Sclerosing epithelioid fibrosarcoma (SEF): rare, malignant fibroblastic neoplasm characterized by epithelioid fibroblasts arranged in cords and nests and embedded in dense, sclerotic, hyalinized stroma
Subset of SEFs are related morphologically and molecularly to low-grade fibromyxoid sarcoma
IMAGING
General Features
Radiographic Findings
CT Findings
MR 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
Murshed KA et al: Sclerosing epithelioid fibrosarcoma: in-depth review of a genetically heterogeneous tumor. APMIS. 129(8):455-60, 2021
Warmke LM et al: Sclerosing epithelioid fibrosarcoma: a distinct sarcoma with aggressive features. Am J Surg Pathol. 45(3):317-28, 2021
Davis JL et al: Sclerosing epithelioid fibrosarcoma. In Fletcher CDM et al: WHO Classification of Tumours: Soft Tissue and Bone Tumours. 5th ed. IARC Press. 130-2, 2020
Yahyaoui M et al: Sclerosing epithelioid fibrosarcoma: rare and serious. Pan Afr Med J. 36:131, 2020
Righi A et al: Sclerosing epithelioid fibrosarcoma of the thigh: report of two cases with synchronous bone metastases. Virchows Arch. 467(3):339-44, 2015
Kransdorf MJ et al: Fibrous and fibrohistiocytic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Lippincott Williams & Wilkins. 325-7, 2014
Luo Y et al: ¹⁸F-fluorodeoxyglucose PET/CT features and correlations with histopathologic characteristics in sclerosing epithelioid fibrosarcoma. Int J Clin Exp Pathol. 7(10):7278-85, 2014
Ossendorf C et al: Sclerosing epithelioid fibrosarcoma: case presentation and a systematic review. Clin Orthop Relat Res. 466(6):1485-91, 2008
Weiss SW et al: Fibrosarcoma. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 310-2, 2008
Meis-Kindblom JM et al: Sclerosing epithelioid fibrosarcoma. In Fletcher CDM et al: WHO Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press. 106-7, 2002
Christensen DR et al: Sclerosing epithelioid fibrosarcoma: short T2 on MR imaging. Skeletal Radiol. 26(10):619-21, 1997
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
TERMINOLOGY
Definitions
Sclerosing epithelioid fibrosarcoma (SEF): rare, malignant fibroblastic neoplasm characterized by epithelioid fibroblasts arranged in cords and nests and embedded in dense, sclerotic, hyalinized stroma
Subset of SEFs are related morphologically and molecularly to low-grade fibromyxoid sarcoma
IMAGING
General Features
Radiographic Findings
CT Findings
MR 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
Murshed KA et al: Sclerosing epithelioid fibrosarcoma: in-depth review of a genetically heterogeneous tumor. APMIS. 129(8):455-60, 2021
Warmke LM et al: Sclerosing epithelioid fibrosarcoma: a distinct sarcoma with aggressive features. Am J Surg Pathol. 45(3):317-28, 2021
Davis JL et al: Sclerosing epithelioid fibrosarcoma. In Fletcher CDM et al: WHO Classification of Tumours: Soft Tissue and Bone Tumours. 5th ed. IARC Press. 130-2, 2020
Yahyaoui M et al: Sclerosing epithelioid fibrosarcoma: rare and serious. Pan Afr Med J. 36:131, 2020
Righi A et al: Sclerosing epithelioid fibrosarcoma of the thigh: report of two cases with synchronous bone metastases. Virchows Arch. 467(3):339-44, 2015
Kransdorf MJ et al: Fibrous and fibrohistiocytic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Lippincott Williams & Wilkins. 325-7, 2014
Luo Y et al: ¹⁸F-fluorodeoxyglucose PET/CT features and correlations with histopathologic characteristics in sclerosing epithelioid fibrosarcoma. Int J Clin Exp Pathol. 7(10):7278-85, 2014
Ossendorf C et al: Sclerosing epithelioid fibrosarcoma: case presentation and a systematic review. Clin Orthop Relat Res. 466(6):1485-91, 2008
Weiss SW et al: Fibrosarcoma. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 310-2, 2008
Meis-Kindblom JM et al: Sclerosing epithelioid fibrosarcoma. In Fletcher CDM et al: WHO Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press. 106-7, 2002
Christensen DR et al: Sclerosing epithelioid fibrosarcoma: short T2 on MR imaging. Skeletal Radiol. 26(10):619-21, 1997
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