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
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
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
Maldonado FR et al: Quantitative characterization of extraocular orbital lesions in children using diffusion-weighted imaging. Pediatr Radiol. 51(1):119-27, 2021
Rogers TN et al: Management of rhabdomyosarcoma in pediatric patients. Surg Oncol Clin N Am. 30(2):339-53, 2021
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
Navarro OM: Pearls and pitfalls in the imaging of soft-tissue masses in children. Semin Ultrasound CT MR. 41(5):498-512, 2020
Harrison DJ et al: The role of 18F-FDG-PET/CT in pediatric sarcoma. Semin Nucl Med. 47(3):229-41, 2017
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
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
Federico SM et al: Comparison of PET-CT and conventional imaging in staging pediatric rhabdomyosarcoma. Pediatr Blood Cancer. 60(7):1128-34, 2013
Thacker MM: Malignant soft tissue tumors in children. Orthop Clin North Am. 44(4):657-67, 2013
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
Navarro OM: Soft tissue masses in children. Radiol Clin North Am. 49(6):1235-59, vi-vii, 2011
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
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
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
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
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
Sebire NJ et al: Myogenin and MyoD1 expression in paediatric rhabdomyosarcomas. J Clin Pathol. 56(6):412-6, 2003
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
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References
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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
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
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
Maldonado FR et al: Quantitative characterization of extraocular orbital lesions in children using diffusion-weighted imaging. Pediatr Radiol. 51(1):119-27, 2021
Rogers TN et al: Management of rhabdomyosarcoma in pediatric patients. Surg Oncol Clin N Am. 30(2):339-53, 2021
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
Navarro OM: Pearls and pitfalls in the imaging of soft-tissue masses in children. Semin Ultrasound CT MR. 41(5):498-512, 2020
Harrison DJ et al: The role of 18F-FDG-PET/CT in pediatric sarcoma. Semin Nucl Med. 47(3):229-41, 2017
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
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
Federico SM et al: Comparison of PET-CT and conventional imaging in staging pediatric rhabdomyosarcoma. Pediatr Blood Cancer. 60(7):1128-34, 2013
Thacker MM: Malignant soft tissue tumors in children. Orthop Clin North Am. 44(4):657-67, 2013
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
Navarro OM: Soft tissue masses in children. Radiol Clin North Am. 49(6):1235-59, vi-vii, 2011
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
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
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
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
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
Sebire NJ et al: Myogenin and MyoD1 expression in paediatric rhabdomyosarcomas. J Clin Pathol. 56(6):412-6, 2003
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
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