Novel Insights into Soft Tissue Sarcoma

Dear Colleagues,

Soft tissue sarcomas (STSs) comprise 1% of adult malignant tumors (7% of childhood malignancies). Sarcomas encompass a heterogeneous group of malignancies of mesenchymal origin and may occur in any site of the body, which represents an additional challenge when it comes to therapy. They are a heterogeneous group of rare tumors, with more than 50 histological subtypes of STSs (WHO classification of tumors) having been identified, of which the most common in adults are malignant fibrous histiocytoma (28%), leiomyosarcoma (12%), liposarcoma (15%), synovial sarcoma (10%), and malignant peripheral nerve sheath tumors (6%). While the prognosis is good for patients diagnosed at an early stage and treated by adequate surgery, unresectable or metastatic diseases shrink the overall survival at 5 years to less than 10%, creating an unmet medical need.

The medical treatment of adult soft tissue sarcomas is more and more dictated by the histological subtype; this applies to both cytotoxic and target therapies. Anthracycline- and ifosfamide-based chemotherapies are the main therapeutic agents in the neoadjuvant, adjuvant, and metastatic adult-type STS disease settings, primarily for high-grade tumors, with the best-established response rates in adult soft tissue sarcomas for several years. In addition to this compound, there is evidence of efficacy of new drugs. Based on our current knowledge, 5–10% of all malignancies are part of hereditary cancer syndromes. Although the increasing diagnostic role of molecular genetic testing make us able to recognize more hereditary cancer patients, the careful exploration of family and clinical history by physicians is still the most important step for the diagnosis. Sarcomas are only 1% of all malignancies, but they often associate with familiar diseases so they can serve as an indicator of these syndromes. The diagnosis of hereditary cancer predisposition syndromes is essential to ensure appropriate therapy and follow-up for our patients. Multidisciplinary approach is mandatory in all cases (involving pathologist, radiologist, surgeons, radiation therapists, medical oncologists), and it should be carried out in reference centers for sarcomas.

Prof. Dr. Zsuzsanna Pápai
Guest Editor

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