Soft tissue sarcomas are rare mesenchymal tumours that originate from the connective and muscular structures. The parts of the body most affected are the limbs in 59% of cases, followed by the retroperitoneum in 15%, trunk 10%, head and neck 5%, visceral areas (oesophagus, intestine, stomach, uterus, et cetera) 10%. They form around 1% of adult cancers, with a prevalence in males, the incidence is around 5 cases a year for every 100,000 people. There are peaks in paediatric age and after six years.
More than 70 different isotypes of soft tissue sarcoma are known, classified according to morphology.
A patient treated at our institution may receive a conventional treatment, as part of consolidated guidelines, or a custom treatment, depending on the individuality of each individual case. There is still the possibility of receiving a treatment as part of the clinical study (for example on a new drug or a new innovative therapeutic strategy).
From a methodological point of view, all clinical decisions are discussed in a multidisciplinary environment, sharing the case between surgeons, oncologists, radiotherapists, thoracic surgeons, plastic reconstructive surgeons, physiotherapists, cancer psychologists, radiologists and pathologists.
The primary curative treatment of sarcomas today is surgery, preferably carried out in centres of reference, in order to apply the due oncological indications and through the greater experience. Surgery can be integrated with chemotherapy and radiotherapy. In particular, resulting to radiotherapy enables obtaining control of the disease, with good results in 85-90% of cases with good disease stabilisation for years.
Often the therapy includes multiple treatment methods, sequentially through the treatment programme, and which consist of surgery, radiotherapy and chemotherapy. These can be combined in different modes and therefore the treatment methods vary according to the disease characteristics. It is therefore essential to attend specialist centres for the management of sarcoma. The heterogeneity of the anatomical presentation is one of the greatest difficulties and the fact that it can arise anywhere means that surgical intervention may also involve all anatomical areas and the collaboration of various surgical specialists.
According to the type of intervention, the site, the isotype, the grading, examination, the surgery will be performed following local radiotherapy. The radiotherapy, i.e. the application of radiation capable of killing cancer cells, is performed on precise sites, with targeted dosage and according to methods which vary by the type of cancer, as not all sarcomas respond in the same way. Lastly, in relation especially to the staging of the disease, chemotherapy is used, which involves intravenous infusion or oral administration of drugs that can select and kill cancer cells. The most active drugs are represented by doxorubicin (or epirubicin), ifosfamide, in soft tissue sarcomas, to which other drugs can be added such as trabectedin, taxanes and more besides.
- Diagnostic radiology, for defining the local extension and morphological characteristics
- Pathological and molecular diagnosis, for defining the class of risk
- Local treatment, neoadjuvant and adjuvant treatment
- Treatment of advanced disease
- Second opinion