Desmoid tumors are rare mesenchymal tumors, with an incidence of 5–6 cases per million inhabitants per year. While they may appear in any anatomical region, they are most frequently found in the abdominal wall of young women. Beta-catenin is the typical pathological marker. Most desmoid tumors are sporadic, but others are associated with syndromes like familial adenomatous polyposis, and their management differs due to their peculiarities.1–4
Surgery has classically been the basic pillar of treatment, and the aim is to achieve complete resection. However, systematic indication for surgery has been called into question by the high rate of local recurrences, together with the considerable morbidity of the surgical procedure, lack of metastatic capacity of these tumors, and the possibility of complete spontaneous regression (up to half of cases, even without treatment).5–7 As a result, the therapeutic approach has changed towards a multidisciplinary model that incorporates targeted medical or hormonal therapies, which are much less invasive options.
In this context, the Desmoid Tumor Working Group8 has created a consensus document that discusses the prospect of adopting an active, personalized surveillance strategy as an initial option, instead of systematic surgical resection. This scenario, together with emerging therapies with promising results, such as cryoablation and gamma-secretase inhibitors,9–12 makes it essential for the management of these patients to be done in specialized centers, by an experienced multidisciplinary team that includes surgeons, oncologists, radiotherapists, pathologists, etc. Thus, correct indication for surgery takes on maximum relevance and becomes equally important as the surgical technique itself.
In this scenario, the indication for surgery has not ended in desmoid tumors, but the role of the surgeon must change radically. Surgery remains the most important option in many cases, but it is no longer systematic. Far from losing protagonism, surgeons need to acquire greater biological knowledge about the disease and non-surgical alternatives that include active, personalized monitoring in order to reduce postoperative morbidity.
