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Soft Tissue Masses
Nonrhabdomyosarcoma Soft Tissue Sarcomas (NRSTS)
Nonrhabdomyosarcoma Soft Tissue Sarcomas (NRSTS)
Arthur B. Meyers, MD; A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Nonrhabdomyosarcoma soft tissue sarcomas (NRSTS)

            • Definitions

              • Soft tissue sarcomas: Heterogenous group of malignancies arising in extraskeletal mesenchymal tissues
              • In children: ~ 40% are rhabdomyosarcoma, ~ 60% are NRSTS
              • > 40 subtypes of NRSTS (e.g., synovial sarcoma, infantile fibrosarcoma, malignant peripheral nerve sheath tumor, undifferentiated sarcoma, extraskeletal Ewing sarcoma, etc.)
                • Most common types in children < 2 years of age: Infantile fibrosarcoma, malignant rhabdoid tumor, undifferentiated sarcoma
                • Most common types in older children: Synovial sarcoma, malignant peripheral nerve sheath tumor, undifferentiated sarcoma
                • Many other types are less commonly seen in children (e.g., dermatofibrosarcoma protuberans, alveolar soft part sarcoma, angiosarcoma, liposarcoma, etc.)

            • Associated Syndromes

              • Li-Fraumeni, neurofibromatosis type I (NF1), rhabdoid tumor predisposition, Noonan, Beckwith-Wiedemann, Costello, Werner, hereditary retinoblastoma, Gorlin, & other syndromes are 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. Maldonado FR et al: Quantitative characterization of extraocular orbital lesions in children using diffusion-weighted imaging. Pediatr Radiol. 51(1):119-27, 2021
                                        2. Milgrom SA et al: Non-rhabdomyosarcoma soft-tissue sarcoma. Pediatr Blood Cancer. 68 Suppl 2:e28279, 2021
                                        3. Renzi S et al: Non-rhabdomyosarcoma soft tissue sarcomas diagnosed in patients at a young age. An overview of clinical, pathological, and molecular findings. Pediatr Blood Cancer. e29022, 2021
                                        4. Cheng H et al: Clinical and prognostic characteristics of 53 cases of extracranial malignant rhabdoid tumor in children. a single-institute experience from 2007 to 2017. Oncologist. 24(7):e551-8, 2019
                                        5. Kao SC: Overview of the clinical and imaging features of the most common non-rhabdomyosarcoma soft-tissue sarcomas. Pediatr Radiol. 49(11):1524-33, 2019
                                        6. Tinkle CL et al: Nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) in pediatric and young adult patients: results from a prospective study using limited-margin radiotherapy. Cancer. 123(22):4419-29, 2017
                                        7. Al-Ibraheemi A et al: Selected diagnostically challenging pediatric soft tissue tumors. Surg Pathol Clin. 8(3):399-418, 2015
                                        8. 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
                                        9. Sangkhathat S: Current management of pediatric soft tissue sarcomas. World J Clin Pediatr. 4(4):94-105, 2015
                                        10. 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
                                        11. Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Lippincott Williams & Wilkins, 2014
                                        12. 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
                                        13. Thacker MM: Malignant soft tissue tumors in children. Orthop Clin North Am. 44(4):657-67, 2013
                                        14. Ferrari A et al: Neonatal soft tissue sarcomas. Semin Fetal Neonatal Med. 17(4):231-8, 2012
                                        15. 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
                                        16. 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
                                        17. Stein-Wexler R: Pediatric soft tissue sarcomas. Semin Ultrasound CT MR. 32(5):470-88, 2011
                                        18. Bixby SD et al: Synovial sarcoma in children: imaging features and common benign mimics. AJR Am J Roentgenol. 195(4):1026-32, 2010
                                        19. Garcés-Iñigo EF et al: Extrarenal rhabdoid tumours outside the central nervous system in infancy. Pediatr Radiol. 39(8):817-22, 2009
                                        20. 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
                                        21. 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
                                        22. 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

                                                  • Abbreviations

                                                    • Nonrhabdomyosarcoma soft tissue sarcomas (NRSTS)

                                                  • Definitions

                                                    • Soft tissue sarcomas: Heterogenous group of malignancies arising in extraskeletal mesenchymal tissues
                                                    • In children: ~ 40% are rhabdomyosarcoma, ~ 60% are NRSTS
                                                    • > 40 subtypes of NRSTS (e.g., synovial sarcoma, infantile fibrosarcoma, malignant peripheral nerve sheath tumor, undifferentiated sarcoma, extraskeletal Ewing sarcoma, etc.)
                                                      • Most common types in children < 2 years of age: Infantile fibrosarcoma, malignant rhabdoid tumor, undifferentiated sarcoma
                                                      • Most common types in older children: Synovial sarcoma, malignant peripheral nerve sheath tumor, undifferentiated sarcoma
                                                      • Many other types are less commonly seen in children (e.g., dermatofibrosarcoma protuberans, alveolar soft part sarcoma, angiosarcoma, liposarcoma, etc.)

                                                  • Associated Syndromes

                                                    • Li-Fraumeni, neurofibromatosis type I (NF1), rhabdoid tumor predisposition, Noonan, Beckwith-Wiedemann, Costello, Werner, hereditary retinoblastoma, Gorlin, & other syndromes are 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. Maldonado FR et al: Quantitative characterization of extraocular orbital lesions in children using diffusion-weighted imaging. Pediatr Radiol. 51(1):119-27, 2021
                                                                              2. Milgrom SA et al: Non-rhabdomyosarcoma soft-tissue sarcoma. Pediatr Blood Cancer. 68 Suppl 2:e28279, 2021
                                                                              3. Renzi S et al: Non-rhabdomyosarcoma soft tissue sarcomas diagnosed in patients at a young age. An overview of clinical, pathological, and molecular findings. Pediatr Blood Cancer. e29022, 2021
                                                                              4. Cheng H et al: Clinical and prognostic characteristics of 53 cases of extracranial malignant rhabdoid tumor in children. a single-institute experience from 2007 to 2017. Oncologist. 24(7):e551-8, 2019
                                                                              5. Kao SC: Overview of the clinical and imaging features of the most common non-rhabdomyosarcoma soft-tissue sarcomas. Pediatr Radiol. 49(11):1524-33, 2019
                                                                              6. Tinkle CL et al: Nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) in pediatric and young adult patients: results from a prospective study using limited-margin radiotherapy. Cancer. 123(22):4419-29, 2017
                                                                              7. Al-Ibraheemi A et al: Selected diagnostically challenging pediatric soft tissue tumors. Surg Pathol Clin. 8(3):399-418, 2015
                                                                              8. 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
                                                                              9. Sangkhathat S: Current management of pediatric soft tissue sarcomas. World J Clin Pediatr. 4(4):94-105, 2015
                                                                              10. 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
                                                                              11. Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Lippincott Williams & Wilkins, 2014
                                                                              12. 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
                                                                              13. Thacker MM: Malignant soft tissue tumors in children. Orthop Clin North Am. 44(4):657-67, 2013
                                                                              14. Ferrari A et al: Neonatal soft tissue sarcomas. Semin Fetal Neonatal Med. 17(4):231-8, 2012
                                                                              15. 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
                                                                              16. 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
                                                                              17. Stein-Wexler R: Pediatric soft tissue sarcomas. Semin Ultrasound CT MR. 32(5):470-88, 2011
                                                                              18. Bixby SD et al: Synovial sarcoma in children: imaging features and common benign mimics. AJR Am J Roentgenol. 195(4):1026-32, 2010
                                                                              19. Garcés-Iñigo EF et al: Extrarenal rhabdoid tumours outside the central nervous system in infancy. Pediatr Radiol. 39(8):817-22, 2009
                                                                              20. 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
                                                                              21. 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
                                                                              22. Murphey MD et al: From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation. Radiographics. 29(7):2143-73, 2009