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Abstract
Desmoid tumors (DT) are deep fibroblastic neoplasms that arise from musculo-aponeurotic stromal elements. DTs may result in significant morbidity by infiltrating vital anatomic structures. Their mortality is often due to the local aggressiveness, most commonly when intra-abdominal in location. Some indolent DTs can be observed expectantly; infiltrative tumors require an aggressive and multidisciplinary approach and are offered conservative therapies such as non-steroidal anti-inflammatories or antiestrogens when surgery is not feasible. Comparably, chemotherapy is considered for those cases not amenable to surgery or radiation. Bowel resection and at times intestinal transplantation may be necessary. However, DTs may recur post-surgery making long term management of these patients. Herein, we review the genetics, clinical presentations, and outcomes, as well as treatments of DTs.
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Publication history
Accepted:
January 13,
2023
Received in revised form:
January 13,
2023
Received:
June 21,
2022
Publication stage
In Press Accepted ManuscriptFootnotes
Conflicts of Interest: The authors disclose no conflicts.
Authors’ contributions:
FC: Figueredo Carlos (Conceptualization, data curation, Project administration, resources, visualization, writing, review and editing process)
ST: Schiano Thomas (Conceptualization, visualization, writing, review and editing process)
Funding: None
Ethical Statement: The study did not require the approval of an institutional review board.
Reporting Guidelines: Non-applicable.
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© 2023 Published by Elsevier, Inc on behalf of the AGA Institute
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