Introduction: Desmoid fibromatosis is a locally aggressive and recurrent benign mesenchymal neoplasm, rare (5-6 cases/million hab). Due to the aforementioned, it is sought to determine the number of cases and the different related perioperative factors, allowing us to know a little more about the behavior of said pathology, as well as to assess the impact of each treatment on the functionality of the patient.
Methods: A retrospective analytical observational study was carried out, 182 medical records were collected, whose data were compiled in an EXCEL database and analyzed in the SPSS 24 database. 82 medical records were excluded. The patients were grouped into those under and over 18 years of age (26 and 74, respectively). Univariate analysis (mean, median, mode); and multivariate analysis (Pearson Correlation).
Results: It was obtained that, of the patients under 18 years of age, 61.5% were male (16); and of those over 18 years of age, 82.4% were women (61). The average age in those under 18 years of age was 12 years; the mean tumor size was 11.5 cm; 69.2% of these patients had a history of a previous surgery. The most frequent location in children under 18 years of age was retroperitoneum and gluteal region (15.4%). They underwent surgical treatment of which: 53.8% reported edges in contact, 19.2% free and the rest were not specified. 46.2% received radiotherapy after surgery and 34.6% presented functional limitation after treatment. In the group older than 18 years, the average age was 38 years, with a mean tumor size of 11.3 cm. 28.6% had a history of previous surgical procedure; the most frequent location was in the abdominal wall (44.6%) and thoracic region (10.8%). Of patients undergoing surgical procedure; 40.5% reported contact margins and 36.5% free margins. 40.5% received radiotherapy treatment after surgery. 13.5% present functional limitation. The average recurrence-free survival in the group under 18 years of age was 9.8 years and in those over 18 years of age it was 10.3 years. In the multivariate analysis in both groups, only a correlation was found with tumor size, the smaller tumor size was related to greater recurrence-free and overall survival (moderate negative correlation, P 0.005)
Conclusions: The pathology under study continues to be rare worldwide. It can be identified that the tumor size is usually large in our population at the time of diagnosis, which hinders the possibility of total resectability depending on its location. Recurrence-free survival is close to 10 years, which allows us to monitor this group of patients at intervals.
Learning Objectives:
To determine the patients with Desmoid Fibromatosis 2000-2019 at the INEN, a national reference center, which will allow us to know the real average figures at the national level
Being a pathology of controversial management, it will allow us to identify the types of treatments, the associated factors, the time free of recurrence.
It will allow analyzing the percentage of patients with functional compromise after surgical and non-surgical treatment, assessing the possibility of observation.