A case report of a feminine patient who complained of nasal ventilatory obstruction and nasosinusal polyposis is presented. She underwent surgery and the pathological study revealed the existence of an aggressive fibromatosis. The AA. review the literature dealing with this illness and explain an update state of the clinical features, treatment and the course of this rare entity, closely related to fibrosarcoma.
Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
Diagnostic utility of LEF1 and β-catenin in WNT pathway tumors with CTNNB1 mutation.
World J Surg Oncol
January 2025
Department of Pathology, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, 1665 Kongjiang Road, Yangpu District, Shanghai, 200092, China.
Objective: This study aimed to compare the expression of lymphoid enhancer factor 1 (LEF1) and β-catenin in basal cell adenoma (BA), desmoid-type fibromatosis (DF), and pancreatic solid pseudopapillary neoplasm (SPN) to evaluate their diagnostic utility in tumors associated with the WNT/β-catenin signaling pathway harboring the mutation of CTNNB1 gene 3 exon.
Methods: Eighty tumor patients, including 26 BAs, 30 DFs, and 24 SPNs, were analyzed. Immunohistochemical staining was identified positive (nuclear staining of LEF1 and β-catenin in > 50% of tumor cells).
Fibromatosis of the breast: a case report and literature review.
AME Case Rep
December 2024
Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Background: Fibromatosis of the breast, also known as desmoid-type fibromatosis (DTF), is a rare tumor marked by the development of non-metastatic, locally aggressive tumors in breast tissue. It represents only 0.2% of all breast tumors.
View Article and Find Full Text PDFA Rare Case of Neonatal Desmoid Tumor Leading to Severe Aortic Coarctation: Review of Literature and Case Report.
Life (Basel)
January 2025
Faculty of Medicine, Carol Davila University of Medicine and Pharmacy, 050474 Bucharest, Romania.
Desmoid tumors are a rare entity, especially in the pediatric population. There are no reports of such a tumor in newborns. They are associated with high rates of morbidity and mortality, even though they are benign soft tissue tumors.
View Article and Find Full Text PDFClinical Benefit of Pegylated Liposomal Doxorubicin and High Prevalence of Pre-Existing Psychiatric Conditions in Patients with Desmoid-Type Fibromatosis.
Cancers (Basel)
January 2025
Department of Medicine, The Masonic Cancer Center, The University of Minnesota Medical School, University of Minnesota, Minneapolis, MN 55455, USA.
Desmoid-type fibromatosis (DTF) is a locally invasive tumor composed of myofibroblast-like cells and collagen; it does not metastasize but can cause significant local morbidity. Most sporadic cases are associated with mutations in the CTNNB1 gene, which encodes beta-catenin. Various treatments have been used with differing efficacy and toxicity profiles.
View Article and Find Full Text PDFThree-dimensional (3D)-printed custom-made titanium ribs for chest wall reconstruction post-desmoid fibromatosis resection.
Comput Assist Surg (Abingdon)
December 2025
Department of Thoracic Surgery, The First People's Hospital of Changzhou, The Third Affiliated Hospital of Soochow University, Changzhou, China.
Desmoid fibromatosis (DF) is a rare low-grade benign myofibroblastic neoplasm that originates from fascia and muscle striae. For giant chest wall DF, surgical resection offer a radical form of treatment and the causing defects usually need repair and reconstruction, which can restore the structural integrity and rigidity of the thoracic cage. The past decade witnessed rapid advances in the application of various prosthetic material in thoracic surgery.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!