Chest wall desmoid-type fibromatoses are rare, locally aggressive tumors that occasionally arise from previous thoracotomy sites. Tumors arising from previous sites of thoracotomy to treat malignant disease should be discriminated from the pleural dissemination of the previous malignancy. In this study, we report a case of desmoid-type fibromatosis arising from a site for thoracotomy to treat lung cancer. Additionally we reviewed 15 reported cases of desmoid-type fibromatosis following thoracotomy and summarized their features. A 62-year-old woman was found to have a tumor on computed tomography (CT) at a 1-year routine checkup for lung cancer. The tumor (diameter, 3.4 cm) was located at the previous thoracotomy site. Positron emission tomography (PET) revealed mild (18)F-fluorodeoxyglucose (FDG) accumulation in the tumor, with a maximal standardized uptake value (SUVmax) of 1.9. CT-guided biopsy revealed only fibrous tissue. Eighteen months after the biopsy, CT revealed apparent tumor growth, and a biopsy revealed the same histology observed previously. The tumor was removed and diagnosed as desmoid-type fibromatosis. Currently, the patient is alive without recurrence 4 years after desmoid surgery.
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http://dx.doi.org/10.5761/atcs.cr.12.02149 | DOI Listing |
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
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 PDFJ Clin Pathol
December 2024
Institute of Tissue Medicine and Pathology, University of Bern, Bern, Switzerland
Aims: Mutations affecting exon 3 of the β-catenin () gene result in constitutive activation of WNT signalling and are a diagnostic hallmark of several tumour entities including desmoid-type fibromatosis. They also define clinically relevant tumour subtypes within certain entities, such as endometrioid carcinoma. In diagnostics, β-catenin immunohistochemistry is widely used as a surrogate for mutations.
View Article and Find Full Text PDFCureus
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.
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