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KEY FACTS
Terminology
Imaging
Clinical Issues
TERMINOLOGY
Abbreviations
- Rhabdomyosarcoma (RMS)
Synonyms
- Embryonal RMS → malignant rhabdomyoma, RMS, myosarcoma, rhabdopoietic sarcoma, rhabdosarcoma, embryonal sarcoma, sarcoma botryoides
- Alveolar RMS → monomorphous round cell RMS, rhabdomyoblastoma, rhabdomyopoietic sarcoma
Definitions
- Malignant tumor showing skeletal muscle differentiation with 4 main subtypes: embryonal, alveolar, pleomorphic, and spindle cell/sclerosing
- Embryonal RMS (55-70% of RMSs)
- Malignant soft tissue tumor with morphologic and immunophenotypic features of embryonic skeletal muscle
- Botryoides "bunch of grapes" RMS is embryonal subtype arising below mucosal surface
- Alveolar RMS (18-45% of RMSs)
- Malignant neoplasm composed of monomorphic population of primitive round cells showing skeletal muscle differentiation
- Presence of either PAX3::FOXO1 or PAX7::FOXO1 fusion gene is detected in most cases
- Pleomorphic RMS
- High-grade pleomorphic sarcoma, usually of adults, composed of bizarre brightly eosinophilic polygonal, round, and spindle cells that display skeletal muscle differentiation
- Called adult-type RMS
- Spindle cell/sclerosing RMS (3-10% of RMSs)
- Type of RMS that has fascicular spindle cell &/or sclerosing morphology
- Embryonal RMS (55-70% of RMSs)
- Malignant tumor showing skeletal muscle differentiation with 4 main subtypes: embryonal, alveolar, pleomorphic, and spindle cell/sclerosing
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic 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
DIAGNOSTIC CHECKLIST
Consider
Reporting Tips
Selected References
- Rashid T et al: Advances in the diagnosis and management of neonatal sarcomas. Clin Perinatol. 48(1):117-45, 2021
- Yechieli RL et al: Rhabdomyosarcoma. Pediatr Blood Cancer. 68 Suppl 2:e28254, 2021
- Agaram NP et al: Spindle cell/sclerosing rhabdomyosarcoma. In Fletcher CDM et al: WHO Classification of Tumours: Soft Tissue and Bone Tumours. 5th ed. IARC Press. 211-3, 2020
- Inarejos Clemente EJ et al: MRI of rhabdomyosarcoma and other soft-tissue sarcomas in children. Radiographics. 40(3):791-814, 2020
- Kohashi K et al: Alveolar rhabdomyosarcoma. In Fletcher CDM et al: WHO Classification of Tumours: Soft Tissue and Bone Tumours. 5th ed. IARC Press. 205-8, 2020
- Montgomery EA et al: Pleomorphic rhabdomyosarcoma. In Fletcher CDM et al: WHO Classification of Tumours: Soft Tissue and Bone Tumours. 5th ed. IARC Press. 209-10, 2020
- Rudzinski ER: Embryonal rhabdomyosarcoma. In Fletcher CDM et al: WHO Classification of Tumours: Soft Tissue and Bone Tumours. 5th ed. IARC Press. 201-4, 2020
- Wasti AT et al: Rhabdomyosarcoma. Paediatr Child Health. 28(4):157-63, 2018. https://doi.org/10.1016/j.paed.2018.03.001
- 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
- Karunanithi S et al: Spectrum of physiologic and pathologic skeletal muscle (18)F-FDG uptake on PET/CT. AJR Am J Roentgenol. 205(2):W141-9, 2015
- Kransdorf MJ et al: Muscle tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Lippincott Williams & Wilkins. 379-91, 2014
- Saboo SS et al: Imaging features of primary and secondary adult rhabdomyosarcoma. AJR Am J Roentgenol. 199(6):W694-703, 2012
- National Cancer Institute: Childhood rhabdomyosarcoma treatment. Updated August 5, 2015. Accessed October 22, 2009. http://www.cancer.gov/cancertopics/pdq/treatment/childrhabdomyosarcoma/HealthProfessional/page6
- Weiss SW et al: Rhabdomyosarcoma. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 595-631, 2008
- 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
- Montgomery E et al: Pleomorphic rhabdomyosarcoma. In Fletcher CDM et al: WHO Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press. 153-4, 2002
- Parham DM et al: Alveolar rhabdomyosarcoma. In Fletcher CDM et al: WHO Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press. 150-2, 2002
- Parham DM et al: Embryonal rhabdomyosarcoma. In Fletcher CDM et al: WHO Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press. 146-9, 2002
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Clinical Issues
TERMINOLOGY
Abbreviations
- Rhabdomyosarcoma (RMS)
Synonyms
- Embryonal RMS → malignant rhabdomyoma, RMS, myosarcoma, rhabdopoietic sarcoma, rhabdosarcoma, embryonal sarcoma, sarcoma botryoides
- Alveolar RMS → monomorphous round cell RMS, rhabdomyoblastoma, rhabdomyopoietic sarcoma
Definitions
- Malignant tumor showing skeletal muscle differentiation with 4 main subtypes: embryonal, alveolar, pleomorphic, and spindle cell/sclerosing
- Embryonal RMS (55-70% of RMSs)
- Malignant soft tissue tumor with morphologic and immunophenotypic features of embryonic skeletal muscle
- Botryoides "bunch of grapes" RMS is embryonal subtype arising below mucosal surface
- Alveolar RMS (18-45% of RMSs)
- Malignant neoplasm composed of monomorphic population of primitive round cells showing skeletal muscle differentiation
- Presence of either PAX3::FOXO1 or PAX7::FOXO1 fusion gene is detected in most cases
- Pleomorphic RMS
- High-grade pleomorphic sarcoma, usually of adults, composed of bizarre brightly eosinophilic polygonal, round, and spindle cells that display skeletal muscle differentiation
- Called adult-type RMS
- Spindle cell/sclerosing RMS (3-10% of RMSs)
- Type of RMS that has fascicular spindle cell &/or sclerosing morphology
- Embryonal RMS (55-70% of RMSs)
- Malignant tumor showing skeletal muscle differentiation with 4 main subtypes: embryonal, alveolar, pleomorphic, and spindle cell/sclerosing
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic 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
DIAGNOSTIC CHECKLIST
Consider
Reporting Tips
Selected References
- Rashid T et al: Advances in the diagnosis and management of neonatal sarcomas. Clin Perinatol. 48(1):117-45, 2021
- Yechieli RL et al: Rhabdomyosarcoma. Pediatr Blood Cancer. 68 Suppl 2:e28254, 2021
- Agaram NP et al: Spindle cell/sclerosing rhabdomyosarcoma. In Fletcher CDM et al: WHO Classification of Tumours: Soft Tissue and Bone Tumours. 5th ed. IARC Press. 211-3, 2020
- Inarejos Clemente EJ et al: MRI of rhabdomyosarcoma and other soft-tissue sarcomas in children. Radiographics. 40(3):791-814, 2020
- Kohashi K et al: Alveolar rhabdomyosarcoma. In Fletcher CDM et al: WHO Classification of Tumours: Soft Tissue and Bone Tumours. 5th ed. IARC Press. 205-8, 2020
- Montgomery EA et al: Pleomorphic rhabdomyosarcoma. In Fletcher CDM et al: WHO Classification of Tumours: Soft Tissue and Bone Tumours. 5th ed. IARC Press. 209-10, 2020
- Rudzinski ER: Embryonal rhabdomyosarcoma. In Fletcher CDM et al: WHO Classification of Tumours: Soft Tissue and Bone Tumours. 5th ed. IARC Press. 201-4, 2020
- Wasti AT et al: Rhabdomyosarcoma. Paediatr Child Health. 28(4):157-63, 2018. https://doi.org/10.1016/j.paed.2018.03.001
- 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
- Karunanithi S et al: Spectrum of physiologic and pathologic skeletal muscle (18)F-FDG uptake on PET/CT. AJR Am J Roentgenol. 205(2):W141-9, 2015
- Kransdorf MJ et al: Muscle tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Lippincott Williams & Wilkins. 379-91, 2014
- Saboo SS et al: Imaging features of primary and secondary adult rhabdomyosarcoma. AJR Am J Roentgenol. 199(6):W694-703, 2012
- National Cancer Institute: Childhood rhabdomyosarcoma treatment. Updated August 5, 2015. Accessed October 22, 2009. http://www.cancer.gov/cancertopics/pdq/treatment/childrhabdomyosarcoma/HealthProfessional/page6
- Weiss SW et al: Rhabdomyosarcoma. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 595-631, 2008
- 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
- Montgomery E et al: Pleomorphic rhabdomyosarcoma. In Fletcher CDM et al: WHO Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press. 153-4, 2002
- Parham DM et al: Alveolar rhabdomyosarcoma. In Fletcher CDM et al: WHO Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press. 150-2, 2002
- Parham DM et al: Embryonal rhabdomyosarcoma. In Fletcher CDM et al: WHO Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press. 146-9, 2002
