We report the case of a 22-year-old patient who presented to the emergency department with epigastric pain and vomiting. Haematological studies showed a rapid decrease in haemoglobin levels from 13.6 g/dl to 4.9 g/dl. Abdominal ultrasonography revealed the presence of fluid around the spleen and the patient was immediately referred for surgery. An intra-abdominal desmoid tumour presenting as a hemorrhagic shock has not previously been described. Given the relatively benign course of the disease and the young age at presentation, this clinical entity should not be overlooked as it has the potential to invade vessels and therefore be fatal.
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http://dx.doi.org/10.1308/003588412X13171221588857 | DOI Listing |
Eur Radiol
January 2025
Department of Radiology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
Objectives: To characterize the radiological findings of desmoid-type fibromatosis (DF).
Methods: This two-institution retrospective study included 152 patients with pathologically confirmed DF who underwent computed tomography (CT), magnetic resonance imaging (MRI), or 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET)/CT between January 2001 and February 2024. Two board-certified radiologists independently evaluated the CT, MRI, and FDG-PET/CT findings, and a third board-certified radiologist resolved discrepancies.
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).
Int J Surg Case Rep
January 2025
Department of Obstetrics and Gynecology, Tahar Sfar University Hospital, 5111 Mahdia, Tunisia.
Introduction And Importance: Desmoid tumours typically arise in the abdomen and extremities. They are rare, originating from mesenchymal cells, with intra-abdominal desmoid tumours (DT) being even less common. While non-malignant and non-metastatic, they can be locally invasive, often necessitating surgical intervention for complete resection.
View Article and Find Full Text PDFAME 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 PDFLife (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.
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