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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
- Embryonal rhabdomyosarcoma (RMS) (60-70% of childhood RMS)
- Mesenchymal sarcoma arising from rhabdomyoblasts (primitive muscle cells); lacks normal differentiation into skeletal muscle
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
- 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
- Stein-Wexler R: Pediatric soft tissue sarcomas. Semin Ultrasound CT MR. 32(5):470-88, 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, 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
- 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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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
- Embryonal rhabdomyosarcoma (RMS) (60-70% of childhood RMS)
- Mesenchymal sarcoma arising from rhabdomyoblasts (primitive muscle cells); lacks normal differentiation into skeletal muscle
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
- 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
- Stein-Wexler R: Pediatric soft tissue sarcomas. Semin Ultrasound CT MR. 32(5):470-88, 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, 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
- 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
