Abstract
Background: When patients with desmoid tumors (DTs) present uncontrolled clinical symptoms, surgery is an effective treatment, but the high postoperative recurrence rate is a major problem. The significance of adjuvant radiotherapy has been debated for many years, and the significance of aggressive surgery has not been reported.Methods: Medical records for DT patients were collected. KM analysis and the Mann–Whitney U-test were performed to evaluate the role of radiotherapy and aggressive surgery in the entire cohort and different subgroups.Results: Of 385 DT patients, 267 patients with R0 resection were included in the final analysis. A total of 53 patients (19.85%) experienced recurrence. Although radiotherapy showed no significant effect on recurrence-free survival (RFS) or time to recurrence (TTR) in the entire cohort, radiotherapy delayed recurrence in the age ≤ 30 years old subgroup (TTR = 35 months with surgery plus radiotherapy, TTR = 11 months with surgery alone; p = 0.014) and the tumor diameter >5 cm subgroup (TTR = 26 months with surgery plus radiotherapy, TTR = 11 months with surgery alone; p = 0.02) among patients with a single tumor. Aggressive surgery improved RFS in the tumor diameter >5 cm subgroup (p = 0.049) but not the entire cohort.Conclusions: Although radiotherapy cannot improve RFS, it can delay recurrence in the age ≤ 30 years old subgroup and the tumor diameter >5 cm subgroup among patients with a single tumor. For patients with large invasive tumors and multiple involved sites, aggressive surgery could be selected to achieve complete tumor resection to improve RFS.
Highlights
Desmoid tumors (DTs) are generally regarded as borderline or low-grade malignant tumors
According to our treatment experience and relevant guidelines, postoperative adjuvant radiotherapy for DT patients is mainly applied in the following patients: [1] patients with recurrent tumors; [2] patients with tumors located in the extremities, head and neck, or chest wall; and [3] patients with large tumor diameters [9, 19]
Our results suggests that radiotherapy could delay recurrence in the tumor diameter > 5 cm subgroup of patients with a single tumor
Summary
Desmoid tumors (DTs) are generally regarded as borderline or low-grade malignant tumors. They have no metastatic potential, they are prone to recurrence after surgery and may cause local damage [1]. DT only accounts for 3% of all soft tissue tumors, and the incidence rate is only 5–6 per million. Mutation of the CTNNB1 gene encoding β-catenin in ∼85–90% of sporadic DTs may lead to the accumulation of β-catenin in the nucleus, which may be related to the pathogenesis of DT [2]. FAP-related DTs are associated with APC gene mutations. When patients with desmoid tumors (DTs) present uncontrolled clinical symptoms, surgery is an effective treatment, but the high postoperative recurrence rate is a major problem. The significance of adjuvant radiotherapy has been debated for many years, and the significance of aggressive surgery has not been reported
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