Sarcoma

Rhabdomyosarcoma

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Rhabdomyosarcoma, Rhabdosarcoma

  • See Also
  1. Sarcoma
  • Epidemiology
  1. Most common Soft Tissue Sarcoma in children
  2. Responsible for up to 8% of Childhood Cancer
  3. Typical onset in under age 8 to 10 years old (rare in adults)
  4. Accounts for 60% of pediatric tumors of the head and neck
  • Risk Factors
  1. Neurofibromatosis
  2. Li-Fraumeni Syndrome
  3. RAS Gene Family
    1. RAS genes encode cell signaling pathway Proteins that control cell growth and death
  • 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%
  • Labs
  • Histologic Types
  1. General findings
    1. Primitive oval to spindle shaped cells with minimal cytoplasm
    2. Areas of small, round blue cells
  2. Embroyonal (60%)
    1. Intermediate prognosis
  3. Alveolar (15%)
    1. Named for alveoli-like clefts
    2. Usually involves trunk or extremities
    3. Worst prognosis of Rhabdomyosarcomas
  4. Botryoid (6%)
    1. Forms grap-like clusters
    2. Tumors of nasopharynx and genitourinary tract
  5. 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
  1. Crist in Behrman (2000) Nelson Pediatrics, p. 1557-8
  2. Hanback and Kosoko (2023) Crit Dec Emerg Med 37(4): 14-5
  3. Achar (2022) Am Fam Physician 105(6): 602-12 [PubMed]
  4. Andrassy (2002) Am J Surg 184:484-91 [PubMed]