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Pediatrics
Diagnosis
Musculoskeletal
Soft Tissue Masses
Fibromatosis
Fibromatosis
Christopher G. Anton, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Desmoid-type fibromatosis, aggressive fibromatosis, extraabdominal desmoid tumor, musculoaponeurotic fibromatosis

            • Definitions

              • Most common of fibrous tumors in children (60%)
              • Locally aggressive with tendency to slowly infiltrate adjacent tissues
              • Frequently recurs but lacks metastatic disease

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • Ultrasonographic Findings

                    • MR Findings

                      • Nuclear Medicine Findings

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Gross Pathologic & Surgical Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Zanchetta E et al: Magnetic resonance imaging patterns of tumor response to chemotherapy in desmoid-type fibromatosis. Cancer Med. 10(13):4356-65, 2021
                                          2. Davis JL et al: Pediatric and infantile fibroblastic/myofibroblastic tumors in the molecular era. Surg Pathol Clin. 13(4):739-62, 2020
                                          3. Garcia-Ortega DY et al: Desmoid-type fibromatosis. Cancers (Basel). 12(7), 2020
                                          4. Navarro OM: Pearls and pitfalls in the imaging of soft-tissue masses in children. Semin Ultrasound CT MR. 41(5):498-512, 2020
                                          5. Caro-Domínguez P et al: Imaging appearances of soft-tissue tumors of the pediatric foot: review of a 15-year experience at a tertiary pediatric hospital. Pediatr Radiol. 47(12):1555-71, 2017
                                          6. Sargar KM et al: Pediatric fibroblastic and myofibroblastic tumors: a pictorial review. Radiographics. 36(4):1195-214, 2016
                                          7. He XD et al: Prognostic factors for the recurrence of sporadic desmoid-type fibromatosis after macroscopically complete resection: analysis of 114 patients at a single institution. Eur J Surg Oncol. 41(8):1013-9, 2015
                                          8. Otero S et al: Desmoid-type fibromatosis. Clin Radiol. 70(9):1038-45, 2015
                                          9. Xu H et al: Desmoid-type fibromatosis of the thorax: CT, MRI, and FDG PET characteristics in a large series from a tertiary referral center. Medicine (Baltimore). 94(38):e1547, 2015
                                          10. Zhou H et al: Abdominal wall desmoid tumor during pregnancy: case report and literature review. Clin Exp Obstet Gynecol. 42(2):253-8, 2015
                                          11. Oka K et al: Usefulness of diffusion-weighted imaging for differentiating between desmoid tumors and malignant soft tissue tumors. J Magn Reson Imaging. 33(1):189-93, 2011
                                          12. Meazza C et al: Aggressive fibromatosis in children and adolescents: the Italian experience. Cancer. 116(1):233-40, 2010
                                          13. Wu C et al: Aggressive fibromatosis (desmoid tumor) is derived from mesenchymal progenitor cells. Cancer Res. 70(19):7690-8, 2010
                                          14. Murphey MD et al: From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation. Radiographics. 29(7):2143-73, 2009
                                          15. Buitendijk S et al: Pediatric aggressive fibromatosis: a retrospective analysis of 13 patients and review of literature. Cancer. 104(5):1090-9, 2005
                                          16. Kingston CA et al: Imaging of desmoid fibromatosis in pediatric patients. AJR Am J Roentgenol. 178(1):191-9, 2002
                                          17. Sorensen A et al: Treatment of aggressive fibromatosis: a retrospective study of 72 patients followed for 1-27 years. Acta Orthop Scand. 73(2):213-9, 2002
                                          18. Netscher DT et al: Infantile myofibromatosis: case report of a solitary hand lesion with emphasis on differential diagnosis and management. Ann Plast Surg. 46(1):62-7, 2001
                                          19. Ahn JM et al: Infantile fibromatosis in childhood: findings on MR imaging and pathologic correlation. Clin Radiol. 55(1):19-24, 2000
                                          20. Mehrotra AK et al: Fibromatoses of the extremities: clinicopathologic study of 36 cases. J Surg Oncol. 74(4):291-6, 2000
                                          21. Eich GF et al: Fibrous tumours in children: imaging features of a heterogeneous group of disorders. Pediatr Radiol. 28(7):500-9, 1998
                                          22. Hartman TE et al: MR imaging of extraabdominal desmoids: differentiation from other neoplasms. AJR Am J Roentgenol. 158(3):581-5, 1992
                                          23. Liu P et al: MRI of fibromatosis: with pathologic correlation. Pediatr Radiol. 22(8):587-9, 1992
                                          24. O'Keefe F et al: Magnetic resonance imaging in aggressive fibromatosis. Clin Radiol. 42(3):170-3, 1990
                                          25. Hudson TM et al: Aggressive fibromatosis: evaluation by computed tomography and angiography. Radiology. 150(2):495-501, 1984
                                          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

                                                      • Desmoid-type fibromatosis, aggressive fibromatosis, extraabdominal desmoid tumor, musculoaponeurotic fibromatosis

                                                    • Definitions

                                                      • Most common of fibrous tumors in children (60%)
                                                      • Locally aggressive with tendency to slowly infiltrate adjacent tissues
                                                      • Frequently recurs but lacks metastatic disease

                                                    IMAGING

                                                    • General Features

                                                      • Radiographic Findings

                                                        • CT Findings

                                                          • Ultrasonographic Findings

                                                            • MR Findings

                                                              • Nuclear Medicine Findings

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Gross Pathologic & Surgical Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              DIAGNOSTIC CHECKLIST

                                                                              • Consider

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Zanchetta E et al: Magnetic resonance imaging patterns of tumor response to chemotherapy in desmoid-type fibromatosis. Cancer Med. 10(13):4356-65, 2021
                                                                                  2. Davis JL et al: Pediatric and infantile fibroblastic/myofibroblastic tumors in the molecular era. Surg Pathol Clin. 13(4):739-62, 2020
                                                                                  3. Garcia-Ortega DY et al: Desmoid-type fibromatosis. Cancers (Basel). 12(7), 2020
                                                                                  4. Navarro OM: Pearls and pitfalls in the imaging of soft-tissue masses in children. Semin Ultrasound CT MR. 41(5):498-512, 2020
                                                                                  5. Caro-Domínguez P et al: Imaging appearances of soft-tissue tumors of the pediatric foot: review of a 15-year experience at a tertiary pediatric hospital. Pediatr Radiol. 47(12):1555-71, 2017
                                                                                  6. Sargar KM et al: Pediatric fibroblastic and myofibroblastic tumors: a pictorial review. Radiographics. 36(4):1195-214, 2016
                                                                                  7. He XD et al: Prognostic factors for the recurrence of sporadic desmoid-type fibromatosis after macroscopically complete resection: analysis of 114 patients at a single institution. Eur J Surg Oncol. 41(8):1013-9, 2015
                                                                                  8. Otero S et al: Desmoid-type fibromatosis. Clin Radiol. 70(9):1038-45, 2015
                                                                                  9. Xu H et al: Desmoid-type fibromatosis of the thorax: CT, MRI, and FDG PET characteristics in a large series from a tertiary referral center. Medicine (Baltimore). 94(38):e1547, 2015
                                                                                  10. Zhou H et al: Abdominal wall desmoid tumor during pregnancy: case report and literature review. Clin Exp Obstet Gynecol. 42(2):253-8, 2015
                                                                                  11. Oka K et al: Usefulness of diffusion-weighted imaging for differentiating between desmoid tumors and malignant soft tissue tumors. J Magn Reson Imaging. 33(1):189-93, 2011
                                                                                  12. Meazza C et al: Aggressive fibromatosis in children and adolescents: the Italian experience. Cancer. 116(1):233-40, 2010
                                                                                  13. Wu C et al: Aggressive fibromatosis (desmoid tumor) is derived from mesenchymal progenitor cells. Cancer Res. 70(19):7690-8, 2010
                                                                                  14. Murphey MD et al: From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation. Radiographics. 29(7):2143-73, 2009
                                                                                  15. Buitendijk S et al: Pediatric aggressive fibromatosis: a retrospective analysis of 13 patients and review of literature. Cancer. 104(5):1090-9, 2005
                                                                                  16. Kingston CA et al: Imaging of desmoid fibromatosis in pediatric patients. AJR Am J Roentgenol. 178(1):191-9, 2002
                                                                                  17. Sorensen A et al: Treatment of aggressive fibromatosis: a retrospective study of 72 patients followed for 1-27 years. Acta Orthop Scand. 73(2):213-9, 2002
                                                                                  18. Netscher DT et al: Infantile myofibromatosis: case report of a solitary hand lesion with emphasis on differential diagnosis and management. Ann Plast Surg. 46(1):62-7, 2001
                                                                                  19. Ahn JM et al: Infantile fibromatosis in childhood: findings on MR imaging and pathologic correlation. Clin Radiol. 55(1):19-24, 2000
                                                                                  20. Mehrotra AK et al: Fibromatoses of the extremities: clinicopathologic study of 36 cases. J Surg Oncol. 74(4):291-6, 2000
                                                                                  21. Eich GF et al: Fibrous tumours in children: imaging features of a heterogeneous group of disorders. Pediatr Radiol. 28(7):500-9, 1998
                                                                                  22. Hartman TE et al: MR imaging of extraabdominal desmoids: differentiation from other neoplasms. AJR Am J Roentgenol. 158(3):581-5, 1992
                                                                                  23. Liu P et al: MRI of fibromatosis: with pathologic correlation. Pediatr Radiol. 22(8):587-9, 1992
                                                                                  24. O'Keefe F et al: Magnetic resonance imaging in aggressive fibromatosis. Clin Radiol. 42(3):170-3, 1990
                                                                                  25. Hudson TM et al: Aggressive fibromatosis: evaluation by computed tomography and angiography. Radiology. 150(2):495-501, 1984