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Undifferentiated Pleomorphic Sarcoma With Prominent Inflammation
Catherine C. Roberts, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Xanthomatous malignant fibrous histiocytoma, malignant xanthogranuloma, malignant fibrous xanthoma, xanthosarcoma, retroperitoneal xanthogranuloma
          • Definitions

            • Very rare malignant sarcoma composed of malignant and benign xanthomatous cells, atypical spindle cells, and both acute and chronic inflammatory cells

          IMAGING

          • General Features

            • Imaging Recommendations

              • Radiographic 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

                                        Selected References

                                        1. 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. 422-5, 2008
                                        2. 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. 279, 2006
                                        3. Hallor KH et al: Two genetic pathways, t(1;10) and amplification of 3p11-12, in myxoinflammatory fibroblastic sarcoma, haemosiderotic fibrolipomatous tumour, and morphologically similar lesions. J Pathol. 217(5):716-27, 2009
                                        4. Coindre JM et al: Inflammatory malignant fibrous histiocytomas and dedifferentiated liposarcomas: histological review, genomic profile, and MDM2 and CDK4 status favour a single entity. J Pathol. 203(3):822-30, 2004
                                        5. Coindre JM: Inflammatory malignant fibrous histiocytoma/Undifferentiated pleomorphic sarcoma with prominent inflammation. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press. 125-6, 2002
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Pathology

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Synonyms

                                                  • Xanthomatous malignant fibrous histiocytoma, malignant xanthogranuloma, malignant fibrous xanthoma, xanthosarcoma, retroperitoneal xanthogranuloma
                                                • Definitions

                                                  • Very rare malignant sarcoma composed of malignant and benign xanthomatous cells, atypical spindle cells, and both acute and chronic inflammatory cells

                                                IMAGING

                                                • General Features

                                                  • Imaging Recommendations

                                                    • Radiographic 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

                                                                              Selected References

                                                                              1. 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. 422-5, 2008
                                                                              2. 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. 279, 2006
                                                                              3. Hallor KH et al: Two genetic pathways, t(1;10) and amplification of 3p11-12, in myxoinflammatory fibroblastic sarcoma, haemosiderotic fibrolipomatous tumour, and morphologically similar lesions. J Pathol. 217(5):716-27, 2009
                                                                              4. Coindre JM et al: Inflammatory malignant fibrous histiocytomas and dedifferentiated liposarcomas: histological review, genomic profile, and MDM2 and CDK4 status favour a single entity. J Pathol. 203(3):822-30, 2004
                                                                              5. Coindre JM: Inflammatory malignant fibrous histiocytoma/Undifferentiated pleomorphic sarcoma with prominent inflammation. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press. 125-6, 2002