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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 muscles in 6- to 8-week fetus
              • Most common type in patients < 15 years old
              • Most commonly genitourinary, head & neck, retroperitoneum
              • Botryoid subtype (sarcoma botryoides) (10% of RMS)
                • Grape-like polyploid masses or clusters protruding into lumen of vagina, bladder, biliary tree, & nasopharynx
            • Alveolar RMS (20% of RMS)
              • Resembles skeletal muscle in 10-week fetus
              • Average age: 15 years
              • Most commonly extremity, trunk, & perianal/perirectal
            • Undifferentiated RMS
              • 30-50 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. 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
                                      2. 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
                                      3. Federico SM et al: Comparison of PET-CT and conventional imaging in staging pediatric rhabdomyosarcoma. Pediatr Blood Cancer. 60(7):1128-34, 2013
                                      4. Thacker MM: Malignant soft tissue tumors in children. Orthop Clin North Am. 44(4):657-67, 2013
                                      5. 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
                                      6. Navarro OM: Soft tissue masses in children. Radiol Clin North Am. 49(6):1235-59, vi-vii, 2011
                                      7. 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
                                      8. Stein-Wexler R: Pediatric soft tissue sarcomas. Semin Ultrasound CT MR. 32(5):470-88, 2011
                                      9. 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
                                      10. Laffan EE, Ngan BY, Navarro OM. 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
                                      11. 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
                                      12. 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
                                      13. Sebire NJ et al: Myogenin and MyoD1 expression in paediatric rhabdomyosarcomas. J Clin Pathol. 56(6):412-6, 2003
                                      14. 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 muscles in 6- to 8-week fetus
                                                  • Most common type in patients < 15 years old
                                                  • Most commonly genitourinary, head & neck, retroperitoneum
                                                  • Botryoid subtype (sarcoma botryoides) (10% of RMS)
                                                    • Grape-like polyploid masses or clusters protruding into lumen of vagina, bladder, biliary tree, & nasopharynx
                                                • Alveolar RMS (20% of RMS)
                                                  • Resembles skeletal muscle in 10-week fetus
                                                  • Average age: 15 years
                                                  • Most commonly extremity, trunk, & perianal/perirectal
                                                • Undifferentiated RMS
                                                  • 30-50 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. 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
                                                                          2. 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
                                                                          3. Federico SM et al: Comparison of PET-CT and conventional imaging in staging pediatric rhabdomyosarcoma. Pediatr Blood Cancer. 60(7):1128-34, 2013
                                                                          4. Thacker MM: Malignant soft tissue tumors in children. Orthop Clin North Am. 44(4):657-67, 2013
                                                                          5. 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
                                                                          6. Navarro OM: Soft tissue masses in children. Radiol Clin North Am. 49(6):1235-59, vi-vii, 2011
                                                                          7. 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
                                                                          8. Stein-Wexler R: Pediatric soft tissue sarcomas. Semin Ultrasound CT MR. 32(5):470-88, 2011
                                                                          9. 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
                                                                          10. Laffan EE, Ngan BY, Navarro OM. 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
                                                                          11. 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
                                                                          12. 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
                                                                          13. Sebire NJ et al: Myogenin and MyoD1 expression in paediatric rhabdomyosarcomas. J Clin Pathol. 56(6):412-6, 2003
                                                                          14. 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