Disease and economic burden of surgery in desmoid tumors: a review

ABSTRACT

Introduction

Desmoid tumors (DT) are soft-tissue tumors that infiltrate into surrounding structures with ill-defined margins. Although surgery is a potential treatment option, complete excision with negative margins is not often possible, the postsurgery recurrence rate is high, and surgery can result in disfigurement and/or loss of function.

Areas covered

We conducted a literature review to assess the burden of surgery in patients with DT, focusing on recurrence rates and functional deficits resulting from surgeries. Since economic data related to DT surgery is lacking, reviews of surgery costs in soft-tissue sarcomas and of general costs of amputations were conducted. Risk factors for DT recurrence after surgery are young age (<30 years), tumor location (extremities), tumor size (>5 cm in greatest diameter), positive resection margins, and history of trauma in the area of the primary tumor. Tumors in the extremities have the highest risk of recurrence (30%–90%). Lower rates of recurrences have been reported when radiotherapy was used after surgery (14%–38%).

Expert opinion

Although effective in specific cases, surgery may be associated with poor long-term functional outcomes and higher economic costs. Therefore, it is imperative to find alternative treatments with acceptable efficacy and safety profiles that do not adversely affect functional aspects in patients.

KEYWORDS:

• Aggressive fibromatosis
• costs
• desmoid tumors
• functional outcomes
• quality of life
• recurrence rate
• surgery
• systemic therapy

Article highlights

• Desmoid tumors (DT) are soft-tissue tumors with aggressive, infiltrative, and destructive growth, and complete excisions with R0 margins are not often possible, with recurrence rates after surgery as high as 90%. Surgery can result in disfigurement and/or loss of function.

• Surgical excision was the most common treatment option before 2000 and the early 2000s. Recent key guidelines recommend active surveillance with continuous monitoring.

• This study assessed the burden of surgery in patients with DT, including rates of recurrences postsurgery and functional deficits resulting from surgery. We also assessed the economic burden associated with surgery in DT.

• The rate of surgery has decreased in the past 10 years, and recurrence rates of DT postsurgery depend on the location of the tumor, resection margins, type of mutation, tumor size, patient age, and history of trauma in the area of the primary tumor. To avoid repeat surgeries, patients should be carefully selected to avoid complications resulting in poor functional outcomes and unnecessary resections and amputations that may contribute to the increased economic burden of this disease. Using surgeries in patients with soft-tissue sarcomas as a proxy due to a lack of published data for DT, resection of a tumor can cost more than $50,000 in 2022 US dollars, depending on type and location of tumor, and the cost of amputations would be even higher.

• Repeat surgery may negatively impact a patient’s quality of life. Therefore, a stepwise approach is recommended for the management of DT, from active surveillance to systemic therapy.

Acknowledgments

The authors thank John Forbes, Medical Editor at RTI Health Solutions, for his contributions of editorial revisions of this manuscript, and Uchenna Iloeje and Jennifer Han from SpringWorks Therapeutics, Inc., for reviewing this manuscript.

Declaration of interest

T. Bell, S. Zhou, B. Tumminello, and A. B. Oton are employees of SpringWorks Therapeutics, Inc., Stamford, CT, U.S.A and have an equity or financial interest in SpringWorks Therapeutics, Inc. S. Khan is an employee of RTI Health Solutions, Research Triangle Park, NC, U.S.A. M. M. Fernandez is a former employee of RTI Health Solutions. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Supplementary material

Supplemental data for this article can be accessed online at https://doi.org/10.1080/14737167.2023.2203915

Additional information

Funding

SpringWorks Therapeutics, Inc., Stamford, CT, USA, provided funding to RTI Health Solutions for conducting this study and was involved in reviewing the manuscript