Abstract
ABSTRACTObjective:Extra-abdominal desmoid tumor (EDT) is a rare condition, caused by proliferation of fibroblasts. Despite being a benign tumor, it is locally aggressive and has unpredictable clinical behavior. The objective of this study is to present the clinical outcomes of patients with EDT treated surgically between 1995 and 2016.Methods:This is a retrospective series of 23 patients with histopathological diagnosis of EDT that underwent surgery at the orthopedic oncology service of our hospital. The information was obtained from the institute's clinical and pathology reports.Results:A total of 223 medical records with histopathological reports were evaluated. Only 23 cases of EDT were included in the present study. The mean age was 22.5 years. Twelve (52.2%) patients had the tumor on the lower limbs, seven (30.4%) on the upper limbs and four (17.4%) cases were reported on the back. Five (21.7%) patients had tumors measuring less than 5 cm, while eighteen (78.3%) patients had tumors measuring more than 5 cm. All patients underwent surgery as the definitive treatment in our institute. Twelve (52.2%) cases presented negative margins (NM) and eleven (47.8%) cases had positive margins (PM). Local recurrence (LR) occurred in eleven (47.8%) patients.Conclusion:Impairment of the surgical margin was the only prognostic factor found for LR of EDT. Level of Evidence IV, Case Series.
Highlights
23 cases of Extra-abdominal desmoid tumor (EDT) were included in the present study
Impairment of the surgical margin was the only prognostic factor found for Local recurrence (LR) of EDT
Extra-abdominal desmoid tumors (EDT) are a rare condition caused by the proliferation of fibroblasts
Summary
Extra-abdominal desmoid tumors (EDT) are a rare condition caused by the proliferation of fibroblasts. The tumor is benign, it is locally aggressive, and its unpredictable clinical behavior leads to disagreement regarding its treatment.[1,2] According to the World Health Organization (WHO), EDT is defined as a monoclonal proliferation of fibroblasts that affects deep soft tissues. It is characterized by infiltrative growth and a tendency toward local recurrence (LR). It does not present distant metastasis (DM).[3] EDT can affect any part of the body, but appears more frequently on the limbs and trunk (Figure 1). These tumors are rare, with an incidence of 2-4 cases/1,000,000
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