INFORMACIÓN GENERAL
Sarcomas are a heterogeneous group of malignant tumours, derived from the mesenchymal tissues of our body (muscles, bones, fat, vessels, cartilage, etc. ….), and are ubiquitous, as they can arise in any part of the body.
This family encompasses more than 80 different histological subtypes, but together they constitute only 1-2% of all adult malignancies.
Sarcomas are classically divided into three subgroups: bone sarcoma, soft tissue sarcoma (STS) and gastrointestinal stromal sarcomas (GIST).
The low incidence of sarcomas, their wide spectrum of histological subtypes, their biological behaviour and their ubiquity make their management difficult. Therefore, their management must be performed in a multidisciplinary setting with specialised teams. In most cases, sarcomas are of unknown cause, although there is a small percentage of cases related to various predisposing factors, such as radiation, viral infections, hereditary syndromes (Li-Fraumeni, retinoblastoma and neurofibromatosis, among others) and previous diseases or conditions (Paget’s disease, chronic lymphoedema).
Approximately 75-80% of sarcomas are localised at the time of diagnosis, and therapeutic planning with curative intent is essential in this situation.
A correct diagnosis is essential for proper clinical management. A diagnostic biopsy prior to surgical treatment is mandatory in suspected sarcoma cases. Unplanned surgery in soft tissue sarcomas (STS) has several consequences: increased likelihood of affected surgical margins and thus contamination of the surgical site. In the short term, they lead to increased morbidity, as they require re-excision, a larger field of irradiation and possible further functional impairment. In the long term, unplanned surgery correlates with worse prognosis, increased likelihood of local relapse and worse survival in series with adequate follow-up.
In all clinical practice guidelines, it is recommended that patients with suspected sarcoma be referred to specialised centres. This is because a better outcome has been observed in these patients treated in centres with multidisciplinary teams and expertise, compared to patients treated in non-specialised centres.
