Variables affecting desmoid tumor risk in Familial Adenomatous Polyposis: A critical review

Introduction: Familial Adenomatous Polyposis (FAP) is a genetic disorder that predisposes to colorectal cancer and many other extracolonic manifestations.Among them, desmoid disease is considered a great challenge and a major source of morbidity in these patients.The aim of the present manuscript is to evaluate the role of many clinical and surgical variables potentially involved in desmoid risk after prophylactic colectomy.Methods: we have searched case series, observational studies, retrospective studies, meta-analyses and systematic reviews (1990 to 2020) dealing with incidence and risk factors for desmoid disease after FAP surgical treatment.Results: desmoid disease may develop in approximately 10-20% of FAP patients leading to complications and mortality.Frequency and risk of desmoid risk have been associated with clinical and surgical variables not always dependent on surgeon control.Female sex, family history of desmoid disease, surgical trauma (reoperations and complications), specific genotypes and timing of surgery have demonstrated some influence on desmoid rates.Otherwise, type of surgery and surgical approach (open or laparoscopic) are not uniformly correlated with this risk.Conclusions: the information extracted from the literature suggests that our ability to influence desmoid disease development is limited.Integration of clinical and genetic data may help to define a higher risk subgroup of patients who may eventually benefit from specific preventive recommendations and postponed surgical intervention.