Abstract
Soft-tissue sarcomas constitute an uncommon and heterogeneous group of tumors of mesenchymal origin. Diagnosis, treatment, and management should be performed by an expert multidisciplinary team. MRI/CT of the primary tumor and biopsy is mandatory before any treatment. Wide surgical resection with tumor-free tissue margin is the mainstay for localized disease. Radiotherapy is indicated in large, deep, high-grade tumors, or after marginal resection not suitable for re-excision. Perioperative chemotherapy should be discussed for high-risk sarcomas of the extremities and trunk-wall. In the case of oligometastatic disease, patients should be considered for local therapies. First-line treatment with anthracyclines (or in combination with ifosfamide) is the treatment of choice. Other drugs have shown activity in second-line therapy and in specific histological subtypes but options are limited and thus, a clinical trial should always be discussed.
Highlights
Soft-tissue sarcomas (STS) constitute an uncommon and heterogeneous group of tumors of mesenchymal origin, with an estimated incidence of 5 cases/100,000 inhabitants/yearAll authors have contributed to the present manuscript and are GEIS members.1 3 Vol:.(1234567890)Clinical and Translational Oncology (2021) 23:922–930 several clinical and pathological features but have some specific characteristics that may impact the treatment.The main objective of this guideline is to provide updated and clear practical recommendations about the management of STS and to contribute to the improvement of STS patient’s care in Spain
R0 Surgery is the mainstay of treatment (II, A)
Its combination with ifosfamide increased the response rate and progression-free survival in sensitive histological types, it increased toxicity and it didnt significantly improve survival in randomized trials (14.3 vs. 12.8 months, HR 0.83) [13] and its reserved for patients who may benefit from tumor reduction for symptom palliation or improving resectability (I, B)
Summary
Soft-tissue sarcomas (STS) constitute an uncommon and heterogeneous group of tumors of mesenchymal origin, with an estimated incidence of 5 cases/100,000 inhabitants/year. All authors have contributed to the present manuscript and are GEIS members. The main objective of this guideline is to provide updated and clear practical recommendations about the management of STS and to contribute to the improvement of STS patient’s care in Spain. Some subtypes, such as rhabdomyosarcoma, gastrointestinal stromal tumors, extraosseus osteosarcoma, and Ewing’s sarcoma are beyond the focus of this guideline because of their specific and differential management
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