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The Desmoid Tumor Research Foundation

July 2017

Desmoid Tumors in Familial Adenomatous Polyposis

Author Information

DE Marchis ML(1), Tonelli F(2), Quaresmini D(3), Lovero D(3), Della-Morte D(4)(5), Silvestris F(3), Guadagni F(1)(4), Palmirotta R(6)

(1)Interinstitutional Multidisciplinary Biobank (BioBIM), IRCCS San Raffaele Pisana, Rome, Italy.(2)Department of Surgery and Translational Medicine, University of Florence, Florence, Italy.(3)Department of Internal Medicine and Clinical Oncology, Aldo Moro University of Bari, Bari, Italy.(4)San Raffaele Roma Open University, Rome, Italy.(5)Department of Systems Medicine, Tor Vergata University, Rome, Italy.(6)Department of Internal Medicine and Clinical Oncology, Aldo Moro University of Bari, Bari, Italy. raffaelepalmirotta@gmail.com.

Publication

Anticancer Res

Summary

Familial adenomatous polyposis (FAP) is a cancer syndrome caused by a germline mutation in the adenomatous polyposis coli (APC) gene. It is characterized by the presence of hundreds of colonic polyps, which have a high tendency to undergo malignant transformation. Among associated lesions in FAP, desmoid tumors represent a common possible life-threatening condition that requires special attention. They are rare tumors occurring with a particularly high incidence in FAP, especially after surgery. In this review, we provide an updated insight into desmoids in FAP syndrome, from molecular pathogenesis to the main issues in management, with special attention given to genetic and molecular features of these tumors.

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