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Desmoid Fibromatosis: Management in an Era of Increasing Options

  • Sarcomas (SR Patel, Section Editor)
  • Published:
Current Oncology Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

Desmoid fibromatosis (DF) is a locally aggressive clonal neoplasm with locally aggressive behavior and no metastatic potential. Historical treatment of DF has consisted primarily of up-front surgery when feasible. In recent years, recognition that DF can spontaneously stabilize or involute has allowed for many patients to be managed with watchful waiting rather than intervention. This review is intended to review recent developments in the treatment of DF.

Recent Findings

Recent studies have demonstrated prospectively that patients with DF often have improvement in their lesions without intervention, enabling an initial period of surveillance as a standard option for patients with mild symptoms. Given the lengthening list of effective systemic treatments, including sorafenib, pazopanib, and experimental agents, there has been a less reliance on local therapies for those patients who require treatment.

Summary

For patients with DF that require treatment, there is a growing list of options that includes radiation therapy (RT), percutaneous ablation, and a growing list of systemic agents with favorable toxicity profiles.

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Authors and Affiliations

  1. Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd Unit 450, Houston, TX, 77030, USA

    Ravin Ratan

  2. Surgical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd Unit 1484, Houston, TX, 77030, USA

    Christina L. Roland

  3. Radiation Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd Unit 0097, Houston, TX, 77030, USA

    Andrew J. Bishop

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  1. Ravin Ratan
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Correspondence to Ravin Ratan.

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Conflict of Interest

Ravin Ratan currently oversees a clinical trial funded by Springworks Therapeutics, and has also received consulting fees from the same. He also has patients treated with Springworks' drug on a compassionate access protocol. Christina L. Roland has received research funding for clinical trials from Bristol-Myers Squibb paid to her institution. Andrew J. Bishop declares that he has no conflict of interest.

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Ratan, R., Roland, C.L. & Bishop, A.J. Desmoid Fibromatosis: Management in an Era of Increasing Options. Curr Oncol Rep 23, 41 (2021). https://doi.org/10.1007/s11912-021-01026-w

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