Conclusions

Abdominal wall and mesenteric DTs are a common manifestation in patients with FAP. The natural his tory is extremely variable and largely depends on the site of DT and its growth rate. Previous abdominal surgery, family history of DTs, APC germline mutation distal to codon 1444 and the female gender significantly increase the susceptibility of developing DTs. Prophylactic colectomy may be delayed in women with an attenuated FAP and in patients belonging to a family with evidence of DTs in more than 50% of the members. It seems probable that an attenuated form of mesenteric fibrosis represents the precursor of infiltrating fibromatosis and large mass. Even if the majority of DTs grow slowly and are asymptomatic, a minority of DTs may present a fast increase causing serious compression of intraabdominal structures and life-threatening complications. In recent years, research has clarified the mechanism of actions of NSAIDs or SERMs and the rationale for their use in DTs. Considering the low toxicity of these drugs, they must be considered either as a first-line treatment when a DT or a mesen-teric fibromatosis is diagnosed or as a preventive measure when DPLs are discovered at surgery. A close surveillance of the lesions by regular clinical and imaging assessment is mandatory. Progression of the tumour or occurrence of symptoms despite this treatment should promptly indicate cytotoxic chemotherapy. The medical treatment must be pursued for a long time, since shrinkage of DTs can be delayed by months or even years. However, regression can continue after discontinuation of the therapy. Surgical therapy is indicated when its consequences are not detrimental. Therefore, only extraabdominal DTs or small mesenteric DTs that are located far from the mesenteric vessels and do not require a large intestinal resection, are susceptible of surgical resection. Postoperative therapy for prevention of recurrence is indicated.

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