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Rhabdomyosarcoma, Musculoskeletal
Christopher G. Anton, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Definitions

          • Mesenchymal sarcoma arising from rhabdomyoblasts (primitive muscle cells); lacks normal differentiation into skeletal muscle
            • Embryonal rhabdomyosarcoma (RMS): 60-70% of childhood RMS
              • Resembles skeletal muscle of 6- to 8-week fetus
              • Most common type in patients < 15 years old
              • Most common locations: GU, head & neck, retroperitoneum
              • Botryoid subtype (sarcoma botryoides): 10% of RMS
                • Grape-like polyploid masses or clusters protruding into lumen of vagina, bladder, biliary tree, or nasopharynx
            • Alveolar RMS: 20% of RMS
              • Resembles skeletal muscle of 10-week fetus
              • Average age: 15 years
              • Most common locations: Extremity, trunk, & perianal/perirectal
            • Undifferentiated RMS
              • 30- to 50-year-olds; rarely in children

        IMAGING

        • General Features

          • Radiographic Findings

            • CT Findings

              • Ultrasonographic Findings

                • MR Findings

                  • Nuclear Medicine Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      Selected References

                                      1. Harrison DJ et al: Metabolic response as assessed by 18 F-fluorodeoxyglucose positron emission tomography-computed tomography does not predict outcome in patients with intermediate- or high-risk rhabdomyosarcoma: a report from the Children's Oncology Group Soft Tissue Sarcoma Committee. Cancer Med. 10(3):857-66, 2021
                                      2. Li H et al: Germline cancer-predisposition variants in pediatric rhabdomyosarcoma: a report from the Children's Oncology Group. J Natl Cancer Inst. 113(7):875-83, 2021
                                      3. Maldonado FR et al: Quantitative characterization of extraocular orbital lesions in children using diffusion-weighted imaging. Pediatr Radiol. 51(1):119-27, 2021
                                      4. Rogers TN et al: Management of rhabdomyosarcoma in pediatric patients. Surg Oncol Clin N Am. 30(2):339-53, 2021
                                      5. Morris CD et al: Surgical management of extremity rhabdomyosarcoma: a consensus opinion from the Children's Oncology Group, the European Pediatric Soft-Tissue Sarcoma Study Group, and the Cooperative Weichteilsarkom Studiengruppe. Pediatr Blood Cancer. ePub, 2020
                                      6. Navarro OM: Pearls and pitfalls in the imaging of soft-tissue masses in children. Semin Ultrasound CT MR. 41(5):498-512, 2020
                                      7. Harrison DJ et al: The role of 18F-FDG-PET/CT in pediatric sarcoma. Semin Nucl Med. 47(3):229-41, 2017
                                      8. Norman G et al: An emerging evidence base for PET-CT in the management of childhood rhabdomyosarcoma: systematic review. BMJ Open. 5(1):e006030, 2015
                                      9. Casey DL et al: Predicting outcome in patients with rhabdomyosarcoma: role of [(18)f]fluorodeoxyglucose positron emission tomography. Int J Radiat Oncol Biol Phys. 90(5):1136-42, 2014
                                      10. Federico SM et al: Comparison of PET-CT and conventional imaging in staging pediatric rhabdomyosarcoma. Pediatr Blood Cancer. 60(7):1128-34, 2013
                                      11. Thacker MM: Malignant soft tissue tumors in children. Orthop Clin North Am. 44(4):657-67, 2013
                                      12. Dharmarajan KV et al: Positron emission tomography (PET) evaluation after initial chemotherapy and radiation therapy predicts local control in rhabdomyosarcoma. Int J Radiat Oncol Biol Phys. 84(4):996-1002, 2012
                                      13. Navarro OM: Soft tissue masses in children. Radiol Clin North Am. 49(6):1235-59, vi-vii, 2011
                                      14. Stegmaier S et al: Prognostic value of PAX-FKHR fusion status in alveolar rhabdomyosarcoma: a report from the cooperative soft tissue sarcoma study group (CWS). Pediatr Blood Cancer. 57(3):406-14, 2011
                                      15. Stein-Wexler R: Pediatric soft tissue sarcomas. Semin Ultrasound CT MR. 32(5):470-88, 2011
                                      16. Bolger JC et al: Alveolar rhabdomyosarcoma originating between the fourth and fifth metatarsal--case report and literature review. Foot Ankle Surg. 16(3):e51-4, 2010
                                      17. 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
                                      18. Völker T et al: Positron emission tomography for staging of pediatric sarcoma patients: results of a prospective multicenter trial. J Clin Oncol. 25(34):5435-41, 2007
                                      19. Zhang ZH et al: [MRI and CT diagnosis of rhabdomyosarcoma in the extremities:a report of nine cases.] Ai Zheng. 26(9):1001-4, 2007
                                      20. Sebire NJ et al: Myogenin and MyoD1 expression in paediatric rhabdomyosarcomas. J Clin Pathol. 56(6):412-6, 2003
                                      21. Sorensen PH et al: PAX3-FKHR and PAX7-FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: a report from the children's oncology group. J Clin Oncol. 20(11):2672-9, 2002
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Clinical Issues

                                            TERMINOLOGY

                                            • Definitions

                                              • Mesenchymal sarcoma arising from rhabdomyoblasts (primitive muscle cells); lacks normal differentiation into skeletal muscle
                                                • Embryonal rhabdomyosarcoma (RMS): 60-70% of childhood RMS
                                                  • Resembles skeletal muscle of 6- to 8-week fetus
                                                  • Most common type in patients < 15 years old
                                                  • Most common locations: GU, head & neck, retroperitoneum
                                                  • Botryoid subtype (sarcoma botryoides): 10% of RMS
                                                    • Grape-like polyploid masses or clusters protruding into lumen of vagina, bladder, biliary tree, or nasopharynx
                                                • Alveolar RMS: 20% of RMS
                                                  • Resembles skeletal muscle of 10-week fetus
                                                  • Average age: 15 years
                                                  • Most common locations: Extremity, trunk, & perianal/perirectal
                                                • Undifferentiated RMS
                                                  • 30- to 50-year-olds; rarely in children

                                            IMAGING

                                            • General Features

                                              • Radiographic Findings

                                                • CT Findings

                                                  • Ultrasonographic Findings

                                                    • MR Findings

                                                      • Nuclear Medicine Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Staging, Grading, & Classification

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          Selected References

                                                                          1. Harrison DJ et al: Metabolic response as assessed by 18 F-fluorodeoxyglucose positron emission tomography-computed tomography does not predict outcome in patients with intermediate- or high-risk rhabdomyosarcoma: a report from the Children's Oncology Group Soft Tissue Sarcoma Committee. Cancer Med. 10(3):857-66, 2021
                                                                          2. Li H et al: Germline cancer-predisposition variants in pediatric rhabdomyosarcoma: a report from the Children's Oncology Group. J Natl Cancer Inst. 113(7):875-83, 2021
                                                                          3. Maldonado FR et al: Quantitative characterization of extraocular orbital lesions in children using diffusion-weighted imaging. Pediatr Radiol. 51(1):119-27, 2021
                                                                          4. Rogers TN et al: Management of rhabdomyosarcoma in pediatric patients. Surg Oncol Clin N Am. 30(2):339-53, 2021
                                                                          5. Morris CD et al: Surgical management of extremity rhabdomyosarcoma: a consensus opinion from the Children's Oncology Group, the European Pediatric Soft-Tissue Sarcoma Study Group, and the Cooperative Weichteilsarkom Studiengruppe. Pediatr Blood Cancer. ePub, 2020
                                                                          6. Navarro OM: Pearls and pitfalls in the imaging of soft-tissue masses in children. Semin Ultrasound CT MR. 41(5):498-512, 2020
                                                                          7. Harrison DJ et al: The role of 18F-FDG-PET/CT in pediatric sarcoma. Semin Nucl Med. 47(3):229-41, 2017
                                                                          8. Norman G et al: An emerging evidence base for PET-CT in the management of childhood rhabdomyosarcoma: systematic review. BMJ Open. 5(1):e006030, 2015
                                                                          9. Casey DL et al: Predicting outcome in patients with rhabdomyosarcoma: role of [(18)f]fluorodeoxyglucose positron emission tomography. Int J Radiat Oncol Biol Phys. 90(5):1136-42, 2014
                                                                          10. Federico SM et al: Comparison of PET-CT and conventional imaging in staging pediatric rhabdomyosarcoma. Pediatr Blood Cancer. 60(7):1128-34, 2013
                                                                          11. Thacker MM: Malignant soft tissue tumors in children. Orthop Clin North Am. 44(4):657-67, 2013
                                                                          12. Dharmarajan KV et al: Positron emission tomography (PET) evaluation after initial chemotherapy and radiation therapy predicts local control in rhabdomyosarcoma. Int J Radiat Oncol Biol Phys. 84(4):996-1002, 2012
                                                                          13. Navarro OM: Soft tissue masses in children. Radiol Clin North Am. 49(6):1235-59, vi-vii, 2011
                                                                          14. Stegmaier S et al: Prognostic value of PAX-FKHR fusion status in alveolar rhabdomyosarcoma: a report from the cooperative soft tissue sarcoma study group (CWS). Pediatr Blood Cancer. 57(3):406-14, 2011
                                                                          15. Stein-Wexler R: Pediatric soft tissue sarcomas. Semin Ultrasound CT MR. 32(5):470-88, 2011
                                                                          16. Bolger JC et al: Alveolar rhabdomyosarcoma originating between the fourth and fifth metatarsal--case report and literature review. Foot Ankle Surg. 16(3):e51-4, 2010
                                                                          17. 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
                                                                          18. Völker T et al: Positron emission tomography for staging of pediatric sarcoma patients: results of a prospective multicenter trial. J Clin Oncol. 25(34):5435-41, 2007
                                                                          19. Zhang ZH et al: [MRI and CT diagnosis of rhabdomyosarcoma in the extremities:a report of nine cases.] Ai Zheng. 26(9):1001-4, 2007
                                                                          20. Sebire NJ et al: Myogenin and MyoD1 expression in paediatric rhabdomyosarcomas. J Clin Pathol. 56(6):412-6, 2003
                                                                          21. Sorensen PH et al: PAX3-FKHR and PAX7-FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: a report from the children's oncology group. J Clin Oncol. 20(11):2672-9, 2002