Challenging aggressive surgical strategies in extraabdominal desmoid tumors: Is tumor volume the missing key?

Summary

Objective: Desmoid tumors are fibroblast-derived rare soft tissue neoplasms with unclear borders that invade the surrounding structures. Despite the non-metastasizing nature, these tumors often relapse due to strong local aggressive behavior. The aim of this study was to analyze the demographic and clinical characteristics of patients with extraabdominal desmoid tumor, who underwent surgery between 2012 and 2022 and to determine the risk factors affecting recurrence. Methods: Thirty-five patients (14 males, 21 females; mean age 41.2 years (range: 11-80) underwent operation between the years 2012 and 2022 and followed up for a mean of 52 months. The diagnosis was confirmed with biopsy, and the plan for surgery and adjuvant radiotherapy was decided by the multidisciplinary tumor council. Demographic, radiologic, and management data of the patients were collected. Potential effects of surgical margin status, estimated tumor volume (ETV), and radiotherapy on recurrence were investigated. Estimated tumor volume was calculated from dimensions on pathologic specimens. Results: While wide surgical resection was aimed for all patients, R0 margins were achieved only in 40%, whereas R1 and R2 margins were obtained in 22.9% and 37.1% of these patients respectively. The local recurrence rate was 40%. No significant correlation was observed between surgical margin positivity and the recurrence or progression of the tumor, when patients with R0 margin are sta-tistically compared with the rest (P = .067). On the other hand, when R0 and R1 margins were compared with each other in terms of recurrence or progression, there was a statistically significant difference (P = .006). While the impact of radiotherapy on recurrence or pro-gression could not be determined due to inadequate sample size, the recurrence rate increased significantly with increasing ETV (P = .01). Conclusion: Extraabdominal desmoid tumors are ill-defined locally aggressive neoplasms with an unpredictable and challenging clinical course. When the disease-free and recurring patients were compared, the recurrence rate increased significantly as the ETV increased. Achieving negative margins is still the benchmark for surgery; however, the risks and benefits of an aggressive surgery should always be reevaluated when there is a high possibility of a complex reconstructive surgery that has the capability to affect the functional outcome adversely.