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Sclerosing Epithelioid Fibrosarcoma
Eric Walker, MD, MHA, FACR, FACHE; Stephanie A. Bernard, MD; Catherine C. Roberts, MD
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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

                                    1. Murshed KA et al: Sclerosing epithelioid fibrosarcoma: in-depth review of a genetically heterogeneous tumor. APMIS. 129(8):455-60, 2021
                                    2. Warmke LM et al: Sclerosing epithelioid fibrosarcoma: a distinct sarcoma with aggressive features. Am J Surg Pathol. 45(3):317-28, 2021
                                    3. 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
                                    4. Yahyaoui M et al: Sclerosing epithelioid fibrosarcoma: rare and serious. Pan Afr Med J. 36:131, 2020
                                    5. 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
                                    6. 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
                                    7. 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
                                    8. Ossendorf C et al: Sclerosing epithelioid fibrosarcoma: case presentation and a systematic review. Clin Orthop Relat Res. 466(6):1485-91, 2008
                                    9. Weiss SW et al: Fibrosarcoma. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 310-2, 2008
                                    10. 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
                                    11. 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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                                    Related Differential Diagnoses
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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

                                                                      1. Murshed KA et al: Sclerosing epithelioid fibrosarcoma: in-depth review of a genetically heterogeneous tumor. APMIS. 129(8):455-60, 2021
                                                                      2. Warmke LM et al: Sclerosing epithelioid fibrosarcoma: a distinct sarcoma with aggressive features. Am J Surg Pathol. 45(3):317-28, 2021
                                                                      3. 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
                                                                      4. Yahyaoui M et al: Sclerosing epithelioid fibrosarcoma: rare and serious. Pan Afr Med J. 36:131, 2020
                                                                      5. 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
                                                                      6. 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
                                                                      7. 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
                                                                      8. Ossendorf C et al: Sclerosing epithelioid fibrosarcoma: case presentation and a systematic review. Clin Orthop Relat Res. 466(6):1485-91, 2008
                                                                      9. Weiss SW et al: Fibrosarcoma. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 310-2, 2008
                                                                      10. 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
                                                                      11. Christensen DR et al: Sclerosing epithelioid fibrosarcoma: short T2 on MR imaging. Skeletal Radiol. 26(10):619-21, 1997