[Current diagnosis and treatment of desmoid tumours in patients with familial adenomatous polyposis - the surgical view]

Abstract

Based on a representative selection of relevant references, the aim of this study was to reflect the change of the algorithm in the surgical management of desmoid tumours (DT) in cases of accompanying familial adenomatous polyposis (FAP). Main focus is concerned with the basics of differential treatment, including additional considerations on epidemiology, diagnosis, outcome and follow-up. DT are rare benign tumours that do not metastasise but tend to invade locally. In contrast to the general population, DT in patients with FAP are more common, show a different pattern of tumour sites and cause considerable morbidity and mortality. Most DT occur in the abdominal cavity and account for the majority of serious problems. Genetic disposition and hormonal factors as well as prior surgical trauma are considered causative for the development of DT. Characteristic symptoms are abdominal pain, nausea and vomiting but DT may also present as acute abdomen. CT scan determines localisation and extension of the tumour. Treatment includes various strategies of medication, surgical resection and radiation. Data concerning diagnostic and therapeutic procedures are based on studies with small case series or case reports only. Therefore data from international multicentre studies are necessary for improving the prognosis and developing reliable and stringent guidelines.