Surgical resection for non-familial adenomatous polyposis-related intra-abdominal fibromatosis

Abstract

Abstract Background 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. Methods Data were analysed from a prospectively collected database at a sarcoma tertiary referral centre. Results 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. Conclusion 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.