Sarcoma

Rhabdomyosarcoma

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Rhabdomyosarcoma, Rhabdosarcoma

  • See Also
  • Epidemiology
  1. Most common Soft Tissue Sarcoma in children
  2. Responsible for up to 8% of Childhood Cancer
  3. Typical onset in under age 10 years old (rare in adults)
  • Risk factors
  • Findings
  1. Presentation: Local swelling and soft tissue displacement
    1. Usually painless
    2. Compression Neuropathy (e.g. Cranial Nerve palsy)
    3. Central Nervous System involvement
  2. Distribution
    1. May be located anywhere
    2. Head and neck: 40%
    3. Genitourinary tract: 20%
    4. Extremities: 20%
    5. Trunk: 10%
  • 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)
  • Imaging
  1. Ultrasound
    1. Typical initial evaluation for Soft Tissue Mass in children
  • Management
  1. Refer to pediatric orthopedic oncology
  2. 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
  3. Adjunctive management
    1. Chemotherapy
    2. Radiation
    3. Further surgical excision
  • References