II. Epidemiology
- Most common Soft Tissue Sarcoma in children
- Responsible for up to 8% of Childhood Cancer
- Typical onset in under age 8 to 10 years old (rare in adults)
- Accounts for 60% of pediatric tumors of the head and neck
III. Risk Factors
- Neurofibromatosis
- Li-Fraumeni Syndrome
- RAS Gene Family
- RAS genes encode cell signaling pathway Proteins that control cell growth and death
IV. Findings
- Presentation: Local swelling and soft tissue displacement
- Usually painless
- Compression Neuropathy (e.g. Cranial Nerve palsy)
- Central Nervous System involvement
- Distribution
- May be located anywhere
- Head and neck: 40%
- Genitourinary tract: 20%
- Extremities: 20%
- Trunk: 10%
V. Labs: Histologic Types
-
General findings
- Primitive oval to spindle shaped cells with minimal cytoplasm
- Areas of small, round blue cells
- 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)
VI. Imaging
-
Ultrasound
- Typical initial evaluation for Soft Tissue Mass in children
VII. Management
- Refer to pediatric orthopedic oncology
- 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
VIII. References
- Crist in Behrman (2000) Nelson Pediatrics, p. 1557-8
- Hanback and Kosoko (2023) Crit Dec Emerg Med 37(4): 14-5
- Achar (2022) Am Fam Physician 105(6): 602-12 [PubMed]
- Andrassy (2002) Am J Surg 184:484-91 [PubMed]