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Other Soft Tissue Sarcomas
A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Definitions

              • Heterogeneous group of malignant mesenchymal tumors
                • 4th most common group of malignancies in children < 16 years old
                • Most common pediatric soft tissue sarcoma: Rhabdomyosarcoma (~ 19-50%)
                • Extraskeletal Ewing sarcoma/primitive neuroectodermal tumor & synovial sarcoma next most common
                • Many other less common lesions
            • Associated Syndromes

              • Li-Fraumeni, Noonan, Beckwith-Wiedemann, Costello, Werner, hereditary retinoblastoma, nevoid basal cell carcinoma, & neurofibromatosis type I at ↑ risk of soft tissue sarcomas

            IMAGING

            • General Features

              • Radiographic Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Nuclear Medicine Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • Staging, Grading, & Classification

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Al-Ibraheemi A et al: Selected diagnostically challenging pediatric soft tissue tumors. Surg Pathol Clin. 8(3):399-418, 2015
                                        2. Alaggio R et al: The evolution of pediatric soft tissue sarcoma classification in the last 50 years. Pediatr Dev Pathol. 18(6):481-94, 2015
                                        3. Sangkhathat S: Current management of pediatric soft tissue sarcomas. World J Clin Pediatr. 4(4):94-105, 2015
                                        4. Sargar K et al: MRI and CT of low-grade fibromyxoid sarcoma in children: a report from children's oncology group study ARST0332. AJR Am J Roentgenol. 205(2):414-20, 2015
                                        5. Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Philadelphia: Lippincott Williams & Wilkins, 2014
                                        6. Partovi S et al: Magnetic resonance/positron emission tomography (MR/PET) oncologic applications: bone and soft tissue sarcoma. Semin Roentgenol. 49(4):345-52, 2014
                                        7. Thacker MM: Malignant soft tissue tumors in children. Orthop Clin North Am. 44(4):657-67, 2013
                                        8. Ferrari A et al: Neonatal soft tissue sarcomas. Semin Fetal Neonatal Med. 17(4):231-8, 2012
                                        9. Neubauer H et al: Diffusion-weighted MRI for detection and differentiation of musculoskeletal tumorous and tumor-like lesions in pediatric patients. World J Pediatr. 8(4):342-9, 2012
                                        10. Ferrari A et al: Soft tissue sarcoma across the age spectrum: a population-based study from the Surveillance Epidemiology and End Results database. Pediatr Blood Cancer. 57(6):943-9, 2011
                                        11. Stein-Wexler R: Pediatric soft tissue sarcomas. Semin Ultrasound CT MR. 32(5):470-88, 2011
                                        12. Bixby SD et al: Synovial sarcoma in children: imaging features and common benign mimics. AJR Am J Roentgenol. 195(4):1026-32, 2010
                                        13. Garcés-Iñigo EF et al: Extrarenal rhabdoid tumours outside the central nervous system in infancy. Pediatr Radiol. 39(8):817-22, 2009
                                        14. Laffan EE et al: Pediatric soft-tissue tumors and pseudotumors: MR imaging features with pathologic correlation: part 2. Tumors of fibroblastic/myofibroblastic, so-called fibrohistiocytic, muscular, lymphomatous, neurogenic, hair matrix, and uncertain origin. Radiographics. 29(4):e36, 2009
                                        15. Navarro OM et al: Pediatric soft-tissue tumors and pseudo-tumors: MR imaging features with pathologic correlation: part 1. Imaging approach, pseudotumors, vascular lesions, and adipocytic tumors. Radiographics. 29(3):887-906, 2009
                                        16. Murphey MD et al: From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation. Radiographics. 29(7):2143-73, 2009
                                        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

                                                  • Definitions

                                                    • Heterogeneous group of malignant mesenchymal tumors
                                                      • 4th most common group of malignancies in children < 16 years old
                                                      • Most common pediatric soft tissue sarcoma: Rhabdomyosarcoma (~ 19-50%)
                                                      • Extraskeletal Ewing sarcoma/primitive neuroectodermal tumor & synovial sarcoma next most common
                                                      • Many other less common lesions
                                                  • Associated Syndromes

                                                    • Li-Fraumeni, Noonan, Beckwith-Wiedemann, Costello, Werner, hereditary retinoblastoma, nevoid basal cell carcinoma, & neurofibromatosis type I at ↑ risk of soft tissue sarcomas

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • MR Findings

                                                        • Ultrasonographic Findings

                                                          • Nuclear Medicine Findings

                                                            • Imaging Recommendations

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • Staging, Grading, & Classification

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Al-Ibraheemi A et al: Selected diagnostically challenging pediatric soft tissue tumors. Surg Pathol Clin. 8(3):399-418, 2015
                                                                              2. Alaggio R et al: The evolution of pediatric soft tissue sarcoma classification in the last 50 years. Pediatr Dev Pathol. 18(6):481-94, 2015
                                                                              3. Sangkhathat S: Current management of pediatric soft tissue sarcomas. World J Clin Pediatr. 4(4):94-105, 2015
                                                                              4. Sargar K et al: MRI and CT of low-grade fibromyxoid sarcoma in children: a report from children's oncology group study ARST0332. AJR Am J Roentgenol. 205(2):414-20, 2015
                                                                              5. Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Philadelphia: Lippincott Williams & Wilkins, 2014
                                                                              6. Partovi S et al: Magnetic resonance/positron emission tomography (MR/PET) oncologic applications: bone and soft tissue sarcoma. Semin Roentgenol. 49(4):345-52, 2014
                                                                              7. Thacker MM: Malignant soft tissue tumors in children. Orthop Clin North Am. 44(4):657-67, 2013
                                                                              8. Ferrari A et al: Neonatal soft tissue sarcomas. Semin Fetal Neonatal Med. 17(4):231-8, 2012
                                                                              9. Neubauer H et al: Diffusion-weighted MRI for detection and differentiation of musculoskeletal tumorous and tumor-like lesions in pediatric patients. World J Pediatr. 8(4):342-9, 2012
                                                                              10. Ferrari A et al: Soft tissue sarcoma across the age spectrum: a population-based study from the Surveillance Epidemiology and End Results database. Pediatr Blood Cancer. 57(6):943-9, 2011
                                                                              11. Stein-Wexler R: Pediatric soft tissue sarcomas. Semin Ultrasound CT MR. 32(5):470-88, 2011
                                                                              12. Bixby SD et al: Synovial sarcoma in children: imaging features and common benign mimics. AJR Am J Roentgenol. 195(4):1026-32, 2010
                                                                              13. Garcés-Iñigo EF et al: Extrarenal rhabdoid tumours outside the central nervous system in infancy. Pediatr Radiol. 39(8):817-22, 2009
                                                                              14. Laffan EE et al: Pediatric soft-tissue tumors and pseudotumors: MR imaging features with pathologic correlation: part 2. Tumors of fibroblastic/myofibroblastic, so-called fibrohistiocytic, muscular, lymphomatous, neurogenic, hair matrix, and uncertain origin. Radiographics. 29(4):e36, 2009
                                                                              15. Navarro OM et al: Pediatric soft-tissue tumors and pseudo-tumors: MR imaging features with pathologic correlation: part 1. Imaging approach, pseudotumors, vascular lesions, and adipocytic tumors. Radiographics. 29(3):887-906, 2009
                                                                              16. Murphey MD et al: From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation. Radiographics. 29(7):2143-73, 2009