Clinical Challenges and Evolving Treatments in Desmoid Fibromatosis: A Single Institution Experience

Abstract

Desmoid tumor (DT), also known as desmoid fibromatosis, is a rare, locally proliferative tumor characterized by an overgrowth of myofibroblastic cells. Due to the varied clinical presentation of DT, there are a multitude of treatment options. This study provides our institutional experience in characterizing and treating DT as well as patient outcomes. A retrospective review was performed for 49 patients diagnosed with DT. Patient demographics, tumor characteristics, treatment characteristics, and tumor recurrence were reported. We reported our institution's treatment trends over time, relative risk analysis for surgery, as well as univariate analysis for recurrence. Thirty-seven patients received surgery with an overall recurrence rate of 29.7% (11/37). In total, ten patients received medical therapy including tamoxifen/sulindac (n = 7), nirogacestat (n = 1), and sorafenib (n = 2). One patient has been followed with active surveillance. Relative risk for surgery and tumor recurrence was not significantly correlated with race, gender, location, or large tumor size > 5 cm. Four patients treated with medical therapy experienced tumor reduction and symptomatic improvement. Management of DT includes many surgical and non-surgical options. We noted a similar recurrence rate in patients who received surgical treatment to what has been reported in the literature roughly 33%. We also noted effective tumor control in patients receiving medical therapy. As such, surgery can be utilized in situations with well-demarcated DT which can be removed en bloc, while utilizing medical therapy for highly invasive tumors.

Keywords: Aggressive fibromatosis; Desmoid tumor; Embolization.