In the submitted review of the literature the authors evaluate contemporary therapeutic possibilities of mesenterial desmoids. A more detailed pattern of cytotoxic and non-cytotoxic chemotherapy, actinotherapy and hormonal therapy is presented. Surgical resection is limited by the size of the tumour and above all by early detection before the size of 15 cm is reached. In larger tumours conservative treatment by a combination of non-cytotoxic and hormonal treatment is better. Actinotherapy of the intraabdominal region is not suitable. The percentage of relapses is high, a standard therapeutic procedure has not been elaborated so far. Despite rather surprising remissions of desmoids in individual cases the general results of different groups are not encouraging. In a case-history the authors describe their experience with the treatment of a young female patient with a mesenterial desmoid which, however, was not successful.
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Ther Adv Med Oncol
January 2025
Department of Medical Oncology, Sarcoma and Bone Tumors Reference Center, A.C. Camargo Cancer Center, R. Prof. Antônio Prudente, 211, São Paulo, SP 01509-010, Brazil.
Introduction: Desmoid tumors are soft-tissue neoplasms that can have profound impacts on the lives of people living with such diseases. As they are rare tumors, patients often have difficulty finding teams specialized in sarcomas and support networks. In low- and middle-income countries, the challenges are exacerbated due to a need for established networks and medication access.
View Article and Find Full Text PDFCancers (Basel)
December 2024
Sarcoma Unit, The Royal Marsden Hospital and Institute of Cancer Research, London SW3 6JZ, UK.
There 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 PDFClin Nucl Med
December 2024
From the Department of Neurology, Yueyang Hospital of Integrated Traditional Chinese and Western Medicine, Shanghai University of Traditional Chinese Medicine.
Gardner syndrome is characterized by multiple intestinal polyps and extraintestinal lesions. We describe FDG PET/CT findings of the extraintestinal lesions in a patient with Gardner syndrome. FDG PET/CT showed 2 hypermetabolic desmoid tumors in the abdominal wall, sclerotic areas with multifocal activity in the maxilla and mandible, multiple osteomas in the bilateral parietal, left frontal, sphenoid and ethmoid bones, an impacted tooth in the right maxilla, and bone islands in the T2 and T5 vertebral bodies.
View Article and Find Full Text PDFBJS Open
December 2024
Unit of Hereditary Digestive Tract Tumours, Fondazione IRCCS Istituto Nazionale dei Tumouri, Milan, Italy.
Background: Familial adenomatous polyposis is a cancer-predisposing syndrome caused by germline pathogenic variants of the adenomatous polyposis coli gene, leading to numerous colorectal polyps and a high risk of colorectal cancer. Desmoid tumours have become significant in the management of familial adenomatous polyposis after a colectomy, yet the exact incidence remains undetermined due to a lack of dedicated surveillance.
Methods: This retrospective study accessed data from the prospectively maintained Hereditary Digestive Tumours Registry from 2000 to 2023.
Front Oncol
December 2024
Angeles Breast Center, Hospital Ángeles Valle Oriente, San Pedro Garza Garcia, Nuevo Leon, Mexico.
Background: Desmoid-type fibromatosis of the breast is a rare, benign, but locally aggressive tumor that typically affects women. Its presentation in male patients is exceedingly rare, and even more so following a cosmetic procedure such as liposuction. This case report describes a unique presentation of breast fibromatosis in a male patient, who developed the condition after undergoing liposuction for cosmetic purposes to define the pectoral area.
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