Soft tissue sarcomas

Sarcomas are malignant tumours that arise from skeletal and extra-skeletal connective tissues, including the peripheral nervous system. The term ‘sarcomas of soft tissues’ embraces all of the malignant tumours which arise from the mesenchymal tissues excluding bone, i.e. malignant fibrous histiocytoma, liposarcoma, leiomyosarcoma, synovial sarcoma, rhabdomyosarcoma, epithelioid sarcoma, angiosarcoma, fibrosarcoma, etc. In addition, malignant tumours of peripheral nerve sheaths are included despite being ectodermal in origin, as their clinical behaviour is not measurably different from the other sarcomas. Gastrointestinal stromal tumours are derived from cells (interstitial cells of Cajal) with neural and smooth muscle features and are also considered soft tissue sarcomas. The relative frequency of different histological subtypes of sarcomas is shown in Table 40.1. This chapter discusses current practice in the management of patients with soft tissue sarcoma (other than rhabdomyosarcoma of the paediatric age group) as well as selected benign, infiltrative soft tissue tumours such as desmoids whose management is similar.