Desmoid Tumours: A single-centre Experience

Summary

Objectives: To reveal the main patient and disease characteristics, treatment management and clinical course of desmoid tumours. Methods: Patients with a diagnosis of desmoid tumour were evaluated retrospectively. Results: Of the 11 patients with a median age of 33.0 (12.1-63.1), 7 (63.6%) were female, and 4 (36.4%) were male. Locations were intraabdominal region, abdominal wall, thoracic wall and lower extremity in 5 (45.5%), 4 (36.4%), 1 (9.1%) and 1 (9.1%) patients, respectively. Two patients (18.2%) had a diagnosis of Familial Adenomatous Polyposis (FAP). Pregnancy and cesarean section in 1 (9.1%) and surgical trauma in 2 (18.2%) patients were the etiological factors. The primary treatment of 10 (90.9%) patients was surgery, and 1 (9.1%) patient was radiotherapy (RT). A second surgical intervention was performed in four patients (36.4%) due to recurrence or progression. Three (27.3%) and 5 (45.5%) patients had received RT and systemic treatments, respectively. Mean overall survival was 130.2 ± 25.0 (95% CI, 81.1-179.2) months, and 3 (27.3%) patients died in follow-up. Conclusion: Desmoid tumours can present with very different clinical behaviours among patients. Large-scale studies are needed to determine the biological characteristics of this extremely rare disease, the optimization of treatment options, and the determination of prognostic factors.