Peds

Rhabdomyosarcoma

search

Rhabdomyosarcoma

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