Objective: 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.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6131287 | PMC |
| http://dx.doi.org/10.1590/1413-785220182604192681 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Desmoid tumour: a rare cause of congenital unilateral calf enlargement mimicking calf hypertrophy.
Neuromuscul Disord
December 2024
The John Walton Muscular Dystrophy Research Centre (JWMDRC), Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, United Kingdom; Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom; Newcastle University Translational and Clinical Research Institute, Newcastle upon Tyne, United Kingdom.
Desmoid tumours, also known as aggressive fibromatosis, are rare tumours derived from mesenchymal stem cells, accounting for only 0.03 % of all tumours. While 85-90 % of cases are sporadic, desmoid tumours can occasionally be associated with Gardner syndrome (or Familial Adenomatous Polyposis), which is linked to variants in the tumour suppressor gene, APC (adenomatous polyposis coli) gene on chromosome 5.
View Article and Find Full Text PDFMesenteric desmoid tumor in its cystic form: Case report of a very rare variant.
Radiol Case Rep
February 2025
Department of Surgery « A », Ibn Sina Hospital, Mohammed V University, Faculty of Medicine and Pharmacy, Rabat, Morocco.
Desmoid tumors, also known as deep fibromatosis or desmoid-type fibromatosis, represent a rare subset of deep fibromatoses. It is a locally aggressive tumor, with no specific symptoms, and no metastatic potential. We report a case of a 38-year-old male patient with an abdominal mass.
View Article and Find Full Text PDFA Case Study of Umbilical Hernia Complicated by the Presence of a Desmoid Tumor.
Cureus
November 2024
Department of General Surgery, Sree Balaji Medical College and Hospital, Chennai, IND.
Desmoid tumors or aggressive fibromatosis are locally aggressive benign tumors. These arise anywhere in the body but are commonly seen in the anterior abdominal wall. The main treatment choices are continuous surveillance, adjuvant chemotherapy, surgery, and postoperative chemotherapy.
View Article and Find Full Text PDFChlorhexidine Dihydrochloride Shows Anti-tumor Effects in Desmoid Tumors and Colorectal Cancer.
Anticancer Res
December 2024
Drug Discovery Platform Research Center, Therapeutics and Biotechnology Division, Korea Research Institute of Chemical Technology (KRICT), Daejeon, Republic of Korea;
Background/aim: Desmoid tumors (DTs), or aggressive fibromatosis, are rare neoplasms arising from connective tissue, frequently exhibiting local invasiveness. The limited treatment options and high recurrence rates of DTs highlight the need for novel therapeutic strategies. This study investigated the efficacy of chlorhexidine dihydrochloride (CD) in inhibiting the growth of DTs and colorectal cancer (CRC).
View Article and Find Full Text PDFAnti-tumor Effects of Idarubicin Hydrochloride in Desmoid Tumors.
Anticancer Res
December 2024
Drug Discovery Platform Research Center, Therapeutics and Biotechnology Division, Korea Research Institute of Chemical Technology (KRICT), Daejeon, Republic of Korea;
Background/aim: Desmoid tumors (DTs), also referred to as aggressive fibromatosis, originate from connective tissues and typically manifest with a propensity for local invasion. Despite extensive research efforts aimed at exploring novel anti-tumor agents for DTs, the development of effective clinical management strategies remains an ongoing challenge due to the limited success of current treatments, which frequently lead to inconsistent outcomes and a high recurrence rate of DTs. To overcome these limitations, we focused our research aim on a drug repositioning approach to identify existing medications that could be effective against DTs.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!