link
Bookmarks
Low-Grade Fibromyxoid Sarcoma
Eric Walker, MD, MHA, FACR, FACHE; Catherine C. Roberts, MD
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Subscribe
0
3
3
0

KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Fibrosarcoma, fibromyxoid type; hyalinizing spindle cell tumor with giant rosettes
          • Definitions

            • Malignant fibroblastic neoplasm characterized by alternating collagenous and myxoid areas, deceptively bland spindle cells with whirling growth pattern, and arcades of small blood vessels
            • These lesions consistently have FUS::CREB3L2 or FUS::CREB3L1 gene fusions

          IMAGING

          • General Features

            • Imaging Recommendations

              • CT Findings

                • MR Findings

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Gross Pathologic & Surgical Features

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  Selected References

                                  1. Martínez-Trufero J et al: Uncommon and peculiar soft tissue sarcomas: multidisciplinary review and practical recommendations for diagnosis and treatment. Spanish Group for Sarcoma Research (GEIS - GROUP). Part I. Cancer Treat Rev. 99:102259, 2021
                                  2. Doyle LA et al: Low-grade fibromyxoid sarcoma. In Fletcher CDM et al: WHO Classification of Tumours: Soft Tissue and Bone Tumours. 5th ed. IARC Press. 127-9, 2020
                                  3. Kransdorf MJ et al: Fibrous and fibrohistiocytic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Lippincott Williams & Wilkins. 325, 2014
                                  4. Yamashita H et al: Intramuscular myxoma of the buttock mimicking low-grade fibromyxoid sarcoma: diagnostic usefulness of MUC4 expression. Skeletal Radiol. 42(10):1475-9, 2013
                                  5. Hwang S et al: Imaging features of low-grade fibromyxoid sarcoma (Evans tumor). Skeletal Radiol. 41(10):1263-72, 2012
                                  6. Evans HL: Low-grade fibromyxoid sarcoma: a clinicopathologic study of 33 cases with long-term follow-up. Am J Surg Pathol. 35(10):1450-62, 2011
                                  7. Weiss SW et al: Fibrosarcoma. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 316-25, 2008
                                  8. Folpe A et al: Low grade fibromyxoid sarcoma. In Fletcher CDM et al: WHO Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press. 104-5, 2002
                                  Related Anatomy
                                  Loading...
                                  Related Differential Diagnoses
                                  Loading...
                                  References
                                  Tables

                                  Tables

                                  KEY FACTS

                                  • Terminology

                                    • Imaging

                                      • Pathology

                                        • Clinical Issues

                                          TERMINOLOGY

                                          • Synonyms

                                            • Fibrosarcoma, fibromyxoid type; hyalinizing spindle cell tumor with giant rosettes
                                          • Definitions

                                            • Malignant fibroblastic neoplasm characterized by alternating collagenous and myxoid areas, deceptively bland spindle cells with whirling growth pattern, and arcades of small blood vessels
                                            • These lesions consistently have FUS::CREB3L2 or FUS::CREB3L1 gene fusions

                                          IMAGING

                                          • General Features

                                            • Imaging Recommendations

                                              • CT Findings

                                                • MR Findings

                                                  DIFFERENTIAL DIAGNOSIS

                                                    PATHOLOGY

                                                    • General Features

                                                      • Gross Pathologic & Surgical Features

                                                        • Microscopic Features

                                                          CLINICAL ISSUES

                                                          • Presentation

                                                            • Demographics

                                                              • Natural History & Prognosis

                                                                • Treatment

                                                                  Selected References

                                                                  1. Martínez-Trufero J et al: Uncommon and peculiar soft tissue sarcomas: multidisciplinary review and practical recommendations for diagnosis and treatment. Spanish Group for Sarcoma Research (GEIS - GROUP). Part I. Cancer Treat Rev. 99:102259, 2021
                                                                  2. Doyle LA et al: Low-grade fibromyxoid sarcoma. In Fletcher CDM et al: WHO Classification of Tumours: Soft Tissue and Bone Tumours. 5th ed. IARC Press. 127-9, 2020
                                                                  3. Kransdorf MJ et al: Fibrous and fibrohistiocytic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Lippincott Williams & Wilkins. 325, 2014
                                                                  4. Yamashita H et al: Intramuscular myxoma of the buttock mimicking low-grade fibromyxoid sarcoma: diagnostic usefulness of MUC4 expression. Skeletal Radiol. 42(10):1475-9, 2013
                                                                  5. Hwang S et al: Imaging features of low-grade fibromyxoid sarcoma (Evans tumor). Skeletal Radiol. 41(10):1263-72, 2012
                                                                  6. Evans HL: Low-grade fibromyxoid sarcoma: a clinicopathologic study of 33 cases with long-term follow-up. Am J Surg Pathol. 35(10):1450-62, 2011
                                                                  7. Weiss SW et al: Fibrosarcoma. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 316-25, 2008
                                                                  8. Folpe A et al: Low grade fibromyxoid sarcoma. In Fletcher CDM et al: WHO Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press. 104-5, 2002