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Undifferentiated Pleomorphic Sarcoma
Catherine C. Roberts, MD; B.J. Manaster, MD, PhD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Malignant fibrous histiocytoma (MFH), storiform or fibroblastic type; malignant fibrous xanthoma; atypical fibroxanthoma (when involving skin); fibroxanthosarcoma
            • Definitions

              • Term undifferentiated high-grade pleomorphic sarcoma is used synonymously with pleomorphic malignant fibrous histiocytoma
                • Terminology updated by World Health Organization in 2002 due to lack of histiocytic differentiation
                • Formerly considered common, distinct sarcoma subtype but now is diagnosis of exclusion
                • Terminology usage varies by institution

            IMAGING

            • General Features

              • Imaging Recommendations

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Ultrasonographic Findings

                        • Nuclear Medicine Findings

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Delisca GO et al: MFH and high-grade undifferentiated pleomorphic sarcoma-what's in a name? J Surg Oncol. 111(2):173-7, 2015
                                              2. Diaz-Beveridge R et al: Primary mesenteric undifferentiated pleomorphic sarcoma masquerading as a colon carcinoma: a case report and review of the literature. Case Rep Oncol Med. 2015:532656, 2015
                                              3. Dineen SP et al: Radiation-associated undifferentiated pleomorphic sarcoma is associated with worse clinical outcomes than sporadic lesions. Ann Surg Oncol. 22(12):3913-20, 2015
                                              4. Hofvander J et al: Recurrent PRDM10 gene fusions in undifferentiated pleomorphic sarcoma. Clin Cancer Res. 21(4):864-9, 2015
                                              5. Schmitz K et al: MET gene copy number alterations and expression of MET and hepatocyte growth factor are potential biomarkers in angiosarcomas and undifferentiated pleomorphic sarcomas. PLoS One. 10(4):e0120079, 2015
                                              6. Weiss SW et al: Malignant fibrous histiocytoma (pleomorphic undifferentiated sarcoma). In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Philadelphia: Elsevier. 406-14, 2008
                                              7. Kransdorf MJ et al: Malignant fibrous and fibrohistiocytic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Philadelphia: Lippincott Williams & Wilkins. 276-93, 2006
                                              8. Fletcher CDM et al: Pleomorphic malignant fibrous histiocytoma/Undifferentiated high grade pleomorphic sarcoma. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press. 120-2, 2002
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Pathology

                                                    • Clinical Issues

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Synonyms

                                                          • Malignant fibrous histiocytoma (MFH), storiform or fibroblastic type; malignant fibrous xanthoma; atypical fibroxanthoma (when involving skin); fibroxanthosarcoma
                                                        • Definitions

                                                          • Term undifferentiated high-grade pleomorphic sarcoma is used synonymously with pleomorphic malignant fibrous histiocytoma
                                                            • Terminology updated by World Health Organization in 2002 due to lack of histiocytic differentiation
                                                            • Formerly considered common, distinct sarcoma subtype but now is diagnosis of exclusion
                                                            • Terminology usage varies by institution

                                                        IMAGING

                                                        • General Features

                                                          • Imaging Recommendations

                                                            • Radiographic Findings

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Ultrasonographic Findings

                                                                    • Nuclear Medicine Findings

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Staging, Grading, & Classification

                                                                            • Gross Pathologic & Surgical Features

                                                                              • Microscopic Features

                                                                                CLINICAL ISSUES

                                                                                • Presentation

                                                                                  • Demographics

                                                                                    • Natural History & Prognosis

                                                                                      • Treatment

                                                                                        DIAGNOSTIC CHECKLIST

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Delisca GO et al: MFH and high-grade undifferentiated pleomorphic sarcoma-what's in a name? J Surg Oncol. 111(2):173-7, 2015
                                                                                          2. Diaz-Beveridge R et al: Primary mesenteric undifferentiated pleomorphic sarcoma masquerading as a colon carcinoma: a case report and review of the literature. Case Rep Oncol Med. 2015:532656, 2015
                                                                                          3. Dineen SP et al: Radiation-associated undifferentiated pleomorphic sarcoma is associated with worse clinical outcomes than sporadic lesions. Ann Surg Oncol. 22(12):3913-20, 2015
                                                                                          4. Hofvander J et al: Recurrent PRDM10 gene fusions in undifferentiated pleomorphic sarcoma. Clin Cancer Res. 21(4):864-9, 2015
                                                                                          5. Schmitz K et al: MET gene copy number alterations and expression of MET and hepatocyte growth factor are potential biomarkers in angiosarcomas and undifferentiated pleomorphic sarcomas. PLoS One. 10(4):e0120079, 2015
                                                                                          6. Weiss SW et al: Malignant fibrous histiocytoma (pleomorphic undifferentiated sarcoma). In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Philadelphia: Elsevier. 406-14, 2008
                                                                                          7. Kransdorf MJ et al: Malignant fibrous and fibrohistiocytic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Philadelphia: Lippincott Williams & Wilkins. 276-93, 2006
                                                                                          8. Fletcher CDM et al: Pleomorphic malignant fibrous histiocytoma/Undifferentiated high grade pleomorphic sarcoma. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press. 120-2, 2002