Desmoid fibromatosis, although histologically benign, infiltrates local structures. The involvement of neural structures can lead to difficult neuropathic pain and the escalating use of analgesics. We report a patient with desmoid fibromatosis of the chest wall causing brachial plexus infiltration. As the tumor was locally invasive and unresectable, he was treated with radiation therapy and oral tamoxifen. On follow-up, there was significant pain relief, sustained reduction in the tumor size, and reduced analgesic requirement. Antineoplastic treatments like local radiation therapy and targeted systemic therapy with hormones or other agents can be considered in the management of selected unresectable desmoid fibromatosis to improve symptom control and reduce polypharmacy.
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http://dx.doi.org/10.4103/0973-1075.92349 | 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 PDFNeuromuscul 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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