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Rhabdomyosarcoma, Head and Neck
Bernadette L. Koch, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Definitions

              • Rhabdomyosarcoma (RMS): Malignant neoplasm of rhabdomyoblasts
                • Most common childhood soft tissue sarcoma

            IMAGING

            • General Features

              • Ultrasonographic Findings

                • 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. Maldonado FR et al: Quantitative characterization of extraocular orbital lesions in children using diffusion-weighted imaging. Pediatr Radiol. 51(1):119-27, 2021
                                          2. Jawad N et al: The clinical and radiologic features of paediatric rhabdomyosarcoma. Pediatr Radiol. 49(11):1516-23, 2019
                                          3. Kim JK et al: Patterns of radiotherapy use and outcomes in head and neck soft-tissue sarcoma in a national cohort. Laryngoscope. 130(1):120-7, 2019
                                          4. Turpin B et al: Sentinel lymph node biopsy in head and neck rhabdomyosarcoma. Pediatr Blood Cancer. 66(3):e27532, 2019
                                          5. 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
                                          6. Chevallier KM et al: Differentiating pediatric rhabdomyosarcoma and Langerhans cell histiocytosis of the temporal bone by imaging appearance. AJNR Am J Neuroradiol. 37(6):1185-9, 2016
                                          7. Hosoi H: Current status of treatment for pediatric rhabdomyosarcoma in the USA and Japan. Pediatr Int. 58(2):81-7, 2016
                                          8. Gluth MB: Rhabdomyosarcoma and other pediatric temporal bone malignancies. Otolaryngol Clin North Am. 48(2):375-90, 2015
                                          9. 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
                                          10. 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
                                          11. 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
                                          12. Sun X et al: Rhabdomyosarcoma: advances in molecular and cellular biology. Sarcoma. 2015:232010, 2015
                                          13. 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
                                          14. 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
                                          15. Zevallos JP et al: Modern multimodality therapy for pediatric nonorbital parameningeal sarcomas. Head Neck. 32(11):1501-5, 2010
                                          16. 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
                                          17. 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
                                          18. 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
                                          19. Simon JH et al: Prognostic factors in head and neck rhabdomyosarcoma. Head Neck. 24(5):468-73, 2002
                                          20. McCarville MB et al: Rhabdomyosarcoma in pediatric patients: the good, the bad, and the unusual. AJR Am J Roentgenol. 176(6):1563-9, 2001
                                          21. Castillo M et al: Rhabdomyosarcoma of the middle ear: imaging features in two children. AJNR Am J Neuroradiol. 14(3):730-3, 1993
                                          22. Yousem DM et al: Rhabdomyosarcomas in the head and neck: MR imaging evaluation. Radiology. 177(3):683-6, 1990
                                          23. 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

                                                    • Definitions

                                                      • Rhabdomyosarcoma (RMS): Malignant neoplasm of rhabdomyoblasts
                                                        • Most common childhood soft tissue sarcoma

                                                    IMAGING

                                                    • General Features

                                                      • Ultrasonographic Findings

                                                        • 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. Maldonado FR et al: Quantitative characterization of extraocular orbital lesions in children using diffusion-weighted imaging. Pediatr Radiol. 51(1):119-27, 2021
                                                                                  2. Jawad N et al: The clinical and radiologic features of paediatric rhabdomyosarcoma. Pediatr Radiol. 49(11):1516-23, 2019
                                                                                  3. Kim JK et al: Patterns of radiotherapy use and outcomes in head and neck soft-tissue sarcoma in a national cohort. Laryngoscope. 130(1):120-7, 2019
                                                                                  4. Turpin B et al: Sentinel lymph node biopsy in head and neck rhabdomyosarcoma. Pediatr Blood Cancer. 66(3):e27532, 2019
                                                                                  5. 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
                                                                                  6. Chevallier KM et al: Differentiating pediatric rhabdomyosarcoma and Langerhans cell histiocytosis of the temporal bone by imaging appearance. AJNR Am J Neuroradiol. 37(6):1185-9, 2016
                                                                                  7. Hosoi H: Current status of treatment for pediatric rhabdomyosarcoma in the USA and Japan. Pediatr Int. 58(2):81-7, 2016
                                                                                  8. Gluth MB: Rhabdomyosarcoma and other pediatric temporal bone malignancies. Otolaryngol Clin North Am. 48(2):375-90, 2015
                                                                                  9. 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
                                                                                  10. 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
                                                                                  11. 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
                                                                                  12. Sun X et al: Rhabdomyosarcoma: advances in molecular and cellular biology. Sarcoma. 2015:232010, 2015
                                                                                  13. 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
                                                                                  14. 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
                                                                                  15. Zevallos JP et al: Modern multimodality therapy for pediatric nonorbital parameningeal sarcomas. Head Neck. 32(11):1501-5, 2010
                                                                                  16. 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
                                                                                  17. 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
                                                                                  18. 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
                                                                                  19. Simon JH et al: Prognostic factors in head and neck rhabdomyosarcoma. Head Neck. 24(5):468-73, 2002
                                                                                  20. McCarville MB et al: Rhabdomyosarcoma in pediatric patients: the good, the bad, and the unusual. AJR Am J Roentgenol. 176(6):1563-9, 2001
                                                                                  21. Castillo M et al: Rhabdomyosarcoma of the middle ear: imaging features in two children. AJNR Am J Neuroradiol. 14(3):730-3, 1993
                                                                                  22. Yousem DM et al: Rhabdomyosarcomas in the head and neck: MR imaging evaluation. Radiology. 177(3):683-6, 1990
                                                                                  23. Latack JT et al: Imaging of rhabdomyosarcomas of the head and neck. AJNR Am J Neuroradiol. 8(2):353-9, 1987