Adult Soft Tissue Sarcoma (PDQ®): Treatment
Soft tissue sarcomas are malignant tumors that may arise in any of the mesodermal tissues of the extremities (50%), trunk and retroperitoneum (40%), or head and neck (10%). Rarely, these tumors arise in the gastrointestinal tract or gastrointestinal stroma. Soft tissue sarcomas occur with greater frequency in patients with:

• von Recklinghausen’s disease (neurofibromatosis).
• Gardner’s syndrome.
• Werner’s syndrome.
• Tuberous sclerosis.
• Basal cell nevus syndrome.
• Li-Fraumeni syndrome (p53 mutations).

Childhood Soft Tissue Sarcoma (PDQ®): Treatment
This cancer treatment information summary provides an overview of the prognosis, diagnosis, classification, and treatment of childhood soft tissue sarcoma.

This is a rare sarcoma but is important from the standpoint that under the microscope, it can mimic many benign and malignant conditions. As the name also suggests, it has microscopic features that can sometimes resemble a carcinoma. Unlike most sarcomas, it has a propensity to spread to lymph nodes.

Marco Ferrone, MD* Marc H. Isler, MD† Soft-tissue sarcoma involving the popliteal fossa remains challenging because it is difficult to achieve wide margins with limb salvage in this location. Adju- vant therapy is frequently necessary, and limb function can be adversely affected. We reviewed our experience with these tumours.
Despite the high rate of microscopically positive margins, the local recurrence rate was 7%. Amputation did not prevent death. We found function to be good to excellent in most patients who had limb-salvaging surgery.
R.E. Turcotte, M. Ferrone, M.H. Isler, C. Wong Can J Surg, Vol. 52, No. 1, February 2009 51

A late effect of ionizing radiation is the development of sarcoma within the field of irradiation, referred to as postradiation sarcoma (PRS). Ionizing radiation has had many varied uses in medicine. In early years, in addition to being used in the treatment of a variety of malignancies, radiation was used to treat benign conditions, such as acne, fungal infections, eczema, and various bone diseases.1, 2, 3, 4, 5, 6, 7, 8 Advances in cancer treatment in recent years have included intensive multiagent chemotherapy and irradiation.9 Despite significant medical use of radiation therapy, PRS is an uncommon tumor. The overall incidence of PRS is less than 1% for patients with cancer who are treated with radiation and survive 5 years.9 Although the implication for individual patients is significant, little doubt exists that the benefits of ionizing radiation far outweigh the potential risks of developing sarcomas.
Synonyms and related keywords: PRS, postirradiation sarcoma, radiation-induced sarcoma, osteosarcoma, fibrosarcoma, malignant fibrous histiocytoma, MFH, chondrosarcoma, angiosarcoma, Ewing sarcoma, malignant peripheral nerve sheath tumor, MPNST
Rao et al 2008