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Rhabdomyosarcoma
Aka: Rhabdomyosarcoma
- See Also
- Sarcoma
- Epidemiology
- Most common soft tissue Sarcoma in children
- Responsible for up to 8% of Childhood Cancer
- Distribution
- May be located anywhere
- Head and neck: 40%
- Genitourinary tract: 20%
- Extremities: 20%
- Trunk: 10%
- Risk factors
- Neurofibromatosis
- Presentation: Local swelling and soft tissue displacement
- Usually painless
- Compression Neuropathy (e.g. Cranial Nerve palsy)
- Central Nervous System involvement
- Histologic Types
- Embroyonal (60%)
- Intermediate prognosis
- Alveolar (15%)
- Named for alveoli-like clefts
- Usually involves trunk or extremities
- Worst prognosis of Rhabdomyosarcomas
- Botryoid (6%)
- Forms grap-like clusters
- Tumors of nasopharynx and genitourinary tract
- Pleomorphic (1% in children, usually seen in adults)
- Management
- Complete local excision if possible
- Directed by site and adjacent structures
- More limited excision at orbit, genitourinary tract
- Regional Lymph Node sampling is also performed
- Adjunctive management
- Chemotherapy
- Radiation
- Further surgical excision
- References
- Crist in Behrman (2000) Nelson Pediatrics, p. 1557-8
- Andrassy (2002) Am J Surg 184:484-91 [PubMed]