Sporadic Abdominal Wall Desmoid: Is it Time to Change Our First-Line Approach?

Abstract

The team of the Royal Marsden of London reported a series of 50 patients who underwent the resection of a primary sporadic abdominal wall desmoid tumor (DT). This field is changing rapidly as new data accumulates, and a personalized approach that takes into account the initial tumor size and/or the evolution after initial surveillance is becoming the preferred method. This series confirms recent data indicating the strong predominance (96 %) of females, particularly those at reproductive age, with tumors in this location. During the study period, the authors systematically performed surgical resection following a biopsy. This approach resulted in an abdominal wall defect that required a prosthetic mesh in 94 % of patients. Not surprisingly, prolonged postoperative pain, hernia or infection affected approximately one patient out of five. Interestingly, no obstetrical impairment due to the mesh repair was observed among the ten pregnancies that reached full term. This study confirms the reports of others that the prognosis is good for abdominal wall DT compared with extra-abdominal DT. In other words, excellent local control was achieved with surgery alone, with low but significant morbidity. Combining all studies that examined the resection of abdominal wall DT, the estimated recurrence rate after surgery calculated by the authors is approximately 5 %. The current issue is when and for whom should we propose this efficient surgical resection? The authors acknowledge that their strategy has evolved recently to adopt active surveillance as a first-line therapy, as better knowledge of the disease has been achieved through the most recent analysis. Indeed, a recent study reported on 147 patients with abdominal wall desmoid, 102 of whom underwent an initial observation. Approximately one-third of the patients remained stable and one-third exhibited spontaneous regression. Only 16 % of the patients received operations at 3 years because of pain or progression. The vast majority of progressions occur during the first 3 years after diagnosis. Adopting a ‘wait and see’ approach enables the identification of patients who really require treatment, the best of which is surgery in this location, as shown in this paper. It is likely that abdominal wall desmoids were overtreated in the past, and surgical resection should now only be proposed for selected cases. The risk of a ‘wait and see’ approach is that the progression may create the need for a more extensive operation, and this risk should be weighed against the risk of performing an unnecessary surgery in the majority of patients. Therefore, the main issue is to detect the few patients who are at a higher risk of progression. In this paper, a tumor size greater than 7 cm was the only factor that affected disease-free survival. This threshold was also found in previous papers. Moreover, when we observe significant percentages of progression according to RECIST (Response Evaluation Criteria In Solid Tumors), the baseline tumor size is important to consider. Others factors, such as the location relative to the inguinal ligament and patient compliance to strict initial monitoring should also be taken into consideration. In the event of progression, the best salvage treatment in this location is clearly surgery. Whether medical treatment can be interposed as a secondline before deciding to resort to surgery is under investigation; patients who develop a DT just after pregnancy are likely the best candidates for this approach. The risk of progression for an in situ DT during pregnancy was evaluated recently in a small series (29 patients) and found to be approximately 50 %, but the condition was safely managed in the majority of cases. We need further Society of Surgical Oncology 2014