Extra-abdominal fibromatosis (desmoid tumour) is a rare aggressive neoplasm with a tendency to infiltrate local structures but rarely metastasises or undergoes spontaneous malignant transformation. The treatment of choice is surgery, however, recurrences have been reported even after wide-field resection. This article presents a case of extra-abdominal fibromatosis that had extensively invaded the mandible.
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Active surveillance in patients with extra-abdominal desmoid-type fibromatosis: a pooled analysis of three prospective observational studies.
Clin Cancer Res
December 2024
Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
Purpose: Three prospective observational studies (Italy, the Netherlands, France) on active surveillance (AS) in patients with extra-abdominal desmoid-type fibromatosis (DTF) support AS as a frontline approach. Identifying prognostic factors for the failure of AS will help determine the strategy. The aim of this study was to investigate the prognostic impact of clinical and molecular variables in a larger series.
View Article and Find Full Text PDFCryoablation in Extra-Abdominal Desmoid Tumors: A 10-Year Experience in Pediatric and Young Adult Patients.
Cardiovasc Intervent Radiol
December 2024
Diagnostic and Interventional Imaging, The University of Texas Health Science Center, MSB2.130B, 6431 Fannin Street, Houston, TX, 77030, USA.
Article Synopsis
- The study investigates the effectiveness and safety of cryoablation for treating desmoid tumors in pediatric and young adult patients over a 10-year period.
- A total of 21 patients underwent 34 cryoablation procedures, with significant pain relief and improved mobility reported; 90% of patients showed symptom improvement.
- Most tumors showed at least partial volume reduction, with some achieving complete response, and only a few minor complications were noted, indicating cryoablation is a promising treatment option.
Desmoid Fibromatosis of the Oesophagus Creating an Oesophageal Diverticulum in a 2-year-old Girl.
Afr J Paediatr Surg
July 2024
Department of Surgical Oncology, Division of Paediatric Surgical Oncology, Tata Memorial Hospital and Advanced Centre for Training Research and Education in Cancer, Tata Memorial Centre, Mumbai, Maharashtra, India.
Article Synopsis
- - The case involves a 2-year-old girl who has a rare condition called extra-abdominal desmoid fibromatosis, which is linked to an esophageal tumor and also has traction diverticula.
- - This situation presented a surgical challenge because it required the treatment of multiple health issues at once.
- - The surgery successfully targeted both the desmoid fibromatosis and the diverticulum, ultimately helping to achieve clear margins in the surgical removal.
Prognostic impact of tumor location and gene expression profile in sporadic desmoid tumor.
Eur J Cancer
September 2024
Health Research Institute Fundación Jiménez Díaz (IIS-FJD, UAM), Madrid, Spain; Medical Oncology Department, University Hospital General de Villalba, Madrid, Spain; Medical Oncology Department, University Hospital Fundación Jiménez Díaz, Madrid, Spain. Electronic address:
Article Synopsis
- Prognostic biomarkers are important for understanding sporadic desmoid tumors (DT) due to their unpredictable nature; this study focused on gene expression differences between DTs located in the thoracic versus abdominal wall.
- A retrospective analysis of 197 sporadic DT patients showed significant relationships between relapse-free survival (RFS) and factors like tumor size, location, and specific gene mutations, notably the CTNNB1 T41A and S45F mutations.
- Results suggest that tumors in the abdominal wall have a better prognosis compared to extra-abdominal locations, with distinct gene expression profiles influencing cancer pathways related to their behavior and outcomes.
Initial treatment for surgery-naïve desmoid tumors by high intensity focused ultrasound.
Front Oncol
July 2024
Department of Medical Oncology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China.
Introduction: Desmoid tumor (DT) is a rare proliferative disease occurring in connective tissues, characterized by high infiltration and recurrence rates. While surgery remains the primary treatment, its recurrence risk is high, and some extra-abdominal desmoid tumors are inoperable due to their locations. Despite attempts with radiotherapy and systemic therapy, the efficacy remains limited.
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