What changed: Desmoid tumour

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Progressing desmoid tumours

First systemic therapy for desmoid tumours (DeFi)

Progressing desmoid tumours requiring systemic treatment

DeFi phase 3 (NEJM 2023); FDA approval 27 November 2023, the first for desmoid tumours.

PFS HR 0.

Active surveillance with MRI at 1 to 2 months, then every 3 to 6 months; treat only on progression or symptoms. (NCCN Category 2A (observation))

Active surveillance as initial management for most patients (Eur J Cancer).

Gounder and colleagues, NEJM.

French Sarcoma Group and others show frequent spontaneous stabilisation, seeding the surveillance-first approach.

Tejpar and colleagues.

Named from the Greek for band-like.