Desmoid tumors (DTs) are rare, aggressive malignancies developing from clonal fibroblastic proliferation originating from soft tissues. Despite their low metastatic potential, their invasiveness towards neighboring organs and a high recurrence rate contribute significantly to morbidity and mortality, thereby impacting the quality of life of patients. Several therapeutic options are available, but standardized protocols are lacking. In this study, we reviewed 14 cases of DT retrospectively over a period of 15 years, from September 2008 to December 2023. The most prevalent tumor locations were in the extremities, and the majority of patients were female. We identified risk factors in two patients, those being surgical trauma and familial adenomatous polyposis (FAP). Half of the patients underwent surgery for DT, and two received salvage radiotherapy. Systemic therapy was used in the first and second lines and comprised of chemotherapy, endocrine therapy, and non-steroidal anti-inflammatory drugs (NSAI). Active surveillance was proposed in three patients. This is the first retrospective study to assess the characteristics of DT in Moroccan patients in a tertiary care setting. It aims to shed light on the challenges faced in treating these rare tumors in the context of a lack of therapeutic standardization.
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http://dx.doi.org/10.7759/cureus.57768 | DOI Listing |
Breast J
January 2025
Australian National University School of Medicine and Psychology, Canberra, ACT 2600, Australia.
Breast desmoid tumour is a rare type of benign breast disease that presents like malignancy. Current guidelines are based on limited evidence derived from case reports and small case series and recommend resection with microscopically-negative margin (R0). There is a high risk of recurrence despite negative surgical margins.
View Article and Find Full Text PDFFront Oncol
December 2024
Angeles Breast Center, Hospital Ángeles Valle Oriente, San Pedro Garza Garcia, Nuevo Leon, Mexico.
Background: Desmoid-type fibromatosis of the breast is a rare, benign, but locally aggressive tumor that typically affects women. Its presentation in male patients is exceedingly rare, and even more so following a cosmetic procedure such as liposuction. This case report describes a unique presentation of breast fibromatosis in a male patient, who developed the condition after undergoing liposuction for cosmetic purposes to define the pectoral area.
View Article and Find Full Text PDFCureus
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.
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