Desmoid tumors are a rare group of locally aggressive, non malignant tumors of fibroblastic origin that can lead to significant morbidity due to local invasion and may even result in a fatal outcome when located around vital organs. Their clinical presentation, biological behavior and natural history can be quite varied and is incompletely understood at the present time. The optimal therapeutic approach depends on various factors, and a multidisciplinary approach is necessary to achieve local control with acceptable morbidity. Despite progress in the understanding of these tumors and the treatment options, local recurrence remains a major problem.
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http://dx.doi.org/10.11604/pamj.2015.21.93.7037 | DOI Listing |
Cureus
November 2024
Otolaryngology - Head and Neck Surgery, Aintree University Hospital, Liverpool, GBR.
Aggressive fibromatosis is a rare, benign proliferative disease with unknown aetiology and high recurrence rate. To date, there are only eight reported cases affecting the larynx. Four were managed with total laryngectomy, whilst spontaneous regression happened in one case.
View Article and Find Full Text PDFJ Clin Gastroenterol
October 2024
Department of Surgery.
Goals: To clarify the characteristics of desmoid tumors in Japanese patients with familial adenomatous polyposis after colectomy.
Background: Few comprehensive reports have been published on desmoid tumors in Asian patients with familial adenomatous polyposis.
Study: This retrospective study included the data of 81 patients with familial adenomatous polyposis who underwent surgery between 1978 and 2021.
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 PDFRadiol Case Rep
February 2025
Radiology, Medical Fitness/Dubai Academic Health Corporation, Dubai, United Arab Emirates.
Cortical desmoid is an uncommon benign bony lesion that can typically be diagnosed through conventional radiographic methods. However, here we present an atypical case of a 14-year-old male who presented with a history of trauma, and the initial X-ray showed a right femur suspicious bony lesion. Still, the subsequent use of CT and MRI confirmed the diagnosis of cortical desmoid, so conservative management and follow-up imaging was recommended.
View Article and Find Full Text PDFRadiol 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.
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