title: An atypical presentation of small bowel obstruction and perforation secondary to sporadic synchronous intra-abdominal desmoid tumours.
abstract: BACKGROUND
  Desmoid tumours (DTs) are rare, soft tissue tumours which account for 0.03% of all neoplasms. They are characteristically locally invasive but do not metastasize. There is frequent association with females of reproductive age, a history of abdominal surgery or trauma and a family history of fibromatoses. Intra-abdominal DTs are infrequently sporadic and more commonly associated with inherited disorders such as familial adenomatous polyposis (FAP), attenuated FAP and Gardener's syndrome.
  
  
  METHODS
  The authors report a rare case of small bowel obstruction and perforation secondary to sporadic, synchronous intra-abdominal DTs in a 54-year old man with atypical symptoms and no risk factors or family history.
  
  
  CONCLUSIONS
  Intra-abdominal DTs have a worse prognosis as they can cause intestinal bleeding, obstruction and perforation. Due to the rarity of these tumours there are no clear guidelines on their management and this is instead based on small case series from specialist centres. In the non-acute setting patients with sporadic intra-abdominal DTs should be managed in a specialist sarcoma unit by a multidisciplinary team. In the presence of FAP or other polyposis syndromes patients with DTs should be managed at a specialist colorectal unit. Emergent presentations require emergency surgery in suitable candidates.
  
  
  CONCLUSIONS
  In non-emergency presentations of DTs, it is essential to exclude FAP, AFAP and other hereditary polyposis syndromes since this affects treatment and subsequent follow-up.
authors: Department of General Surgery, University Hospital Lewisham, High Street, Lewisham, London, SE13 6LH, UK. Electronic address: sala3001@doctors.org.uk.
authors: Sala
authors: S
authors: Abdalla
authors: Department of General Surgery, University Hospital Lewisham, High Street, Lewisham, London, SE13 6LH, UK.
authors: Michelle
authors: M
authors: Wilkinson
authors: Department of Histopathology, University Hospital Lewisham, High Street, Lewisham, London, SE13 6LH, UK.
authors: Mark
authors: M
authors: Wilsher
authors: Department of General Surgery, University Hospital Lewisham, High Street, Lewisham, London, SE13 6LH, UK.
authors: Aleksandras
authors: A
authors: Uzkalnis
keywords: Desmoid; Polyposis; Sarcoma; Sporadic