Background: Desmoid tumour is a benign, non metastasising neoplasm characterised by an elevated deposition of organic macromolecules in the extracellular matrix (ECM). The matrix metalloproteinases (MMPs) are a family of zinc-dependent proteinases involved in the degradation of ECM macromolecules. The MMPs and their natural inhibitors (TIMPs) have been implicated in tumour growth, invasion and metastasis. In this study we provide evidence that the in vitro cultured cell line from desmoid tumour accumulates more collagen fibres in the ECM than healthy fibroblasts.
Methods: We investigated collagen accumulation by 3H-thymidine incorporation, MMP expression by substrate gel zymography and TIMP expression by Western blot analysis.
Results: Desmoid fibroblasts showed a reduction in MMP activity and an increase of type I and III collagen and TIMPs compared to normal fibroblasts.
Conclusion: The increase in collagen in desmoid fibroblasts was due to inhibited collagen degradation (reduction of MMP activity) rather than to increased collagen synthesis. Adding toremifene, an anti-estrogen triphenylethylene derivate, to desmoid fibroblasts reduced collagen accumulation by decreasing mRNA expression and increasing collagen degradation.
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http://dx.doi.org/10.1186/1471-2407-5-22 | DOI Listing |
Cureus
December 2024
Diagnostic Radiology and Nuclear Medicine, Institute of Science Tokyo, Tokyo, JPN.
Desmoid fibromatosis (DF) is a rare, non-metastasizing but locally aggressive mesenchymal tumor arising from fibroblasts or myofibroblasts. We report a solitary case of DF involving the retropharyngeal and danger spaces, a location rarely documented. The patient, a woman in her 70s, presented with progressive pharyngeal discomfort over six months.
View Article and Find Full Text PDFLab Invest
November 2024
Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea. Electronic address:
Cureus
October 2024
General Surgery, George Washington University, Washington, DC, USA.
Desmoid fibromatosis (DF) is a rare tumor that arises from fibroblasts and myofibroblasts and typically presents on the trunk and limbs. While metastasis of fibromatosis is exceedingly uncommon, DF can spread rapidly to adjacent tissue. Its aggressive nature and frequent recurrence pose challenges for treatment, often requiring a multidisciplinary approach.
View Article and Find Full Text PDFClin Cancer Res
January 2025
Department of Surgery, McGill University, Montreal, Quebec, Canada.
Purpose: Desmoid tumors are bland fibroblastic tumors that do not metastasize but have a high rate of local recurrence. Previously published studies proposed two different transcriptomic signatures to predict relapse. Molecular heterogeneity has been well established in high-grade sarcomas, but little is known about molecular variability within locally aggressive tumors such as desmoids.
View Article and Find Full Text PDFFront Oncol
October 2024
Department of Radiology, The Fourth Affiliated Hospital of School of Medicine, and International School of Medicine, International Institutes of Medicine, Zhejiang University, Yiwu, China.
Cervical aggressive fibromatosis is a rare intermediate tumor characterized by invasive growth. Aggressive fibromatosis (AF), also referred to as desmoid-type fibrosarcoma or grade I fibrosarcoma, is a clonal proliferative lesion of fibroblasts located in deep soft tissues. While many cases have been reported, there are relatively few involving aggressive fibromatosis in the anterior inferior margin muscle space of the neck trapezius muscle.
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