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Desmoid-Type Fibromatosis
Christopher M. Walker, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Desmoid
              • Desmoid tumor
              • Musculoaponeurotic fibromatosis
              • Aggressive fibromatosis
              • Deep fibromatosis
            • Definitions

              • Fibromatoses: Variety of benign fibroblastic proliferations and fibroblastic soft tissue neoplasms
              • Categorized by anatomic location and patient age
                • Superficial (slow growing and small)
                  • Palmar fibromatosis (Dupuytren contracture)
                  • Plantar fibromatosis (Ledderhose disease)
                  • Penile fibromatosis (Peyronie disease)
                  • Knuckle pads
                  • Calcifying aponeurotic fibroma (children)
                  • Lipofibromatosis (children)
                  • Inclusion body fibromatosis (children)
                • Deep (fast growing and large)
                  • Desmoid-type fibromatosis (Intraabdominal or extraabdominal)
                  • Abdominal wall fibromatosis
                  • Fibromatosis colli (children)
                  • Myofibroma (children)
                  • Myofibromatosis (children)
            • Associated Syndromes

              • Gardner syndrome
                • 10% of affected patients develop desmoid-type fibromatosis (usually intraabdominal, small, and multiple)
                • Tend to follow surgery (within 4 years after colectomy)
                • High rate of recurrent disease after surgical resection

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Angiographic Findings

                        • Image-Guided Biopsy

                          • Imaging Recommendations

                            DIFFERENTIAL DIAGNOSIS

                              PATHOLOGY

                              • General Features

                                • Staging, Grading, & Classification

                                  • Gross Pathologic & Surgical Features

                                    • Microscopic Features

                                      CLINICAL ISSUES

                                      • Presentation

                                        • Demographics

                                          • Natural History & Prognosis

                                            • Treatment

                                              DIAGNOSTIC CHECKLIST

                                              • Consider

                                                Selected References

                                                1. George V et al: Chronic fibrosing conditions in abdominal imaging. Radiographics. 33(4):1053-80, 2013
                                                2. Nam SJ et al: Imaging of primary chest wall tumors with radiologic-pathologic correlation. Radiographics. 31(3):749-70, 2011
                                                3. Souza FF et al: Case report. PET/CT appearance of desmoid tumour of the chest wall. Br J Radiol. 83(986):e39-42, 2010
                                                4. Bölke E et al: Chest wall and intrathoracic desmoid tumors: surgical experience and review of the literature. Eur J Med Res. 14(6):240-3, 2009
                                                5. Murphey MD et al: From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation. Radiographics. 29(7):2143-73, 2009
                                                6. Datir A et al: MRI of soft-tissue masses: the relationship between lesion size, depth, and diagnosis. Clin Radiol. 63(4):373-8; discussion 379-80, 2008
                                                7. Kreuzberg B et al: Diagnostic problems of abdominal desmoid tumors in various locations. Eur J Radiol. 62(2):180-5, 2007
                                                8. Lee JC et al: Aggressive fibromatosis: MRI features with pathologic correlation. AJR Am J Roentgenol. 186(1):247-54, 2006
                                                9. Mendenhall WM et al: Aggressive fibromatosis. Am J Clin Oncol. 28(2):211-5, 2005
                                                10. Robbin MR et al: Imaging of musculoskeletal fibromatosis. Radiographics. 21(3):585-600, 2001
                                                11. Siegel MJ: Magnetic resonance imaging of musculoskeletal soft tissue masses. Radiol Clin North Am. 39(4):701-20, 2001
                                                12. Hartman TE et al: MR imaging of extraabdominal desmoids: differentiation from other neoplasms. AJR Am J Roentgenol. 158(3):581-5, 1992
                                                13. Casillas J et al: Imaging of intra- and extraabdominal desmoid tumors. Radiographics. 11(6):959-68, 1991
                                                14. Quinn SF et al: MR imaging in fibromatosis: results in 26 patients with pathologic correlation. AJR Am J Roentgenol. 156(3):539-42, 1991
                                                Related Anatomy
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                                                Related Differential Diagnoses
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                                                References
                                                Tables

                                                Tables

                                                KEY FACTS

                                                • Terminology

                                                  • Imaging

                                                    • Top Differential Diagnoses

                                                      • Clinical Issues

                                                        • Diagnostic Checklist

                                                          TERMINOLOGY

                                                          • Synonyms

                                                            • Desmoid
                                                            • Desmoid tumor
                                                            • Musculoaponeurotic fibromatosis
                                                            • Aggressive fibromatosis
                                                            • Deep fibromatosis
                                                          • Definitions

                                                            • Fibromatoses: Variety of benign fibroblastic proliferations and fibroblastic soft tissue neoplasms
                                                            • Categorized by anatomic location and patient age
                                                              • Superficial (slow growing and small)
                                                                • Palmar fibromatosis (Dupuytren contracture)
                                                                • Plantar fibromatosis (Ledderhose disease)
                                                                • Penile fibromatosis (Peyronie disease)
                                                                • Knuckle pads
                                                                • Calcifying aponeurotic fibroma (children)
                                                                • Lipofibromatosis (children)
                                                                • Inclusion body fibromatosis (children)
                                                              • Deep (fast growing and large)
                                                                • Desmoid-type fibromatosis (Intraabdominal or extraabdominal)
                                                                • Abdominal wall fibromatosis
                                                                • Fibromatosis colli (children)
                                                                • Myofibroma (children)
                                                                • Myofibromatosis (children)
                                                          • Associated Syndromes

                                                            • Gardner syndrome
                                                              • 10% of affected patients develop desmoid-type fibromatosis (usually intraabdominal, small, and multiple)
                                                              • Tend to follow surgery (within 4 years after colectomy)
                                                              • High rate of recurrent disease after surgical resection

                                                          IMAGING

                                                          • General Features

                                                            • Radiographic Findings

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Ultrasonographic Findings

                                                                    • Angiographic Findings

                                                                      • Image-Guided Biopsy

                                                                        • Imaging Recommendations

                                                                          DIFFERENTIAL DIAGNOSIS

                                                                            PATHOLOGY

                                                                            • General Features

                                                                              • Staging, Grading, & Classification

                                                                                • Gross Pathologic & Surgical Features

                                                                                  • Microscopic Features

                                                                                    CLINICAL ISSUES

                                                                                    • Presentation

                                                                                      • Demographics

                                                                                        • Natural History & Prognosis

                                                                                          • Treatment

                                                                                            DIAGNOSTIC CHECKLIST

                                                                                            • Consider

                                                                                              Selected References

                                                                                              1. George V et al: Chronic fibrosing conditions in abdominal imaging. Radiographics. 33(4):1053-80, 2013
                                                                                              2. Nam SJ et al: Imaging of primary chest wall tumors with radiologic-pathologic correlation. Radiographics. 31(3):749-70, 2011
                                                                                              3. Souza FF et al: Case report. PET/CT appearance of desmoid tumour of the chest wall. Br J Radiol. 83(986):e39-42, 2010
                                                                                              4. Bölke E et al: Chest wall and intrathoracic desmoid tumors: surgical experience and review of the literature. Eur J Med Res. 14(6):240-3, 2009
                                                                                              5. Murphey MD et al: From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation. Radiographics. 29(7):2143-73, 2009
                                                                                              6. Datir A et al: MRI of soft-tissue masses: the relationship between lesion size, depth, and diagnosis. Clin Radiol. 63(4):373-8; discussion 379-80, 2008
                                                                                              7. Kreuzberg B et al: Diagnostic problems of abdominal desmoid tumors in various locations. Eur J Radiol. 62(2):180-5, 2007
                                                                                              8. Lee JC et al: Aggressive fibromatosis: MRI features with pathologic correlation. AJR Am J Roentgenol. 186(1):247-54, 2006
                                                                                              9. Mendenhall WM et al: Aggressive fibromatosis. Am J Clin Oncol. 28(2):211-5, 2005
                                                                                              10. Robbin MR et al: Imaging of musculoskeletal fibromatosis. Radiographics. 21(3):585-600, 2001
                                                                                              11. Siegel MJ: Magnetic resonance imaging of musculoskeletal soft tissue masses. Radiol Clin North Am. 39(4):701-20, 2001
                                                                                              12. Hartman TE et al: MR imaging of extraabdominal desmoids: differentiation from other neoplasms. AJR Am J Roentgenol. 158(3):581-5, 1992
                                                                                              13. Casillas J et al: Imaging of intra- and extraabdominal desmoid tumors. Radiographics. 11(6):959-68, 1991
                                                                                              14. Quinn SF et al: MR imaging in fibromatosis: results in 26 patients with pathologic correlation. AJR Am J Roentgenol. 156(3):539-42, 1991