M J Wilkinson1, J E F Fitzgerald, J M Thomas, A J Hayes, D C Strauss. 1. Sarcoma and Melanoma Unit, Department of Academic Surgery, Royal Marsden Hospital NHS Foundation Trust, Fulham Road, London SW3 6JJ, UK. michelle.wilkinson@icr.ac.uk
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.
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.
Authors: Dave Moore; Lucy Burns; Ben Creavin; Eanna Ryan; Kevin Conlon; Michael Eamon Kelly; Dara Kavanagh Journal: Ir J Med Sci Date: 2022-04-21 Impact factor: 1.568