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Rhabdomyosarcoma, Genitourinary
Sara M. O'Hara, MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Rhabdomyosarcoma (RMS), genitourinary (GU)
          • Synonyms

            • Botryoid tumor, sarcoma botryoides, embryonal RMS
          • Definitions

            • Malignant tumor of striated muscle originating from any pelvic organ (but paratesticular site is discussed separately)

          IMAGING

          • General Features

            • Radiographic Findings

              • Ultrasonographic Findings

                • Fluoroscopic Findings

                  • CT Findings

                    • MR Findings

                      • Nuclear Medicine Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            Selected References

                                            1. Rogers TN et al: Management of rhabdomyosarcoma in pediatric patients. Surg Oncol Clin N Am. 30(2):339-53, 2021
                                            2. Harrison DJ et al: PET with 18F-fluorodeoxyglucose/computed tomography in the management of pediatric sarcoma. PET Clin. 15(3):333-47, 2020
                                            3. Indelicato DJ et al: Outcomes following proton therapy for group iii pelvic rhabdomyosarcoma. Int J Radiat Oncol Biol Phys. 106(5):968-76, 2020
                                            4. Sa R et al: Utility of [18F] fluoro-deoxyglucose positron emission tomography/computed tomography for staging and therapy response evaluation in pediatric rhabdomyosarcoma: a case series and literature review. Front Med (Lausanne). 7:281, 2020
                                            5. Guillerman RP et al: Imaging of DICER1 syndrome. Pediatr Radiol. 49(11):1488-505, 2019
                                            6. Jawad N et al: The clinical and radiologic features of paediatric rhabdomyosarcoma. Pediatr Radiol. 49(11):1516-23, 2019
                                            7. Koivusalo AI et al: Combined management of perianal rhabdomyosarcoma with chemotherapy, radical surgery, and irradiation: a series of three consecutive children. J Pediatr Surg. 54(2):285-7, 2019
                                            8. Bueno MT et al: Pediatric imaging in DICER1 syndrome. Pediatr Radiol. 47(10):1292-301, 2017
                                            9. Kim JR et al: Rhabdomyosarcoma in children and adolescents: patterns and risk factors of distant metastasis. AJR Am J Roentgenol. 209(2):409-16, 2017
                                            10. Shelmerdine SC et al: Pearls and pitfalls in diagnosing pediatric urinary bladder masses. Radiographics. 37(6):1872-91, 2017
                                            11. Bethell GS et al: A case of embryonal rhabdomyosarcoma presenting as a lobulated protrusion from the urethral meatus at birth. Urology. 86(4):805-7, 2015
                                            12. Chung EM et al: Solid tumors of the peritoneum, omentum, and mesentery in children: radiologic-pathologic correlation: from the radiologic pathology archives. Radiographics. 35(2):521-46, 2015
                                            13. Dong Y et al: 18[F]FDG PET/CT is useful in initial staging, restaging for pediatric rhabdomyosarcoma. Q J Nucl Med Mol Imaging. 61(4):438-46, 2015
                                            14. Fernandez-Pineda I et al: Bilateral internal hemipelvectomy for osteosarcoma in a pediatric patient previously treated for rhabdomyosarcoma. Orthop Traumatol Surg Res. 101(3):395-7, 2015
                                            15. 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
                                            16. Rodeberg DA et al: Delayed primary excision with subsequent modification of radiotherapy dose for intermediate-risk rhabdomyosarcoma: a report from the Children's Oncology Group Soft Tissue Sarcoma Committee. Int J Cancer. 137(1):204-11, 2015
                                            17. Jenney M et al: Conservative approach in localised rhabdomyosarcoma of the bladder and prostate: results from International Society of Paediatric Oncology (SIOP) studies: malignant mesenchymal tumour (MMT) 84, 89 and 95. Pediatr Blood Cancer. 61(2):217-22, 2014
                                            18. Kirsch CH et al: Outcome of female pediatric patients diagnosed with genital tract rhabdomyosarcoma based on analysis of cases registered in SEER database between 1973 and 2006. Am J Clin Oncol. 37(1):47-50, 2014
                                            19. Komasara L et al: A review on surgical techniques and organ sparing procedures in bladder/prostate rhabdomyosarcoma. Eur J Pediatr Surg. 24(6):467-73, 2014
                                            20. Sinclair N et al: A rare and unusual case of Burkitt's lymphoma presenting with a prostate mass in a 12-year-old boy. Case Rep Radiol. 2014:106176, 2014
                                            21. Federico SM et al: Comparison of PET-CT and conventional imaging in staging pediatric rhabdomyosarcoma. Pediatr Blood Cancer. 60(7):1128-34, 2013
                                            22. Kuru TH et al: Pediatric case report on magnetic resonance imaging/transrectal ultrasound-fusion biopsy of rhabdomyosarcoma of the bladder/prostate: a new tool to reduce therapy-associated morbidity? Urology. 81(2):417-20, 2013
                                            23. Pai DR et al: Magnetic resonance imaging of pediatric pelvic masses. Magn Reson Imaging Clin N Am. 21(4):751-72, 2013
                                            24. Walther A et al: Renal rhabdomyosarcoma in a pancake kidney. Urology. 82(2):458-60, 2013
                                            25. Grimsby GM et al: Pediatric urologic oncology. Pediatr Clin North Am. 59(4):947-59, 2012
                                            26. Ricard F et al: Additional benefit of F-18 FDG PET/CT in the staging and follow-up of pediatric rhabdomyosarcoma. Clin Nucl Med. 36(8):672-7, 2011
                                            27. Rodeberg DA et al: Comparison of outcomes based on treatment algorithms for rhabdomyosarcoma of the bladder/prostate: combined results from the Children's Oncology Group, German Cooperative Soft Tissue Sarcoma Study, Italian Cooperative Group, and International Society of Pediatric Oncology Malignant Mesenchymal Tumors Committee. Int J Cancer. 128(5):1232-9, 2011
                                            28. Raney B et al: Impact of tumor viability at second-look procedures performed before completing treatment on the Intergroup Rhabdomyosarcoma Study Group protocol IRS-IV, 1991-1997: a report from the children's oncology group. J Pediatr Surg. 45(11):2160-8, 2010
                                            29. Esfahani SA et al: An antenatally diagnosed rhabdomyosarcoma of the bladder treated without extensive surgery. Nat Rev Urol. 6(8):449-53, 2009
                                            30. Kobi M et al: Sarcoma botryoides: MRI findings in two patients. J Magn Reson Imaging. 29(3):708-12, 2009
                                            31. Rypens F et al: Paratesticular rhabdomyosarcoma presenting as thickening of the tunica vaginalis. Pediatr Radiol. 39(9):1010-2, 2009
                                            32. Stehr M: Pediatric urologic rhabdomyosarcoma. Curr Opin Urol. 19(4):402-6, 2009
                                            33. Citak EC et al: Vincristine-induced peripheral neuropathy and urinary bladder paralysis in a child with rhabdomyosarcoma. J Pediatr Hematol Oncol. 30(1):61-2, 2008
                                            34. Cowles RA et al: Ovarian repositioning in pediatric cancer patients: Flexible techniques accommodate pelvic radiation fields. Pediatr Blood Cancer. 49(3):339-41, 2007
                                            35. Striegel AM et al: Vaginal discharge and bleeding in girls younger than 6 years. J Urol. 176(6 Pt 1):2632-5, 2006
                                            36. Spunt SL et al: Late effects of pelvic rhabdomyosarcoma and its treatment in female survivors. J Clin Oncol. 23(28):7143-51, 2005
                                            37. Gruessner SE et al: Management of stage I cervical sarcoma botryoides in childhood and adolescence. Eur J Pediatr. 163(8):452-6, 2004
                                            38. Raney RB et al: Results of treatment of fifty-six patients with localized retroperitoneal and pelvic rhabdomyosarcoma: a report from The Intergroup Rhabdomyosarcoma Study-IV, 1991-1997. Pediatr Blood Cancer. 42(7):618-25, 2004
                                            39. Sanghvi DA et al: Primary rhabdomyosarcoma of the seminal vesicle. Br J Radiol. 77(914):159-60, 2004
                                            40. Akbar SA et al: Multimodality imaging of paratesticular neoplasms and their rare mimics. Radiographics. 23(6):1461-76, 2003
                                            41. Nakada K et al: Successful resection of prostatic rhabdomyosarcoma by the posterior sagittal approach combined with preoperative chemotherapy. J Pediatr Surg. 38(7):E6-8, 2003
                                            42. Ng TY et al: Alveolar rhabdomyosarcoma of the cervix. Gynecol Oncol. 91(3):623-6, 2003
                                            43. Ortega JA et al: Presence of well-differentiated rhabdomyoblasts at the end of therapy for pelvic rhabdomyosarcoma: implications for the outcome. J Pediatr Hematol Oncol. 22(2):106-11, 2000
                                            44. Silvan AM et al: Organ-preserving management of rhabdomyosarcoma of the prostate and bladder in children. Med Pediatr Oncol. 29(6):573-5, 1997
                                            45. Duel BP et al: Reconstructive options in genitourinary rhabdomyosarcoma. J Urol. 156(5):1798-804, 1996
                                            46. Breen JL et al: Genital tract tumors in children. Pediatr Clin North Am. 28(2):355-67, 1981
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Pathology

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Rhabdomyosarcoma (RMS), genitourinary (GU)
                                                    • Synonyms

                                                      • Botryoid tumor, sarcoma botryoides, embryonal RMS
                                                    • Definitions

                                                      • Malignant tumor of striated muscle originating from any pelvic organ (but paratesticular site is discussed separately)

                                                    IMAGING

                                                    • General Features

                                                      • Radiographic Findings

                                                        • Ultrasonographic Findings

                                                          • Fluoroscopic Findings

                                                            • CT Findings

                                                              • MR Findings

                                                                • Nuclear Medicine Findings

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Staging, Grading, & Classification

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      Selected References

                                                                                      1. Rogers TN et al: Management of rhabdomyosarcoma in pediatric patients. Surg Oncol Clin N Am. 30(2):339-53, 2021
                                                                                      2. Harrison DJ et al: PET with 18F-fluorodeoxyglucose/computed tomography in the management of pediatric sarcoma. PET Clin. 15(3):333-47, 2020
                                                                                      3. Indelicato DJ et al: Outcomes following proton therapy for group iii pelvic rhabdomyosarcoma. Int J Radiat Oncol Biol Phys. 106(5):968-76, 2020
                                                                                      4. Sa R et al: Utility of [18F] fluoro-deoxyglucose positron emission tomography/computed tomography for staging and therapy response evaluation in pediatric rhabdomyosarcoma: a case series and literature review. Front Med (Lausanne). 7:281, 2020
                                                                                      5. Guillerman RP et al: Imaging of DICER1 syndrome. Pediatr Radiol. 49(11):1488-505, 2019
                                                                                      6. Jawad N et al: The clinical and radiologic features of paediatric rhabdomyosarcoma. Pediatr Radiol. 49(11):1516-23, 2019
                                                                                      7. Koivusalo AI et al: Combined management of perianal rhabdomyosarcoma with chemotherapy, radical surgery, and irradiation: a series of three consecutive children. J Pediatr Surg. 54(2):285-7, 2019
                                                                                      8. Bueno MT et al: Pediatric imaging in DICER1 syndrome. Pediatr Radiol. 47(10):1292-301, 2017
                                                                                      9. Kim JR et al: Rhabdomyosarcoma in children and adolescents: patterns and risk factors of distant metastasis. AJR Am J Roentgenol. 209(2):409-16, 2017
                                                                                      10. Shelmerdine SC et al: Pearls and pitfalls in diagnosing pediatric urinary bladder masses. Radiographics. 37(6):1872-91, 2017
                                                                                      11. Bethell GS et al: A case of embryonal rhabdomyosarcoma presenting as a lobulated protrusion from the urethral meatus at birth. Urology. 86(4):805-7, 2015
                                                                                      12. Chung EM et al: Solid tumors of the peritoneum, omentum, and mesentery in children: radiologic-pathologic correlation: from the radiologic pathology archives. Radiographics. 35(2):521-46, 2015
                                                                                      13. Dong Y et al: 18[F]FDG PET/CT is useful in initial staging, restaging for pediatric rhabdomyosarcoma. Q J Nucl Med Mol Imaging. 61(4):438-46, 2015
                                                                                      14. Fernandez-Pineda I et al: Bilateral internal hemipelvectomy for osteosarcoma in a pediatric patient previously treated for rhabdomyosarcoma. Orthop Traumatol Surg Res. 101(3):395-7, 2015
                                                                                      15. 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
                                                                                      16. Rodeberg DA et al: Delayed primary excision with subsequent modification of radiotherapy dose for intermediate-risk rhabdomyosarcoma: a report from the Children's Oncology Group Soft Tissue Sarcoma Committee. Int J Cancer. 137(1):204-11, 2015
                                                                                      17. Jenney M et al: Conservative approach in localised rhabdomyosarcoma of the bladder and prostate: results from International Society of Paediatric Oncology (SIOP) studies: malignant mesenchymal tumour (MMT) 84, 89 and 95. Pediatr Blood Cancer. 61(2):217-22, 2014
                                                                                      18. Kirsch CH et al: Outcome of female pediatric patients diagnosed with genital tract rhabdomyosarcoma based on analysis of cases registered in SEER database between 1973 and 2006. Am J Clin Oncol. 37(1):47-50, 2014
                                                                                      19. Komasara L et al: A review on surgical techniques and organ sparing procedures in bladder/prostate rhabdomyosarcoma. Eur J Pediatr Surg. 24(6):467-73, 2014
                                                                                      20. Sinclair N et al: A rare and unusual case of Burkitt's lymphoma presenting with a prostate mass in a 12-year-old boy. Case Rep Radiol. 2014:106176, 2014
                                                                                      21. Federico SM et al: Comparison of PET-CT and conventional imaging in staging pediatric rhabdomyosarcoma. Pediatr Blood Cancer. 60(7):1128-34, 2013
                                                                                      22. Kuru TH et al: Pediatric case report on magnetic resonance imaging/transrectal ultrasound-fusion biopsy of rhabdomyosarcoma of the bladder/prostate: a new tool to reduce therapy-associated morbidity? Urology. 81(2):417-20, 2013
                                                                                      23. Pai DR et al: Magnetic resonance imaging of pediatric pelvic masses. Magn Reson Imaging Clin N Am. 21(4):751-72, 2013
                                                                                      24. Walther A et al: Renal rhabdomyosarcoma in a pancake kidney. Urology. 82(2):458-60, 2013
                                                                                      25. Grimsby GM et al: Pediatric urologic oncology. Pediatr Clin North Am. 59(4):947-59, 2012
                                                                                      26. Ricard F et al: Additional benefit of F-18 FDG PET/CT in the staging and follow-up of pediatric rhabdomyosarcoma. Clin Nucl Med. 36(8):672-7, 2011
                                                                                      27. Rodeberg DA et al: Comparison of outcomes based on treatment algorithms for rhabdomyosarcoma of the bladder/prostate: combined results from the Children's Oncology Group, German Cooperative Soft Tissue Sarcoma Study, Italian Cooperative Group, and International Society of Pediatric Oncology Malignant Mesenchymal Tumors Committee. Int J Cancer. 128(5):1232-9, 2011
                                                                                      28. Raney B et al: Impact of tumor viability at second-look procedures performed before completing treatment on the Intergroup Rhabdomyosarcoma Study Group protocol IRS-IV, 1991-1997: a report from the children's oncology group. J Pediatr Surg. 45(11):2160-8, 2010
                                                                                      29. Esfahani SA et al: An antenatally diagnosed rhabdomyosarcoma of the bladder treated without extensive surgery. Nat Rev Urol. 6(8):449-53, 2009
                                                                                      30. Kobi M et al: Sarcoma botryoides: MRI findings in two patients. J Magn Reson Imaging. 29(3):708-12, 2009
                                                                                      31. Rypens F et al: Paratesticular rhabdomyosarcoma presenting as thickening of the tunica vaginalis. Pediatr Radiol. 39(9):1010-2, 2009
                                                                                      32. Stehr M: Pediatric urologic rhabdomyosarcoma. Curr Opin Urol. 19(4):402-6, 2009
                                                                                      33. Citak EC et al: Vincristine-induced peripheral neuropathy and urinary bladder paralysis in a child with rhabdomyosarcoma. J Pediatr Hematol Oncol. 30(1):61-2, 2008
                                                                                      34. Cowles RA et al: Ovarian repositioning in pediatric cancer patients: Flexible techniques accommodate pelvic radiation fields. Pediatr Blood Cancer. 49(3):339-41, 2007
                                                                                      35. Striegel AM et al: Vaginal discharge and bleeding in girls younger than 6 years. J Urol. 176(6 Pt 1):2632-5, 2006
                                                                                      36. Spunt SL et al: Late effects of pelvic rhabdomyosarcoma and its treatment in female survivors. J Clin Oncol. 23(28):7143-51, 2005
                                                                                      37. Gruessner SE et al: Management of stage I cervical sarcoma botryoides in childhood and adolescence. Eur J Pediatr. 163(8):452-6, 2004
                                                                                      38. Raney RB et al: Results of treatment of fifty-six patients with localized retroperitoneal and pelvic rhabdomyosarcoma: a report from The Intergroup Rhabdomyosarcoma Study-IV, 1991-1997. Pediatr Blood Cancer. 42(7):618-25, 2004
                                                                                      39. Sanghvi DA et al: Primary rhabdomyosarcoma of the seminal vesicle. Br J Radiol. 77(914):159-60, 2004
                                                                                      40. Akbar SA et al: Multimodality imaging of paratesticular neoplasms and their rare mimics. Radiographics. 23(6):1461-76, 2003
                                                                                      41. Nakada K et al: Successful resection of prostatic rhabdomyosarcoma by the posterior sagittal approach combined with preoperative chemotherapy. J Pediatr Surg. 38(7):E6-8, 2003
                                                                                      42. Ng TY et al: Alveolar rhabdomyosarcoma of the cervix. Gynecol Oncol. 91(3):623-6, 2003
                                                                                      43. Ortega JA et al: Presence of well-differentiated rhabdomyoblasts at the end of therapy for pelvic rhabdomyosarcoma: implications for the outcome. J Pediatr Hematol Oncol. 22(2):106-11, 2000
                                                                                      44. Silvan AM et al: Organ-preserving management of rhabdomyosarcoma of the prostate and bladder in children. Med Pediatr Oncol. 29(6):573-5, 1997
                                                                                      45. Duel BP et al: Reconstructive options in genitourinary rhabdomyosarcoma. J Urol. 156(5):1798-804, 1996
                                                                                      46. Breen JL et al: Genital tract tumors in children. Pediatr Clin North Am. 28(2):355-67, 1981