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No guidelines for desmoid tumors (DT) management are available and DT have now become the first cause of death in FAP patients who had restorative proctocolectomy. We aimed to assess the results of the different treatments used for DT in Familial Adenomatous Polyposis (FAP) patients. All patients followed for FAP who developed a DT between 1970 and 2010 were collated. We analysed separately the history of DT according to location: mesenteric, parietal or mixed. 79 FAP patients [45 females (56 %); mean age 33.3 ± 12.5] presented 149 DT and were included; 16(20 %) had a DT diagnosed during or before first abdominal surgery and 47 (59 %) had isolated mesenteric DT. 11 patients had only surgical treatment, 17 only medical treatments, 31 had combined treatment and 20 had no treatment with spontaneous DT regression or stabilization. Overall, 80 treatment lines were administered to patients with a progression free or regression rate of 43 % (34/80). Response rates were: chemotherapy 77 % (10/13); Sulindac + tamoxifen 50 % (6/12); Tamoxifen 40 % (6/15); Imatinib 36 % (4/11); Sulindac 28 % (8/29). Among the 42 surgical procedures, an R0 resection was performed in 26 (62 %) allowing the absence of recurrence for 54 %. After a median follow-up of 81 months, 8 patients died of their DT and 6 died of other cause. Overall and DT-specific survival at 20 years were 52 and 79 %, respectively. Chemotherapy was the most efficient treatment. For intra-abdominal DT, we consider it as a first choice treatment and reserve surgery when it is impossible or when DT are life threatening.

Background: In locally advanced rectal cancer (LARC) patients, major response to neoadjuvant radiotherapy (NR) has been associated with favorable long-term outcomes. Positive pathologic nodal status was recently proven to be associated with poor prognosis even after total regression of primary tumor (ypT0). The aim of this study was to evaluate the rate of lymph node (LN) involvement in patients with complete (ypT0) or major (TRG1: very few viable tumor cells) response., Methods: Included were patients with complete or major response after radiotherapy followed by surgery and histological examination of the whole specimen., Results: From 1996 to 2010, 245 patients with LARC were treated by NR. We collected clinical data for 53 patients (21.6 %) with ypT0 (n = 26, 49 %) or TRG1 (n = 27, 51 %) response. Sphincter-preserving surgery was performed in 40 patients (75 %). Overall, nine patients (16.9 %) presented LN involvement: 2 (7.7 %) in the ypT0 group and 7 (25.9 %) in the TRG1 group (NS). Patients with ypT3 tumors had significantly more invaded LN than patients with ypT1-T2 tumors (6 of 13 [46 %] vs 1 of 14 [7 %], p = .032). After median follow-up of 30 months (range, 1-160 months), 5-year disease-free and overall survivals were 88.2 and 89.0 %, respectively., Conclusions: There was a clear cutoff between patients with ypT0-T2 (3 of 40, 7.5 %) and ypT3 (6 of 13, 46 %) concerning the incidence of metastatic LN in patients achieving pathologic complete or major response after NR. In patients with good clinical response, local full-thickness resection of the residual tumor could be a first step, followed by standard rectal resection in cases of ypT3.

Background: Despite the results of combined chemoradiation therapy for anal canal squamous cell carcinoma (SCC), up to 30 % of patients will undergo abdominoperineal resection (APR). The aim of this study was to evaluate oncologic outcomes, survival, and recurrence, following APR for anal canal SCC performed in a single center over a 13-year period., Methods: All patients who underwent APR for anal canal SCC between 1996 and 2009 were retrospectively included. Demographic data, details on treatments, pathological report, and follow-up were noted. Survival curves were plotted using the Kaplan-Meier method and potential prognostic factors were evaluated using Cox proportional hazards models., Results: A total of 105 patients (77 women) were included. Indications for APR included tumor persistence (n = 42; 40 %), recurrence (n = 55; 52.4 %), or a contraindication to radiotherapy (n = 8; 7.6 %). Median follow-up was 33.3 months (range, 1.5-174.3 months). Overall survival and disease-free survival were, respectively, 61 and 48 % at 5 years. In multivariate analysis, tumor stage (T3 or T4), positive margin on pathologic examination and existence of distant metastases at the time of the surgery were associated with a poor prognosis. The indication for APR (persistent vs recurrent disease), gender, concurrent HIV infection, or performance of a VRAM flap did not influence OS or DFS. Overall recurrence rate was 42.6 % (n = 43 of 101). The type of recurrence did not exert a significant effect on survival (p = .4571)., Conclusion: This study describes the largest single series of APR for anal carcinoma. Major prognostic factors for survival and recurrence were T status and involved margin. The 5-year overall survival was 60 %.

Purpose: Pancreaticoduodenectomy is an alternative to pancreas-sparing duodenectomy for radical treatment of duodenal lesions. The aims of this study were to assess the results of pylorus-preserving pancreaticoduodenectomy (PPPD) for severe duodenal polyposis in familial adenomatous polyposis in terms of morbidity, long-term influence on functional results, the recurrence rate of cancer or jejunal polyps, and survival., Methods: All patients operated on for a PPPD between 1992 and 2009 were included. Clinical data, endoscopic findings, and pathologic examinations were evaluated., Results: A total of 19 patients underwent PPPD for severe duodenal polyposis (17 Spigelman IV, 1 Spigelman III, and 1 invasive carcinoma). Postoperative mortality was nil. The postoperative morbidity rate was 42%, including 4 pancreatic fistulae (21%) and 2 delayed gastric emptying (11%). Pathologic examination found 7 invasive carcinomas, of which only 1 was known before resection. One third of patients operated on without a preoperative diagnosis of malignancy already had an invasive duodenal carcinoma. After a mean follow-up of 58 months, 16 patients were alive. Thirteen patients underwent endoscopic follow-up, and new adenomas were found in 4 (31%). All were treated successfully during the same endoscopic procedure. PPPD did not modify the functional result after coloproctectomy., Conclusions: PPPD remains a safe and efficient therapeutic option for severe duodenal polyposis in familial adenomatous polyposis patients.

Background: Complete cytoreductive surgery (CCRS) with intraperitoneal chemotherapy (IPC) is becoming the gold-standard treatment for resectable peritoneal carcinomatosis, when feasible. However, this approach has not yet been evaluated for isolated peritoneal re-recurrences after previous IPC. The aim of this study was to evaluate the postoperative course and long-term outcome after repeat (re-)CCRS + IPC., Methods: From 1996 to 2007, 25 re-CCRS + IPC were performed in 20 patients with isolated peritoneal re-recurrences, among 393 CCRS + IPC performed during the same period in the department. Selection was based on the supposedly limited extent of the peritoneal disease and an interval of more than 12 months between the first CCRS + IPC procedure and recurrence. After a re-CCRS, we used re-IPC modalities (method and/or drug) that were systematically different from those of the first IPC. The origins of the tumors were pseudomyxoma (n = 12), colorectal cancer (n = 4), mesothelioma (n = 3), and carcinoid tumor (n = 1)., Results: At laparotomy, mean peritoneal index was 7.6 +/- 4.8. Among the 25 procedures, hyperthermic intraperitoneal chemotherapy was used in 15 cases and early postoperative intraperitoneal chemotherapy in 10 cases. The postoperative mortality rate was 4% (n = 1) and six grade 3-4 postoperative complications occurred. The overall 5- and 10-year actuarial survival rates were 72.5% and 58%, respectively. The 5-year disease-free survival rate was 19%. The long-term outcome was not different, whatever the type of primary tumor., Conclusion: Re-CCRS + IPC is feasible and yields prolonged survival in highly selected patients. Also, the switch to another type of IPC or regimen may have contributed to this prolonged survival.