Active surveillance in desmoid-type fibromatosis: A systematic literature review

This study evaluates the results of the active surveillance (AS) approach in adult patients with desmoid-type fibromatosis (DTF) because AS is advocated as a front-line approach for DTF in the European consensus guidelines. A systematic literature search was conducted (December 19th, 2019, updated on April 14th, 2020). Studies describing the outcomes of the AS approach were included. The PRISMA guidelines were used. Twenty-five articles were included for data retrieval. Forty-two percent of reported patients (1480 of 3527 patients) received AS, the majority were women and the majority had a primary tumour. The median age at diagnosis ranged from 28 to 59 years. Common tumour sites were the extremities/girdles (n = 273), the abdominal wall (n = 253) and the trunk (n = 153). The median reported percentage of progressive disease, stable disease and partial response was 20% (interquartile range [IQR]: 13–35%), 59% (IQR: 37–69%) and 19% (IQR 3–23%), respectively. In 640 patients, the outcome was not specified. The median reported percentage of shifting to an active form of treatment was 29%, most commonly to systemic treatment (n = 195) and surgery (n = 107). The reported median follow-up time ranged between 8 and 73 months. The reported median time to progression and/or initiation of the subgroup shifting from AS to ‘active’ therapy ranged from 6.3 months to 19.7 months. The majority of patients undergoing AS have either stable disease or a partial response, and about one-third of patients shift to an active form of treatment. Selecting patients who will benefit from active surveillance upfront should be the priority of future studies. •Active surveillance (AS) is the front-line approach in treating desmoid-type fibromatosis.•42% of patients received AS in studies reported since 2006.•About 30% of patients need to shift to another form of treatment after AS.•The median time to disease progression or shift ranged from 6.3 to 19.7 months.•Surgery and systemic treatment are common forms of active treatment after AS.