Expert opinion document on the management of desmoid tumours in adult patients in Spain.

Authors

Rosa María Álvarez Álvarez, Javier Zenón Martín Broto, Javier Martínez Trufero, Ana Sebio García, Claudia María Valverde Morales, Jorge García López, Celia Martín Machín, Alicia Gil

Published in

Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico. Jul 11, 2026. Epub Jul 11, 2026.

Abstract

Desmoid tumours (DT), or aggressive fibromatosis are rare, nonmetastatic soft tissue neoplasms characterized by monoclonal fibroblast proliferation and infiltrative local behaviour that can affect organs and adjacent structures, resulting in sustained clinical burden and impacting patient's quality of life.
The aim of this work is to determine the current management of DT in Spain, including unmet needs and to propose the positioning of nirogacestat in the current treatment algorithm.
A literature review was conducted in June 2025 to identify published evidence on the epidemiology, diagnosis, management and unmet needs of DT in Spain. An expert panel of five Spanish oncologists specialized in sarcomas was convened. Each expert completed a questionnaire relating to research topics. Responses were consolidated and analysed, with a consensus threshold set at 80%. Findings were discussed and consensus reached at two virtual meetings.
The therapeutic approach to DT is currently based on an individualized strategy according to symptoms and tumour's biological behaviour. In asymptomatic patients, active surveillance represents the first recommended strategy, given the potential for spontaneous tumour stabilization or regression. Symptomatic, functionally limiting, or documented progression cases require active treatment. The current unmet needs include lack of systemic treatments with approved indication and favourable safety profile. Nirogacestat, the first treatment authorised for patients with progressing DT who require systemic therapy, should be positioned as standard of care in the treatment algorithm.
This first Spanish expert opinion document on DT management addresses evidence gaps and proposes a treatment algorithm to harmonise clinical practice.

PMID:
42435159
Bibliographic data and abstract were imported from PubMed on 12 Jul 2026.

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