The case in question is discussed and images of radiological and histological findings are included. A literature review was undertaken to identify similar cases and review latest management guidelines.
This 65 year old man presented with acute epigastric pain and vomiting and peritonitis on examination. He proceeded to emergency laparotomy which revealed a well-circumscribed cystic mass arising from the small bowel mesentery as well as purulent fluid in the pelvis. The tumour was resected and he made a good post-operative recovery. A follow-up colonoscopy screening for Familial Adenomatous Polyposis was negative. Histology demonstrated an intra-abdominal desmoid tumour. There were focal areas of necrosis and bacterial colonies and vegetable particles were identified within the tumour suggesting fistualisation into adjacent bowel.
A literature review revealed that as the disease is rare, there are no clear management guidelines. Surgery is a first-line option in readily resectable disease, however the disease has a high recurrence rate despite resection (up to 60 percent at 5 years) and surgery itself is a known risk factor for desmoid tumour formation. In patients with Familial Adenomatous Polyposis decisions around prophylactic colectomy must be balanced against the risk of precipitating desmoid tumours. Other management options include a ‘watch and wait’ approach, radiotherapy (which is generally not recommended for intra-abdominal tumours) and systemic therapies. These include NSAIDs, tamoxifen, toremifene, methotrexate and vinblastine, low-dose interferon, doxorubicin-based regimens, and imatinib.
This case is of interest as while a number of case reports describe desmoid tumours presenting as a palpable abdominal mass, complication with necrosis or abscess is rare. As discussed above, management options are varied and somewhat controversial and therefore provide an interesting discussion point.