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Desmoid-Type Fibromatosis
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

          • Synonyms

            • Extraabdominal desmoid, desmoid tumor, aggressive fibromatosis, musculoaponeurotic fibromatosis, well-differentiated nonmetastasizing fibrosarcoma
          • Definitions

            • Locally aggressive, but nonmetastasizing, myofibroblastic neoplasm with infiltrative growth and high rate of local recurrence
              • Formerly histologically divided by location (extraabdominal, abdominal, intraabdominal)

          IMAGING

          • General Features

            • Imaging Recommendations

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Angiographic 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. Penel N et al: Desmoid-type fibromatosis: toward a holistic management. Curr Opin Oncol. 33(4):309-14, 2021
                                            2. Fritchie KJ et al: Desmoid fibromatosis. In Fletcher CDM et al: WHO Classification of Tumours: Soft Tissue and Bone Tumours. 5th ed. IARC Press. 93-5, 2020
                                            3. Napolitano A et al: Recent advances in desmoid tumor therapy. Cancers (Basel). 12(8), 2020
                                            4. Kransdorf MJ et al: Fibrous and fibrohistiocytic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Lippincott Williams & Wilkins. 265-86, 2014
                                            5. Lee CH et al: Lipomatosis of the sciatic nerve secondary to compression by a desmoid tumor. Skeletal Radiol. 42(12):1751-4, 2013
                                            6. Oweis Y et al: Extra-abdominal desmoid tumor with osseous involvement. Skeletal Radiol. 41(4):483-7, 2012
                                            7. Walker EA et al: Imaging features of superficial and deep fibromatoses in the adult population. Sarcoma. 2012:215810, 2012
                                            8. Walker EA et al: Magnetic resonance imaging of benign soft tissue neoplasms in adults. Radiol Clin North Am. 49(6):1197-217, vi, 2011
                                            9. Ilaslan H et al: Radiofrequency ablation: another treatment option for local control of desmoid tumors. Skeletal Radiol. 39(2):169-73, 2010
                                            10. Walker EA et al: Magnetic resonance imaging of soft-tissue masses. Semin Roentgenol. 45(4):277-97, 2010
                                            11. Murphey MD et al: From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation. Radiographics. 29(7):2143-73, 2009
                                            12. McDonald ES et al: Best cases from the AFIP: extraabdominal desmoid-type fibromatosis. Radiographics. 28(3):901-6, 2008
                                            13. Weiss SW et al: Fibromatoses. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 237-53, 2008
                                            14. Hosalkar HS et al: Desmoid tumors and current status of management. Orthop Clin North Am. 37(1):53-63, 2006
                                            15. Levy AD et al: From the archives of the AFIP: benign fibrous tumors and tumorlike lesions of the mesentery: radiologic-pathologic correlation. Radiographics. 26(1):245-64, 2006
                                            16. Azizi L et al: MRI features of mesenteric desmoid tumors in familial adenomatous polyposis. AJR Am J Roentgenol. 184(4):1128-35, 2005
                                            17. Goldblum J et al: Desmoid-type fibromatoses. In Fletcher CDM et al: WHO Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press. 83-4, 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

                                                      • Extraabdominal desmoid, desmoid tumor, aggressive fibromatosis, musculoaponeurotic fibromatosis, well-differentiated nonmetastasizing fibrosarcoma
                                                    • Definitions

                                                      • Locally aggressive, but nonmetastasizing, myofibroblastic neoplasm with infiltrative growth and high rate of local recurrence
                                                        • Formerly histologically divided by location (extraabdominal, abdominal, intraabdominal)

                                                    IMAGING

                                                    • General Features

                                                      • Imaging Recommendations

                                                        • Radiographic Findings

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic Findings

                                                                • Angiographic 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. Penel N et al: Desmoid-type fibromatosis: toward a holistic management. Curr Opin Oncol. 33(4):309-14, 2021
                                                                                      2. Fritchie KJ et al: Desmoid fibromatosis. In Fletcher CDM et al: WHO Classification of Tumours: Soft Tissue and Bone Tumours. 5th ed. IARC Press. 93-5, 2020
                                                                                      3. Napolitano A et al: Recent advances in desmoid tumor therapy. Cancers (Basel). 12(8), 2020
                                                                                      4. Kransdorf MJ et al: Fibrous and fibrohistiocytic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Lippincott Williams & Wilkins. 265-86, 2014
                                                                                      5. Lee CH et al: Lipomatosis of the sciatic nerve secondary to compression by a desmoid tumor. Skeletal Radiol. 42(12):1751-4, 2013
                                                                                      6. Oweis Y et al: Extra-abdominal desmoid tumor with osseous involvement. Skeletal Radiol. 41(4):483-7, 2012
                                                                                      7. Walker EA et al: Imaging features of superficial and deep fibromatoses in the adult population. Sarcoma. 2012:215810, 2012
                                                                                      8. Walker EA et al: Magnetic resonance imaging of benign soft tissue neoplasms in adults. Radiol Clin North Am. 49(6):1197-217, vi, 2011
                                                                                      9. Ilaslan H et al: Radiofrequency ablation: another treatment option for local control of desmoid tumors. Skeletal Radiol. 39(2):169-73, 2010
                                                                                      10. Walker EA et al: Magnetic resonance imaging of soft-tissue masses. Semin Roentgenol. 45(4):277-97, 2010
                                                                                      11. Murphey MD et al: From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation. Radiographics. 29(7):2143-73, 2009
                                                                                      12. McDonald ES et al: Best cases from the AFIP: extraabdominal desmoid-type fibromatosis. Radiographics. 28(3):901-6, 2008
                                                                                      13. Weiss SW et al: Fibromatoses. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 237-53, 2008
                                                                                      14. Hosalkar HS et al: Desmoid tumors and current status of management. Orthop Clin North Am. 37(1):53-63, 2006
                                                                                      15. Levy AD et al: From the archives of the AFIP: benign fibrous tumors and tumorlike lesions of the mesentery: radiologic-pathologic correlation. Radiographics. 26(1):245-64, 2006
                                                                                      16. Azizi L et al: MRI features of mesenteric desmoid tumors in familial adenomatous polyposis. AJR Am J Roentgenol. 184(4):1128-35, 2005
                                                                                      17. Goldblum J et al: Desmoid-type fibromatoses. In Fletcher CDM et al: WHO Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press. 83-4, 2002