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Review ArticleReviewsR

Desmoid Tumors in Familial Adenomatous Polyposis

MARIA LAURA DE MARCHIS, FRANCESCO TONELLI, DAVIDE QUARESMINI, DOMENICA LOVERO, DAVID DELLA-MORTE, FRANCO SILVESTRIS, FIORELLA GUADAGNI and RAFFAELE PALMIROTTA
Anticancer Research July 2017, 37 (7) 3357-3366;
MARIA LAURA DE MARCHIS
1Interinstitutional Multidisciplinary Biobank (BioBIM), IRCCS San Raffaele Pisana, Rome, Italy
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FRANCESCO TONELLI
2Department of Surgery and Translational Medicine, University of Florence, Florence, Italy
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DAVIDE QUARESMINI
3Department of Internal Medicine and Clinical Oncology, Aldo Moro University of Bari, Bari, Italy
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DOMENICA LOVERO
3Department of Internal Medicine and Clinical Oncology, Aldo Moro University of Bari, Bari, Italy
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DAVID DELLA-MORTE
4San Raffaele Roma Open University, Rome, Italy
5Department of Systems Medicine, Tor Vergata University, Rome, Italy
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FRANCO SILVESTRIS
3Department of Internal Medicine and Clinical Oncology, Aldo Moro University of Bari, Bari, Italy
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FIORELLA GUADAGNI
1Interinstitutional Multidisciplinary Biobank (BioBIM), IRCCS San Raffaele Pisana, Rome, Italy
4San Raffaele Roma Open University, Rome, Italy
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RAFFAELE PALMIROTTA
3Department of Internal Medicine and Clinical Oncology, Aldo Moro University of Bari, Bari, Italy
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  • For correspondence: raffaelepalmirotta@gmail.com
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Abstract

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 agreement with Knudson's ‘two-hit’ theory, the inactivation of the residual APC gene in FAP is a critical step in the development of both colorectal cancer and desmoids. Several lines of evidence show that germline mutations affect the functional domains of the APC gene that are responsible for interactions of the transcript with β-catenin, whereas somatic second mutations involve the downstream region of the gene. Hence, an understanding of the molecular pathways underlying desmoid progression in FAP could be important for research and a valid resource for the early prevention and tailored treatment of this disease. 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.

  • Desmoid
  • APC gene
  • familial adenomatous polyposis
  • β-catenin
  • review
  • Received May 16, 2017.
  • Revision received May 26, 2017.
  • Accepted May 30, 2017.
  • Copyright© 2017, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved
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Anticancer Research: 37 (7)
Anticancer Research
Vol. 37, Issue 7
July 2017
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Desmoid Tumors in Familial Adenomatous Polyposis
MARIA LAURA DE MARCHIS, FRANCESCO TONELLI, DAVIDE QUARESMINI, DOMENICA LOVERO, DAVID DELLA-MORTE, FRANCO SILVESTRIS, FIORELLA GUADAGNI, RAFFAELE PALMIROTTA
Anticancer Research Jul 2017, 37 (7) 3357-3366;

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Desmoid Tumors in Familial Adenomatous Polyposis
MARIA LAURA DE MARCHIS, FRANCESCO TONELLI, DAVIDE QUARESMINI, DOMENICA LOVERO, DAVID DELLA-MORTE, FRANCO SILVESTRIS, FIORELLA GUADAGNI, RAFFAELE PALMIROTTA
Anticancer Research Jul 2017, 37 (7) 3357-3366;
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  • Article
    • Abstract
    • Epidemiology
    • Biological Basis
    • APC Gene Mutations and Genotype–Phenotype Correlations
    • Other Molecular Markers
    • Clinical Presentation
    • Prophylactic Measures to Avoid the Onset of DTs
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Keywords

  • Desmoid
  • APC gene
  • Familial adenomatous polyposis
  • β-catenin
  • review
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