Soft Tissue/Visceral Sarcomas Clinical Practice Guidelines (2018)

European Society for Medical Oncology and European Network for Rare Adult Solid Cancer

Reviewed and summarized by Medscape editors

June 07, 2018

The clinical practice guidelines on soft tissue/visceral sarcomas were released on May 28, 2018, by the European Society for Medical Oncology and the European Network for Rare Adult Solid Cancer.[1]

Management of Local/Locoregional Disease

Surgery is the standard treatment of all patients with an adult type, localised STS. It must be carried out by a surgeon specifically trained in the treatment of this disease. The standard surgical procedure is a wide excision with negative margins.

The typical wide excision is followed by RT as the standard treatment of high-grade (G2–3), deep, >5 cm lesions.

Options for limb-preserving surgery include ChT and/or RT, or isolated hyperthermic limb perfusion with tumor necrosis factor-alpha + melphalan, or regional hyperthermia combined with ChT.

Adjuvant ChT is not standard treatment in adult-type STS. It can be proposed as an option to the high-risk individual patient.

Neoadjuvant ChT with anthracyclines plus ifosfamide for at least 3 cycles is an option in the high-risk individual patient.

Management of Advanced/Metastatic Disease

Metachronous (disease-free interval ≥1 year), resectable lung metastases without extrapulmonary disease are managed with surgery as standard treatment, if complete excision of all lesions is feasible.

Standard ChT is based on anthracyclines as the first-line treatment. Multi-agent ChT with adequate-dose anthracyclines plus ifosfamide may be the treatment of choice, particularly in subtypes sensitive to ifosfamide, when a tumor response is felt to be potentially advantageous and patient performance status is good.

The combination of doxorubicin with an anti-PDGFRA (platelet-derived growth factor receptor alpha) agent, olaratumab, is an option.

Gemcitabine/docetaxel combination is not generally recommended as a first-line therapy for advanced STS patients.

Imatinib is standard medical therapy for those rare patients with dermatofibrosarcoma protuberans.

Trabectedin is an option for second line and beyond and is approved for advanced previously treated STS.

Pazopanib is an option in non-adipogenic STS.

Eribulin is an option in patients with liposarcomas and leiomyosarcomas.

The combination of dacarbazine and gemcitabine or gemcitabine/docetaxel is an option in doxorubicin-pretreated patients.

Regorafenib is an option in doxorubicin-pretreated advanced, non-adipogenic STS patients.


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