SARCOMAS – GENERAL INFORMATION

SARCOMAS – GENERAL INFORMATION

GENERAL INFORMATION

Sarcoma is a heterogenous group of malignancies, derived from mesenchymal tissues of our organism (muscles, bones, fat, vases, cartilages and etc.…), and they are ubiquitous, as they can arise at any part of the organism.

This family encompasses more than 80 different histologic subtypes, but, as a whole, they constitute only 1-2% of all the malignant neoplasm of the adult.

Sarcomas are classically divided in 3 subgroups: bone sarcoma, soft-tissue sarcoma (STS) and gastrointestinal stromal sarcomas (GIST).

Sarcomas low incidence, wide spectrum of histologic subtypes, biological behaviors and ubiquity, make sarcoma management challenging. For these reasons, their management has to be done in a multidisciplinary setting with proper expertise teams In the majority of cases, sarcomas are of unknown cause, although there are a small percentage of cases related with several predisposing factors such as: radiation, viral infections, hereditary syndromes (Li Fraumeni, Retinoblastoma, and Neurofibromatosis, among others) and previous diseases or conditions (Paget disease, chronic lymphedema).

Approximately 75-80% of sarcomas are localized at the time of diagnosis, and therapeutic planning with a curative intent is fundamental in this situation.

A correct diagnosis is essential for a proper clinical management. A diagnostic biopsy previous to surgical treatment is mandatory in suspected cases of sarcoma. Unplanned surgeries in STS have several consequences: a greater probability of surgical affected margins and therefore, a contamination of the surgical bed. In the short term, they generate greater morbidity since they require re-excision, a greater field of irradiation and a potential greater functional impairment. In the long-term, unplanned surgeries correlate with: poorer prognosis, greater probability of local relapses and , worst survival, in series with adequate follow-up

In all clinical practice guidelines, it is recommended that patients with suspected sarcomas have to be referred to expert centers. This is due to the observation of a better outcome for these patients managed in centers with multidisciplinary teams and expertise, when compared with patients managed in non-expert centers.