TY - JOUR
T1 - Desmoid Fibromatosis
T2 - Management in an Era of Increasing Options
AU - Ratan, Ravin
AU - Roland, Christina L.
AU - Bishop, Andrew J.
N1 - Funding Information:
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.
Publisher Copyright:
© 2021, The Author(s), under exclusive licence to Springer Science+Business Media, LLC part of Springer Nature.
PY - 2021/4
Y1 - 2021/4
N2 - 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.
AB - 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.
KW - Aggressive fibromatosis
KW - Radiation therapy
KW - Sorafenib
KW - Systemic therapy
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U2 - 10.1007/s11912-021-01026-w
DO - 10.1007/s11912-021-01026-w
M3 - Review article
C2 - 33719012
AN - SCOPUS:85102554157
SN - 1523-3790
VL - 23
JO - Current oncology reports
JF - Current oncology reports
IS - 4
M1 - 41
ER -