II. Definitions
- Sarcoma
- Cancer arrising in mesenchymal (connective) tissue
- Tumors may originate from bones, fat, Muscle, vessels or Lymphatics
- Derived from greek word "sar" meaning fleshy
III. Epidemiology
- Age
- More common in adults over age 35 years (but represent <1% of adult cancers)
- Sarcomas represent 10-15% of childhood malignancy (esp. in extremities)
- Soft Tissue Sarcomas are the sixth most common Childhood Cancer (esp. rhabomyosarcoma)
IV. Types
- Soft-tissue Sarcomas
- Kaposi's Sarcoma
- Hepatic angiosarcoma
- Dermatofribosarcoma Protuberans
- Synoviosarcoma
- Rhabdomyosarcoma
- Most common Soft Tissue Sarcoma in children (esp. age <8 years old)
- Account for 60% of pediatric tumors of the head and neck
- Leiomyosarcoma
- Involves Uterus in adults
- Involves Retroperitoneum in children
- Liposarcoma
- Rare adipose tumor (usually invades locally only)
- Involves deep thigh or Retroperitoneum
- Fibrosarcoma
- Most common Soft Tissue Sarcoma in age <1 year
- Involves deep thigh or Retroperitoneum
- Vascular Sarcoma
- Angiosarcoma (Stewart-Treves Syndrome)
- Malignancy of blood vessels and lymph vessels
- Presents with red to violaceous skin Papules
- May invade deeper tissue with tumor growth, leading to ulceration, edema, bleeding
- Hemangioendothelioma
- Hemangiopericytoma
- Angiosarcoma (Stewart-Treves Syndrome)
- Bone Tumors
V. Precautions
- See Soft Tissue Mass for red flag findings
- Delayed presentation is common (often >1 month from finding a Soft Tissue Mass)
- Most Soft Tissue Sarcomas gradually enlarge without pain or constitutional symptoms
VI. Evaluation
- See Soft Tissue Mass (includes red flag findings and imaging)
VII. Prognosis
- Five year survival: 60%
- Ranges between Stage 1 (five year survival 80%) and Stage 4 (five year survival 20%)
VIII. References
- Hanback and Kosoko (2023) Crit Dec Emerg Med 37(4): 14-5
- Achar (2022) Am Fam Physician 105(6): 602-12 [PubMed]