SEOM Clinical Guideline of management of soft-tissue sarcoma (2016)

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.

Evidence mapping based on systematic reviews of therapeutic interventions for soft tissue sarcomas

“…For this reason, the evidence base in this area is weak, as non-experimental designs include information about treatment effects which may probably be biased or unreliable [41]. For instance, although some clinical practice guidelines (CPGs) recommend the combination of doxorubicin and ifosfamide as the most effective first-line chemotherapy for patients with locally advanced unresectable and/or metastatic STS [42], the authors of two SRs report this option as "inconclusive". Likewise, one recent randomised controlled trial published did not observe any effect on overall survival and its use would only be justified if the specific therapeutic goal is tumour shrinkage [43].…”

In Reply

“…Regarding the last comment, ESMO‐EURACAN soft‐tissue sarcoma guidelines , recently updated, state “Management should be carried out in reference centres for sarcomas and/or within reference networks sharing multidisciplinary expertise and treating a high number of patients annually.” This is stated in this and other guidelines given the evidence showing a better outcome for those patients managed in centers with expertise . This expertise in such a rare group of diseases cannot be achieved in centers managing few cases per year.…”

One-bloc percutaneous large biopsy of soft-tissue tumours: feasibility study and possible indications

“…2,3 Biopsy is a prerequisite for optimal management. 4,5 The completeness of initial surgery (R0) is the strongest favourable prognostic factor. The surgery is often extensive, complying with specific oncology rules in order to avoid recurrence.…”