Intra-abdominal fibromatosis (IAF) in the context of familial adenomatosis polyposis (FAP) is associated with significant morbidity and high recurrence rates after surgical resection. Non-surgical treatments are therefore advocated. This study explored outcomes in patients with IAF not associated with FAP who underwent surgical resection. Data were analysed from a prospectively collected database at a sarcoma tertiary referral centre. From 2001 to 2011, 15 patients without FAP underwent primary curative surgical resection of IAF. Their median (range) age was 42 (19–64) years. Median tumour size was 18 (8·5–25) cm and weight 1306 (236–2228) g. Complete macroscopic clearance was obtained in all patients. There were no deaths in hospital or within 30 days and only one patient developed a major complication. Median follow-up was 40 (6–119) months. During follow-up two patients developed a recurrence after a disease-free interval of 12 and 16 months.In contrast to FAP-associated IAF, non-FAP-associated IAF has a very low recurrence rate after surgical resection. Surgical resection is therefore advocated as first-line treatment in patients with non-FAP-associated IAF when resection can be performed with low morbidity.
Mesenteric fibromatosis is a benign fibrous tumor, characterized by proliferations of fibroblasts and myofibroblasts, locally aggressive but non-metastasizing. It can occur rarely in association with familial adenomatous polyposis or sporadically (related with previous trauma, abdominal surgery o...
Desmoid tumours are a rare group of tumours arising in the deep musculoaponeurotic structures and although they have no metastatic potential they can be locally aggressive with relapse rates of between 23–40%. Three sub-sites are reported: extra-abdominal, abdominal wall and intra-abdominal. Th...
Desmoids are myofibroblastic proliferations occurring in 15% of patients with familial adenomatous polyposis (FAP), 70% being intra-abdominal desmoids (IAD). Since the morbidity and mortality due to desmoids is almost entirely attributable to IAD, we aimed to identify specifically risk factors pr...
Desmoid tumours (DT) are myofibroblastic proliferations found in 10-25% of familial adenomatous polyposis (FAP) patients, either intra-abdominally (IA), in the abdominal wall (AW) or elsewhere (extra-abdominal (EA)). Most DT occur following prophylactic colectomy but occasionally patients present...
Laparoscopic colonic surgery is now widely accepted. We assessed the safety and effectiveness of using a total intracorporeal surgical strategy to perform intracorporeal functional end-to-end anastomosis with an endoscopic linear stapler to treat colon cancer. Forty-three selected patients underw...
Duodenal polyps are a rare finding in patients presenting for gastroscopy, being found in 0.3–4.6% of cases. The majority of patients are asymptomatic. The most common lesions necessitating removal are duodenal adenomas which should be differentiated from other mucosal lesions such as ectop...
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Management and outcomes for duodenal adenomas may vary based on etiology, familial versus sporadic. We reviewed the records of patients managed at our institution for duodenal adenomatous polyps for the 20-year period ending July 2006. Discussion Methods of polyp resection (endoscopic, local surg...
Familial adenomatous polyposis [FAP (OMIM 175100)] is an autosomal dominant colorectal cancer predisposition syndrome characterized by hundreds to thousands of colonic polyps and, if untreated by a combination of screening and/or surgical intervention, a ~99% lifetime risk of colorectal cancer. A...
In this retrospective study, 28 years of surgical treatment of children and young adults with familial adenomatous polyposis (FAP) was analyzed.Forty-three patients were operated on before the age of 26 years. Endoscopic aspects, operative data, and complications were analyzed, and the resection ...
Intra-abdominal fistulas occur in one-third of patients with Crohn's disease (CD). Although they are common, these fistulas may pose difficult problems for the surgeon. We assessed the clinical presentation of intra-abdominal fistulas in patients with CD and compared the clinicopathologic charact...
