Gronchi, Ann Oncol 2021 - Soft Tissue and Visceral Sarcomas: ESMO–EURACAN–GENTURIS Clinical Practice Guidelines for Diagnosis, Treatment and Follow-Up - Article Summary

Soft Tissue and Visceral Sarcomas: ESMO–EURACAN–GENTURIS Clinical Practice Guidelines for Diagnosis, Treatment and Follow-Up

Gronchi, ESMO Guidelines Committee, EURACAN and GENTURIS. Electronic address: clinicalguidelines@esmo.org, et al.·Ann Oncol·2021·901 citations·Clinical guideline

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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.

Key Findings

Expert pathological re-review is mandatory when a sarcoma diagnosis is made outside a reference center. Discordance rates against sub-specialized sarcoma pathologists run 8–11% for major errors and 16–35% for minor errors — errors that redirect entire treatment pathways and underscore why unplanned outside excisions are so dangerous.
The Sarculator nomogram identifies a 10-year predicted OS below 60% as the threshold for recommending neoadjuvant anthracycline-ifosfamide chemotherapy in localized high-risk STS. Three cycles of neoadjuvant AI was shown non-inferior to five cycles in a randomized trial, making the shorter course the preferred approach [II, B].
Preoperative radiotherapy in resectable retroperitoneal sarcoma did not improve RFS or OS overall in a randomized trial [I, D], but showed efficacy signals specifically in the low-/intermediate-grade liposarcoma subgroup [II, B]. In resectable retroperitoneal LMS and high-grade dedifferentiated liposarcoma, preoperative RT should not be used.
Eribulin outperformed dacarbazine in liposarcoma and LMS with an overall median OS gain of 2 months, but the liposarcoma subgroup gained 7 months. The basis for its second-line regulatory approval specifically in liposarcoma [II, A; ESMO-MCBS 3]. The benefit was most pronounced in pleomorphic liposarcoma.
Pan-NTRK IHC in STS carries only ~80% sensitivity and ~75% specificity. Positive staining alone is not sufficient to start an NTRK inhibitor. Molecular confirmation of rearrangement is required before treatment. NTRK inhibitors (larotrectinib, entrectinib) are standard of care for confirmed NTRK-rearranged advanced sarcoma [III, A; ESMO-MCBS 3].
Pazopanib provides a median PFS benefit of ~3 months in previously treated, non-adipogenic advanced STS [I, B; ESMO-MCBS 3]. Liposarcomas were excluded from the pivotal trial. Pazopanib is not indicated in adipogenic histotypes.
Active surveillance is the recommended first-line strategy for all newly diagnosed primary desmoid-type fibromatosis without life-threatening presentation [III, A]. Treatment is reserved for progressing cases. Spontaneous regression occurs, and there is no metastatic potential.

Board PearlUse Sarculator to identify STS patients with predicted 10-year OS below 60% — these patients benefit from neoadjuvant anthracycline-ifosfamide chemotherapy.

Reference

Soft Tissue and Visceral Sarcomas: ESMO–EURACAN–GENTURIS Clinical Practice Guidelines for Diagnosis, Treatment and Follow-Up

Gronchi, ESMO Guidelines Committee, EURACAN and GENTURIS. Electronic address: clinicalguidelines@esmo.org, et al.Ann Oncol2021901 citationsClinical guideline