These ESMO–EURACAN–GENTURIS guidelines provide consensus-based management recommendations for approximately 80 WHO-defined soft tissue and visceral sarcoma entities. They cover the full treatment pathway: biopsy, pathological diagnosis, staging, local treatment, systemic therapy for advanced disease, and follow-up. Recommendations emphasize histotype-specific decision-making and management at specialist reference centers.
Any deep soft tissue mass, or a superficial lesion 5 cm or larger, requires referral to a sarcoma reference center before biopsy — outside-center diagnosis carries up to a 35% discordance rate, and an unplanned excision outside a specialist setting compromises the single best chance at curative en bloc resection.
When you have a localized high-grade STS, run the Sarculator nomogram. Predicted 10-year OS below 60% is your threshold to recommend neoadjuvant anthracycline-ifosfamide for at least 3 cycles. Preoperative RT should be favored over postoperative when margin preservation is the goal. It offsets the negative impact of R1 margins far more effectively than postoperative RT.
For second-line advanced disease, histotype drives drug selection: eribulin for liposarcoma (not LMS), pazopanib for non-adipogenic histotypes, trabectedin broadly from second-line with particular activity in myxoid liposarcoma and LMS.
For desmoid fibromatosis, resist the urge to operate or treat immediately. Active surveillance is the guideline-recommended first-line approach, and spontaneous regression is a real possibility.
These ESMO–EURACAN–GENTURIS guidelines provide consensus-based management recommendations for approximately 80 WHO-defined soft tissue and visceral sarcoma entities. They cover the full treatment pathway: biopsy, pathological diagnosis, staging, local treatment, systemic therapy for advanced disease, and follow-up. Recommendations emphasize histotype-specific decision-making and management at specialist reference centers.
Any deep soft tissue mass, or a superficial lesion 5 cm or larger, requires referral to a sarcoma reference center before biopsy — outside-center diagnosis carries up to a 35% discordance rate, and an unplanned excision outside a specialist setting compromises the single best chance at curative en bloc resection.
When you have a localized high-grade STS, run the Sarculator nomogram. Predicted 10-year OS below 60% is your threshold to recommend neoadjuvant anthracycline-ifosfamide for at least 3 cycles. Preoperative RT should be favored over postoperative when margin preservation is the goal. It offsets the negative impact of R1 margins far more effectively than postoperative RT.
For second-line advanced disease, histotype drives drug selection: eribulin for liposarcoma (not LMS), pazopanib for non-adipogenic histotypes, trabectedin broadly from second-line with particular activity in myxoid liposarcoma and LMS.
For desmoid fibromatosis, resist the urge to operate or treat immediately. Active surveillance is the guideline-recommended first-line approach, and spontaneous regression is a real possibility.