Current Treatment Concepts for Extra-Abdominal Desmoid-Type Fibromatosis: A Narrative Review

Abstract

Extra-abdominal desmoid-type fibromatosis (EADTF) is a rare neoplastic condition of monoclonal fibroblastic proliferation characterized by local aggressiveness with a distinct tendency to recur. Although EADTF is a benign disease entity, these tumors have a tendency to infiltrate surrounding normal tissues, making it difficult to completely eliminate them without adjacent healthy tissue injury. Surgical excision of these locally aggressive tumors without clear resection margins often leads to local recurrence. The aim of this thorough review was to assess the current treatment concepts for these rare tumors. A comprehensive search of articles published in the Cochrane Library, MEDLINE (PubMed), and EMBASE databases between January 2008 and February 2023 was conducted. Surgical intervention is no longer the first-line approach for most cases; instead, strategies like active surveillance or systemic therapies are used as initial treatment options. With the exception of EADTFs situated near vital structures, a minimum of 6–12 months of active surveillance is currently advocated for, during which some disease progression may be considered acceptable. Non-surgical interventions such as radiation or cryoablation may be employed in certain patients to achieve local control. The currently preferred systemic treatment options include tyrosine kinase inhibitors, low-dose chemotherapy, and gamma-secretase inhibitors, while hormone therapy is not advised. Nonsteroidal anti-inflammatory drugs are utilized primarily for pain management.

Unraveling the Mystery of Desmoid Tumors: Insights From a Moroccan Tertiary Center

“…Historically, surgery has long been the mainstay treatment of DT [7]. However, a high recurrence rate (up to 60%) even after surgical resection with negative margins and the results of many retrospective and prospective studies demonstrating no difference in PFS and overall survival between initial surgery and active surveillance have made it more of an option to be discussed, particularly in cases of symptomatic tumors or progressive ones [14]. Our series serves as an excellent example of this paradigm change, since the majority of patients who underwent surgery as a first line of treatment were those who were diagnosed before 2016, even though one patient had a cervical site that was thought to be difficult to resect.…”

“…It's interesting to note that three-quarters of patients with extended negative margins after surgery which is more than half of resected individuals relapsed. Contrarily, patients who did not obtain sufficient margins (microscopic invasion) experienced a wider range of outcomes, with one example showing no recurrence at all and another showing one after 15 years [14]. Since there has not been agreement on what a sufficient broad margin is, in part due to contradictory reports and findings, it seems that the objective for DT resections should be to achieve a negative surgical margin, even if it is almost microscopic [8,14,15].…”

“…But nowadays, active surveillance is the standard first approach to consider for the majority of patients [5,6,14]. Yet as simple as it can appear, this approach requires not only the availability of an MRI but also respect for close intervals, especially at the start [6].…”

“…The rationale behind using endocrine therapy is based on the fact that 30% of these tumors express hormone receptors [3], yet there is only one prospective phase II study assessing antihormonal therapy with NSAIDs and showing 36% PFS at two years; current clinical guidelines do not place this option as a viable one [14,17]. Consequently, NSAIDs are not currently considered for the treatment of DT [6,14].…”

“…Half of our patients received systemic therapy as the first and second line, with vinorelbine as the most commonly administered cytotoxic agent. When used, it is recommended to give for at least one year with more than 40 cycles; this ensures a long-lasting effect past the cessation of the treatment [6,14]. Another option used in one of our patients with a symptomatic intra-abdominal DT is MTX-VBL [18,19].…”