Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up
- Study type
- Clinical guideline
- Study name
- ESMO STS 2021
- Design
- ESMO clinical practice guideline developed with EURACAN and GENTURIS at a virtual expert consensus meeting (5 December 2020); levels of evidence I to V and grades of recommendation A to E
- Source
- Ann Oncol · 2021
In plain words
This guideline is the official summary of European experts on soft tissue sarcoma: imaging and a needle biopsy before surgery, review of the sample by a specialist pathologist, complete surgery at a specialist sarcoma centre often combined with radiotherapy, and chemotherapy for disease that has spread or is high risk. Each patient's treatment should be planned by a multidisciplinary team according to the exact type of sarcoma.
Key points
Key guideline recommendations
- III, A
Management of soft tissue sarcoma should be carried out in sarcoma reference centres or tertiary paediatric oncology centres, as appropriate for the patient's age.
- IV, A
Pathological diagnosis should be made by a sarcoma expert pathologist according to the 2020 WHO classification.
- IV, A
Available staging classifications (UICC-AJCC) are of limited clinical value; risk assessment is better obtained through the available nomograms.
- IV, A
Staging is routinely carried out with contrast-enhanced chest, abdomen and pelvis CT; whole-body MRI may be an alternative, especially in selected histotypes; brain CT or MRI only in alveolar soft part sarcoma, clear-cell sarcoma and angiosarcoma; FDG-PET/CT is a problem-solving tool in equivocal cases.
- IV, A
The surgical report should include the preoperative and intraoperative diagnosis, possible contamination or tumour rupture, completeness of resection and the planned quality of microscopic margins.
- IV, A
TP53 testing should be carried out in selected patients with soft tissue sarcoma under the age of 46.
- II, A
Surgery is the standard treatment for all patients with an adult-type, localised soft tissue sarcoma and must be carried out by a surgeon specifically trained in treating sarcoma; the standard procedure is an en bloc wide excision with R0 margins.
- II, B
Wide excision plus radiotherapy is the standard treatment of high-grade (G2–3) lesions; the sequence varies between institutions, but there is an overall shift towards preoperative radiotherapy, especially when preserving a critical structure is a goal. Radiotherapy is omitted only after multidisciplinary discussion in reference centres.
- III, A; III, A; I, B
Options for limb-preserving surgery include chemotherapy and/or radiotherapy [III, A], isolated limb perfusion [III, A] or regional hyperthermia combined with chemotherapy [I, B].
- II, B
Adjuvant or neoadjuvant anthracycline plus ifosfamide chemotherapy for at least 3 cycles can be proposed to patients at high risk of death.
- IV, B
The standard treatment of metachronous (disease-free interval of 1 year or more), resectable lung metastases without extrapulmonary disease is surgery, if complete excision of all lesions is feasible.
- I, B
Standard chemotherapy is anthracycline-based in the first line [I, A]. Multi-agent chemotherapy with adequate-dose AI or dacarbazine may be the treatment of choice, particularly in subtypes sensitive to ifosfamide or dacarbazine, when a tumour response is felt to be advantageous and performance status is good.
- I, D
Gemcitabine–docetaxel is not generally recommended as first-line therapy for advanced soft tissue sarcoma.
- III, A
Imatinib is the standard medical therapy for dermatofibrosarcoma protuberans, and NTRK inhibitors are the standard treatment of advanced NTRK-rearranged sarcomas.
- I, B
Trabectedin is an option in second and further lines for advanced soft tissue sarcoma (ESMO-MCBS: 2), and pazopanib is an option in non-adipogenic sarcoma in second and further lines (ESMO-MCBS: 3).
- II, A
Eribulin is an option in liposarcoma (ESMO-MCBS: 3); dacarbazine–gemcitabine or gemcitabine–docetaxel is an option after doxorubicin [II, B].
Study population
Adults with soft tissue and visceral sarcomas (about 80 entities), including sarcomas of the limbs, trunk, retroperitoneum and uterus and desmoid-type fibromatosis. GIST, extraskeletal Ewing sarcoma and related round cell sarcomas are covered by separate guidelines, and Kaposi sarcoma and embryonal and alveolar rhabdomyosarcoma are not discussed.
Endpoints
This is a guideline with no predefined endpoints; recommendations carry a level of evidence (I to V), a grade of recommendation (A to E) and, for new therapies, an ESMO-MCBS score.
Safety and side effects
The guideline stresses the toxicity of radiotherapy (more wound complications with preoperative radiotherapy and more fibrosis, oedema, fractures and joint stiffness with postoperative radiotherapy) and of chemotherapy; in TP53 carriers, radiotherapy should be avoided where possible.
Clinical significance and place in guidelines
This is the main European reference for soft tissue and visceral sarcoma. It emphasises centralised care in reference centres, a planned core needle biopsy, expert pathology review, en bloc R0 surgery with radiotherapy for high-grade lesions, selection of high-risk patients for AI chemotherapy with a nomogram (Sarculator), anthracyclines first line in advanced disease, later-line treatments (trabectedin, pazopanib, eribulin, gemcitabine combinations) and molecular therapies (imatinib in DFSP, NTRK inhibitors).
Limitations
Many recommendations rest on level III to V evidence and expert consensus, because randomised trials in sarcoma are rare. Some of the drugs mentioned are not approved or reimbursed in all European countries. The guideline was developed in December 2020 and later evidence (such as the ESTRO 2026 radiotherapy guideline) is not included.
This summary was prepared by the editorial team from the published paper; the original paper is the final reference. Read the original abstract on PubMed.