Background: Ossifying fibromyxoid tumour is a rare mesenchymal soft tissue sarcoma with uncertain differentiation and variable metastatic potential.
Patients And Methods: This study offers a retrospective analysis of 23 patients diagnosed with OFMT between 1993 and 2024.
Results: The tumours most commonly arose in the extremities and trunk, with all patients undergoing surgical resection of the primary tumour.
A tracheoesophageal fistula (TEF) represents a condition characterized by abnormal communication between the gastrointestinal tract and the airways. Although the current gold-standard treatment is surgery, pre-existing clinical conditions may represent contraindications. We therefore propose a bronchoscopic approach through rigid bronchoscopy without tracheostomy for total repair in patients suffering from benign tracheoesophageal fistulas.
View Article and Find Full Text PDFThere has been noteworthy progress in molecular characterisation and therapeutics in soft tissue sarcomas. Novel agents have gained regulatory approval by the FDA. Examples are the tyrosine kinase inhibitors avapritinib and ripretinib in gastrointestinal stromal tumours (GIST), the immune check point inhibitor atezolizumab in alveolar soft part tissue sarcoma, the γ-secretase inhibitor nirogacestat in desmoid tumours, the NTRK inhibitors larotrectinib and entrectinib in tumours with fusions, the mTOR inhibitor nab-sirolimus in PEComa, and the EZH-2 inhibitor tazemetostat in epithelioid sarcoma.
View Article and Find Full Text PDFLiver transplantation (LTx) is increasingly used in Urea Cycle Defects (UCDs) to prevent recurrent hyperammonemia and related neurological irreversible injury. Among UCDs, argininosuccinate lyase deficiency (ASLD) has a more complex phenotype than other UCDs, with long-term neurocognitive deficits. Therefore, the role of LTx in ASLD is still debated.
View Article and Find Full Text PDFBackground And Purpose: The interaction between language and other cognitive networks in patients harboring brain tumors is poorly understood. We studied the modification of the cognitive control network (CCN) induced by brain tumors and its participation in language reorganization. We hypothesized that patients with brain tumors and reorganized language would show modification of the CCN compared to patients who remain left dominant.
View Article and Find Full Text PDF