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Rhabdomyosarcoma
Bernadette L. Koch, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Rhabdomyosarcoma (RMS)
            • Definitions

              • Malignant neoplasm arising from rhabdomyoblasts
              • Most common childhood soft tissue sarcoma

            IMAGING

            • General Features

              • CT 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

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        Selected References

                                        1. Kim JK et al: Patterns of radiotherapy use and outcomes in head and neck soft-tissue sarcoma in a national cohort. Laryngoscope. ePub, 2019
                                        2. Turpin B et al: Sentinel lymph node biopsy in head and neck rhabdomyosarcoma. Pediatr Blood Cancer. 66(3):e27532, 2019
                                        3. Häußler SM et al: Head and neck rhabdomyosarcoma in children: a 20-year retrospective study at a tertiary referral center. J Cancer Res Clin Oncol. 144(2):371-9, 2018
                                        4. Chevallier KM et al: Differentiating pediatric rhabdomyosarcoma and Langerhans cell histiocytosis of the temporal bone by imaging appearance. AJNR Am J Neuroradiol. ePub, 2016
                                        5. Hosoi H: Current status of treatment for pediatric rhabdomyosarcoma in the USA and Japan. Pediatr Int. 58(2):81-7, 2016
                                        6. Gluth MB: Rhabdomyosarcoma and other pediatric temporal bone malignancies. Otolaryngol Clin North Am. 48(2):375-90, 2015
                                        7. Kubo T et al: Prognostic value of PAX3/7-FOXO1 fusion status in alveolar rhabdomyosarcoma: Systematic review and meta-analysis. Crit Rev Oncol Hematol. 96(1):46-53, 2015
                                        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. Norman G et al: Mind the gap: extent of use of diffusion-weighted MRI in children with rhabdomyosarcoma. Pediatr Radiol. 45(5):778-81, 2015
                                        10. Sun X et al: Rhabdomyosarcoma: advances in molecular and cellular biology. Sarcoma. 2015:232010, 2015
                                        11. Malempati S et al: Rhabdomyosarcoma: review of the Children's Oncology Group (COG) Soft-Tissue Sarcoma Committee experience and rationale for current COG studies. Pediatr Blood Cancer. 59(1):5-10, 2012
                                        12. Hecker RM et al: p21 Downregulation is an important component of PAX3/FKHR oncogenicity and its reactivation by HDAC inhibitors enhances combination treatment. Oncogene. 29(27):3942-52, 2010
                                        13. Zevallos JP et al: Modern multimodality therapy for pediatric nonorbital parameningeal sarcomas. Head Neck. 32(11):1501-5, 2010
                                        14. Davicioni E et al: Molecular classification of rhabdomyosarcoma--genotypic and phenotypic determinants of diagnosis: a report from the Children's Oncology Group. Am J Pathol. 174(2):550-64, 2009
                                        15. Wharam MD et al: Failure pattern and factors predictive of local failure in rhabdomyosarcoma: a report of group III patients on the third Intergroup Rhabdomyosarcoma Study. J Clin Oncol. 22(10):1902-8, 2004
                                        16. Breneman JC et al: Prognostic factors and clinical outcomes in children and adolescents with metastatic rhabdomyosarcoma--a report from the Intergroup Rhabdomyosarcoma Study IV. J Clin Oncol. 21(1):78-84, 2003
                                        17. Simon JH et al: Prognostic factors in head and neck rhabdomyosarcoma. Head Neck. 24(5):468-73, 2002
                                        18. McCarville MB et al: Rhabdomyosarcoma in pediatric patients: the good, the bad, and the unusual. AJR Am J Roentgenol. 176(6):1563-9, 2001
                                        19. Castillo M et al: Rhabdomyosarcoma of the middle ear: imaging features in two children. AJNR Am J Neuroradiol. 14(3):730-3, 1993
                                        20. Yousem DM et al: Rhabdomyosarcomas in the head and neck: MR imaging evaluation. Radiology. 177(3):683-6, 1990
                                        21. Latack JT et al: Imaging of rhabdomyosarcomas of the head and neck. AJNR Am J Neuroradiol. 8(2):353-9, 1987
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Pathology

                                                • Clinical Issues

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Rhabdomyosarcoma (RMS)
                                                  • Definitions

                                                    • Malignant neoplasm arising from rhabdomyoblasts
                                                    • Most common childhood soft tissue sarcoma

                                                  IMAGING

                                                  • General Features

                                                    • CT 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

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Consider

                                                                              Selected References

                                                                              1. Kim JK et al: Patterns of radiotherapy use and outcomes in head and neck soft-tissue sarcoma in a national cohort. Laryngoscope. ePub, 2019
                                                                              2. Turpin B et al: Sentinel lymph node biopsy in head and neck rhabdomyosarcoma. Pediatr Blood Cancer. 66(3):e27532, 2019
                                                                              3. Häußler SM et al: Head and neck rhabdomyosarcoma in children: a 20-year retrospective study at a tertiary referral center. J Cancer Res Clin Oncol. 144(2):371-9, 2018
                                                                              4. Chevallier KM et al: Differentiating pediatric rhabdomyosarcoma and Langerhans cell histiocytosis of the temporal bone by imaging appearance. AJNR Am J Neuroradiol. ePub, 2016
                                                                              5. Hosoi H: Current status of treatment for pediatric rhabdomyosarcoma in the USA and Japan. Pediatr Int. 58(2):81-7, 2016
                                                                              6. Gluth MB: Rhabdomyosarcoma and other pediatric temporal bone malignancies. Otolaryngol Clin North Am. 48(2):375-90, 2015
                                                                              7. Kubo T et al: Prognostic value of PAX3/7-FOXO1 fusion status in alveolar rhabdomyosarcoma: Systematic review and meta-analysis. Crit Rev Oncol Hematol. 96(1):46-53, 2015
                                                                              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. Norman G et al: Mind the gap: extent of use of diffusion-weighted MRI in children with rhabdomyosarcoma. Pediatr Radiol. 45(5):778-81, 2015
                                                                              10. Sun X et al: Rhabdomyosarcoma: advances in molecular and cellular biology. Sarcoma. 2015:232010, 2015
                                                                              11. Malempati S et al: Rhabdomyosarcoma: review of the Children's Oncology Group (COG) Soft-Tissue Sarcoma Committee experience and rationale for current COG studies. Pediatr Blood Cancer. 59(1):5-10, 2012
                                                                              12. Hecker RM et al: p21 Downregulation is an important component of PAX3/FKHR oncogenicity and its reactivation by HDAC inhibitors enhances combination treatment. Oncogene. 29(27):3942-52, 2010
                                                                              13. Zevallos JP et al: Modern multimodality therapy for pediatric nonorbital parameningeal sarcomas. Head Neck. 32(11):1501-5, 2010
                                                                              14. Davicioni E et al: Molecular classification of rhabdomyosarcoma--genotypic and phenotypic determinants of diagnosis: a report from the Children's Oncology Group. Am J Pathol. 174(2):550-64, 2009
                                                                              15. Wharam MD et al: Failure pattern and factors predictive of local failure in rhabdomyosarcoma: a report of group III patients on the third Intergroup Rhabdomyosarcoma Study. J Clin Oncol. 22(10):1902-8, 2004
                                                                              16. Breneman JC et al: Prognostic factors and clinical outcomes in children and adolescents with metastatic rhabdomyosarcoma--a report from the Intergroup Rhabdomyosarcoma Study IV. J Clin Oncol. 21(1):78-84, 2003
                                                                              17. Simon JH et al: Prognostic factors in head and neck rhabdomyosarcoma. Head Neck. 24(5):468-73, 2002
                                                                              18. McCarville MB et al: Rhabdomyosarcoma in pediatric patients: the good, the bad, and the unusual. AJR Am J Roentgenol. 176(6):1563-9, 2001
                                                                              19. Castillo M et al: Rhabdomyosarcoma of the middle ear: imaging features in two children. AJNR Am J Neuroradiol. 14(3):730-3, 1993
                                                                              20. Yousem DM et al: Rhabdomyosarcomas in the head and neck: MR imaging evaluation. Radiology. 177(3):683-6, 1990
                                                                              21. Latack JT et al: Imaging of rhabdomyosarcomas of the head and neck. AJNR Am J Neuroradiol. 8(2):353-9, 1987