2):236-42, 2020
- Antonescu CR et al: Sarcomas with CIC-rearrangements are a distinct pathologic entity with aggressive outcome: a clinicopathologic and molecular study of 115 cases. Am J Surg Pathol. 41(7):941-9, 2017
- Huh J et al: Imaging features of primary tumors and metastatic patterns of the extraskeletal Ewing sarcoma family of tumors in adults: a 17-year experience at a single institution. Korean J Radiol. 16(4):783-90, 2015
- Murphey MD et al: From the radiologic pathology archives: Ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics. 33(3):803-31, 2013
- Javery O et al: A to Z of extraskeletal Ewing sarcoma family of tumors in adults: imaging features of primary disease, metastatic patterns, and treatment responses. AJR Am J Roentgenol. 197(6):W1015-22, 2011
- Bahk WJ et al: Primary cutaneous Ewing's sarcoma/primitive neuroectodermal tumor manifesting numerous small and huge ulcerated masses: its complete remission by chemotherapy and magnetic resonance imaging findings. Skeletal Radiol. 39(6):595-600, 2010
- Weiss SW et al: Ewing's sarcoma/PNET tumor family and related lesions. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 963-79, 2008
- Kransdorf MJ et al: Neurogenic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Lippincott Williams & Wilkins. 371-4, 2006
- Carvajal R et al: Ewing's sarcoma and primitive neuroectodermal family of tumors. Hematol Oncol Clin North Am. 19(3):501-25, vi-vii, 2005
- Christie DR et al: Diagnostic difficulties in extraosseous Ewing's sarcoma: a proposal for diagnostic criteria. Australas Radiol. 41(1):22-8, 1997
- Allam K et al: MR of primary extraosseous Ewing sarcoma. AJNR Am J Neuroradiol. 15(2):305-7, 1994
- Winer-Muram HT et al: Primitive neuroectodermal tumors of the chest wall (Askin tumors): CT and MR findings. AJR Am J Roentgenol. 161(2):265-8, 1993
- O'Keeffe F et al: Radiological features of extraskeletal Ewing sarcoma. Br J Radiol. 63(750):456-60, 1990
- Rose JS et al: Extraskeletal Ewing sarcoma with computed tomography correlation. Skeletal Radiol. 9(4):234-7, 1983
