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. This is due to them exhibiting locally aggressive growth with the compression or invasion of adjacent structures. Abdominal localization is most commonly reported, although there are reports of mediastinal desmoid tumors. We present the case of a 6-day male patient with a mediastinal desmoid tumor that led to severe aortic coarctation with hemodynamic instability. The tumor also compressed the left pulmonary artery and obstructed the left main bronchus. The initial management consisted of successful emergency surgery with partial resection of the tumor mass and coarctation repair. In the postoperative setting, the patient evolved with severe respiratory dysfunction which was managed with tracheostomy, allowing weaning the child from the mechanical ventilation one month after surgery, along with chemotherapy. We also review the literature, focusing on the management of desmoid tumors.
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http://dx.doi.org/10.3390/life15010123 | DOI Listing |
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 PDFComput 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 PDFAm J Clin Pathol
January 2025
Department of Pathology and Laboratory Medicine, University of Miami Miller School of Medicine and Jackson Memorial Hospital, Miami, FL, US.
Objectives: Abdominal wall and intra-abdominal fibromatoses are locally aggressive, nonmetastasizing neoplasms. Surgery has been the mainstay of local control, but new forms of therapy have been developed that may influence the clinical course and morbidity. We studied the clinical features and outcomes of patients with abdominal and intra-abdominal fibromatoses over time.
View Article and Find Full Text PDFEur Radiol
January 2025
Department of Radiology, Stanford School of Medicine, Stanford, CA, 94305, USA.
Objective: To identify MRI features of desmoid tumors (DTs) that predict the growth of residual disease following ablation.
Methods: Patients who underwent MRI-guided ablation for DTs between February 2013 and April 2021 were included in this single-center IRB-approved retrospective study. MRI scans assessed three suspicious tissue features: intermediate T2 signal [+iT2], nodular appearance [+NOD], and contrast enhancement [+ENH].
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.
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