Acta Orthopaedica et Traumatologica Turcica
Research Articles

Extra-abdominal desmoid fibromatosis: An evaluation of clinical factors affecting local recurrence rates


Department of Orthopedics and Traumatology, Ondokuz Mayıs University, School of Medicine, Samsun, Turkey

AOTT 2021; 55: 547-551
DOI: 10.5152/j.aott.2021.21033
Read: 864 Downloads: 263 Published: 22 October 2021

Objective: The aim of this study was to determine the prognostic factors for recurrence in patients with extra-abdominal desmoid tumors (EDTs) treated surgically.

Methods: This single-institution, retrospective study included patients with a histologically-proven extra-abdominal desmoid tumor between 2007 and 2018. The demographic characteristics (age, sex) of the patients, tumor characteristics (region, size, proximity to neurovascular structures, margins), treatment management (surgery and/or adjuvant radiotherapy), and clinical results were analyzed. The effects of these possible prognostic factors on overall and disease-free survival rates and the risk of local recurrence were evaluated.

Results: Evaluation was made of 22 patients (16 females, 6 males) with a mean age at diagnosis of 34.7 years (range = 22-76 years). The mean follow-up was 104 months (range = 4.8-168). Tumor localization was in the upper extremity in 4 patients (18.1%), the lower extremity in 11 (50.0%), and the trunk in 7 (31.8%). The mean tumor size (maximum diameter) was 5.2 cm (range = 0.6-13 cm; median = 5.8 cm), and the mean tumor volume was 181.3 ± 531.4 ml. All the 22 patients were treated surgically along with adjuvant radiotherapy (RT) administered to 8 in addition to surgery for the primary treatment of the tumor. Following primary surgery, resection margins were R0 in 11 patients, R1 in 9 and R2 in 2. Local recurrence (LR) developed in 6 patients (27.2%) during the follow-up period. Recurrence-free survival rate (RFS) was 90.9% at one year, 74.1% at 5 years, and 61.7% at 10 years. During the follow-up, no patient died, and distant metastasis was not detected. Tumor length, resection margins, and adjuvant RT were observed to influence the risk of local recurrence (P < 0.05).

Conclusion: The results of this study have demonstrated that tumor size ≥ 5 cm and the presence of microscopic or macroscopic positive surgical margins can increase the risk of LR, and adjuvant RT can reduce the development of LR in the management of EDT.

Cite this article as: Coşkun HS, Erdoğan F, Çinka H, Dabak N. Extra-abdominal desmoid fibromatosis: An evaluation of clinical factors affecting local recurrence rates. [published online ahead of print, 2021 October 18]. Acta Orthop Traumatol Turc; 10.5152/j.aott.2021.21033. DOI: 10.5152/j.aott.2021.21033.

ISSN 1017-995X EISSN 2589-1294