Abstract
Soft-tissue sarcomas are uncommon and heterogeneous tumors of mesenchymal origin. A soft-tissue mass that is increasing in size, greater than 5 cm, or located under deep fascia are criteria for suspicion of sarcoma. Diagnosis, treatment, and management should preferably be performed by a multidisciplinary team in reference centers. MRI and lung CT scan are mandatory for local and distant assessment. A biopsy indicating histological type and grade is needed previous to the treatment. Wide surgical resection with tumor-free tissue margin is the primary treatment for localized disease. Radiotherapy is indicated in large, deep, high-grade tumors, or after marginal resection not likely of being improved with reexcision. Neoadjuvant and adjuvant chemotherapy improve survival in selected cases, usually in high-grade sarcomas of the extremities. In the case of metastatic disease, patients with exclusive lung metastasis could be considered for surgery. First-line treatment with anthracyclines (or in combination with ifosfamide) is the treatment of choice. New drugs have shown activity in second-line therapy and in specific histological subtypes.
Highlights
Soft-tissue sarcomas (STS) constitute an uncommon and heterogeneous group of tumors of mesenchymal origin, with an estimated incidence of five cases per 100,000 people per year in Europe
A review of abstracts of relevant, which do not published, yet phase III studies focused on STS therapy presented at international oncology meetings as the American Society of Clinical Oncology (ASCO), European Society of Medical Oncology (ESMO) and Connective Tissue Oncology Society (CTOS) meetings, in the recent years, was performed
The different sections were written by different responsible experts and after all the members discussed the results and determined the level of evidence and the grade for each recommendation according to ESMO guidelines
Summary
Soft-tissue sarcomas (STS) constitute an uncommon and heterogeneous group of tumors of mesenchymal origin, with an estimated incidence of five cases per 100,000 people per year in Europe. STS comprise different histopathological subtypes (more than 50 according the 2013 WHO classification), they share several clinical and pathological features and are usually considered as a group for diagnostic and therapeutic purposes, with the exception of specific particularities of some subtypes, such as rhabdomyosarcoma, gastrointestinal stromal tumors, extraosseus osteosarcoma, and Ewing’s sarcoma. STS can arise anywhere in the body, but most originate in the extremities, less frequently in the trunk, retroperitoneum, head and neck, and viscera. They can occur at any age, and more common in middle aged and older adults, they are seen in children and young adults
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