The Desmoid Tumor Research Foundation

July 2021

Desmoid fibromatosis: is the current picture changing?

Author Information

(1) Pandrowala S, (2) Jones RL, (3) Gupta S, (1) Gulia A.

(1) Dept of Surgical Oncology, Bone & Soft Tissue Service, Tata Memorial Hospital and Homi Bhabha National Institute (HBNI), Mumbai, 400012, India, (2) Dept of Medicine, Sarcoma Unit, Royal Marsden Hospital, Fulham Road, London, SW3 6JJ, UK, .(3) Dept of Musculoskeletal Oncology, Glasgow Royal Infirmary, Scotland, UK.

Publication

Future Oncol

Summary

Desmoid fibromatosis is a locally aggressive tumor with an unpredictable clinical course. Surgery was once the mainstay of treatment, but the treatment protocol has been constantly evolving and currently active surveillance is the front-line approach. There have been significant insights into the molecular biology with the addition of mutational analysis of CTNNB1 adding to prognostic information. We present a review of the literature with current practice guidelines, also including novel therapeutic targets and ongoing clinical trials, to unravel the next step in the management of sporadic desmoid fibromatosis.

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