1: Soft Tissue Sarcoma

Soft tissue sarcomas are mesenchymal tumours that do not arise from bone or cartilage. They are more common than bone sarcomas. There is a broad spectrum of pathological types which have been poorly differentiated in the past. The most common site is the lower limbs, followed by the trunk, the upper limbs, and finally the head and neck. A special exception is in children, where head and neck rhabdomyosarcoma commonly occurs in the head and neck region.

General Principles

Surgery is the mainstay of treatment and should be performed where possible for cure, if the disease is localised. Radiotherapy has a role in most cases, except for low grade sarcomas that are less than 5 cm in size of the extremities. Chemotherapy has a role in the paediatric sarcomas (rhabdomyosarcoma) but the benefit in other sarcomas is controversial (4% overall survival benefit at 10 years on meta-analysis).

Soft Tissue Sarcoma of the Limbs

The limbs are the most common site for soft tissue sarcoma (60%), and two thirds of cases occur in the lower limbs.

  • Surgery: Surgery is the established treatment against which newer developments are tested. Although amputation was popular 30-40 years ago, it has been replaced by limb salvage operations. Margins should be > 1 cm where possible or > 0.5 cm if radiotherapy is used adjuvantly.
  • Radiotherapy: Radiotherapy has an important role to play in the treatment of soft tissue sarcoma of the extremities, excluding low grade, completely resected tumours. Radiotherapy was shown adjuvantly to reduce the risk of local recurrence after limb sparing surgery in a randomised trial. A randomised Canadian trial demonstrated reduced long term toxicity with neoadjuvant therapy over adjuvant treatment, and this is now 'standard of care' (50.4 Gy in 28 fractions).
  • Chemotherapy: Chemotherapy is a controversial topic. A number of small randomised trials, and meta-analyses of these, suggest a survival benefit from doxorubicin/ifosfamide. However, the largest studies show no impact on survival and were not included in the meta-analysis. Chemotherapy is often an individual decision for each patient, taking into account their risk factors and co-morbidities.

Soft Tissue Sarcoma of the Retroperitoneum

Vascular Sarcomas

Rare Soft Tissue Sarcomas