Incidence
- Usually 20 – 30 years
- Usually affects lower limbs
Clinically
- Rapidly growing swelling from tendon sheath or joint
- Knee the commonest site
- The most common sarcoma of the foot
- 90% have X;18 translocation
- 30% have lymph node and/or lung metastasis at presentation
X-Rays
- Bony and soft tissue defects show snowstorm appearance
- Spotty calcification in 25%
Pathology
- Unknown histogenesis, but involves soft tissues around the joint and extends along fascial planes
- Highly aggressive
- May invade bone
- Intra-articular synovial involvement is rare
- Histology divides into two types
- Biphasic with spindle cells and gland like spaces with better prognosis
- Anaplastic with no glands with high rate of local recurrence as well as metastases
Treatment
Combination therapy (radiation, chemotherapy and surgery)
Prognosis
- 55% 5 year survival
- 38% 10 year survival
- Prognostic features :
- Younger patients
- Female patients
- Size of tumour less than 5 cm
- Located on lower extremity
- Wide excision performed
- If all met, has better survival