Referral patterns, prognostic models and treatment in soft tissue sarcomas

Soft tissue sarcomas are cancers of supporting tissues in the body. The incidence is low, but rises, with 750 new patients in 2017 in the Netherlands. Because of low prevalence and heterogeneous clinical behavior, sarcoma patients should be referred to one of the sarcoma centers for consultation and/or treatment. Recognizing sarcomas is difficult and leads to considerable delays in making the diagnosis of the patient and doctor. An adequate training of doctors and an efficient diagnostic process in the hospital are of vital importance.
Individualized oncology treatment is on the rise. Proliferation markers can contribute to differentiating low and high risk sarcoma patients for whom additional therapy - chemotherapy, radiation and/or systemic therapy - is necessary to reduce the chance of recurrence and/or improve survival.
For locally advanced sarcomas of the extremities, combined therapy with isolated regional perfusion with chemotherapy and/or radiation is used to reduce the vital tumor and enable local resection, thereby avoiding amputation in approximately 85% of patients.
Angiosarcomas and desmoid-type fibromatosis are rare types of sarcomas that require special surgical approaches. Surgery with large margins is the cornerstone for local aggressively growing and metastatic angiosarcomas. In the only locally infiltrating desmoid-type fibromatosis tumors, smaller surgical margins suffice and observation with intensive follow-up seems justified.
The optimization in the treatment of soft tissue sarcomas has been achieved in recent years through more insight into the biological behavior, centralization and the personalized treatment of these rare tumors.