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Hans F. A. Vasen - One of the best experts on this subject based on the ideXlab platform.
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Duodenal surveillance improves the prognosis after Duodenal Cancer in familial adenomatous polyposis
Colorectal Disease, 2012Co-Authors: S Bulow, Heikki Järvinen, Jan Björk, A Lepistö, Ib Jarle Christensen, H Hojen, M Elmberg, Marry H Nieuwenhuis, Hans F. A. VasenAbstract:Aim Duodenal adenomatosis in familial adenomatous polyposis results in a Cancer risk that increases with age. Endoscopic surveillance has been recommended, but the effect has not yet been documented. The aim of this study was to present the results of long-term Duodenal surveillance and to evaluate the risk of Cancer development. Method Follow up of patients in a previous study with gastroduodenoscopy in 1990–2010. Statistical analysis included the χ2 test, actuarial method and Kaplan–Meier analysis. Results Among 304 patients, 261 (86%) had more than one endoscopy. The median follow up was 14 (interquartile range, 9–17) years. The cumulative lifetime risk of Duodenal adenomatosis was 88% (95% CI, 84–93), and of Spigelman stage IV was 35% (95% CI, 25–45). The Spigelman stage improved in 32 (12%) patients, remained unchanged in 88 (34%) and worsened in 116 (44%). Twenty (7%) patients had Duodenal Cancer at a median age of 56 (range, 44–82) years. The cumulative Cancer incidence was 18% at 75 years of age (95% CI, 8–28) and increased with increasing Spigelman stage at the index endoscopy to 33% in Spigelman stage IV (P < 0.0001). The median overall survival was 6.4 years (95% CI, 1.7 to not estimated): 8 years after a screen-detected Cancer vs 0.8 years (95% CI, 0.03–1.7) after a symptomatic Cancer (P < 0.0001). The location of the mutation in the APC gene did not influence the risk of developing Spigelman stage IV (P = 0.46) or Duodenal Cancer (P = 0.83). Conclusion The risk of Duodenal Cancer in familial adenomatous polyposis is considerable, and regular surveillance and Cancer prophylactic surgery result in a significantly improved prognosis.
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Surgical management for advanced Duodenal adenomatosis and Duodenal Cancer in Dutch patients with familial adenomatous polyposis: A nationwide retrospective cohort study
Surgery, 2012Co-Authors: Bjorn W. H. Van Heumen, Hans F. A. Vasen, Jan Dees, Marry H Nieuwenhuis, Harry Van Goor, Lisbeth Mathus-vliegen, Evelien Dekker, Dirk J. Gouma, Casper H.j. Van Eijck, Fokko M. NagengastAbstract:BACKGROUND: Duodenal Cancer is a major cause of mortality in patients with familial adenomatous polyposis (FAP). The clinical challenge is to perform duodenectomy before Cancer develops; however, procedures are associated with complications. Our aim was to gain insight into the pros and cons of prophylactic duodenectomy. METHODS: Patients with FAP from the nationwide Dutch polyposis registry who underwent prophylactic duodenectomy or were diagnosed with Duodenal Cancer were identified and classified as having benign disease or Cancer at preoperative endoscopy. Surveillance, clinical presentation, surgical management, outcome, survival, and recurrence were compared. RESULTS: Of 1,066 patients with FAP in the registry, 52 (5%; 25 males) were included: 36 with benign adenomatosis (median: 48 years old; including two (6%) Cancer cases diagnosed after operation), and 16 with Cancer (median: 53 years old). Cancer cases had been diagnosed with colorectal Cancer more often (6% vs 44%; P < .01). Forty-three patients underwent duodenectomy (35 benign/eight Cancer): 30-day mortality was 4.7% (n = 2), and in-hospital morbidity occurred in 21 patients (49%), without differences between patients with benign adenomatosis and Cancer. Adenomas recurred in reconstructed proximal small bowel in 14 of 28 patients (50%, median time to recurrence: 75 months), and one patient developed Cancer. Median survival of all 18 Cancer cases in the registry (1.7%; 12 ampullary/six Duodenal) was 11 months. CONCLUSION: Prognosis of Duodenal Cancer in patients with FAP is poor, which justifies an aggressive approach to advanced benign adenomatosis. Strict adherence to recommended surveillance intervals is essential for a well-timed intervention. Given the substantial morbidity and mortality of duodenectomy, patients' individual characteristics are to be critically evaluated preoperatively. As adenomas recur, postoperative endoscopic surveillance is mandatory.
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expanded extracolonic tumor spectrum in mutyh associated polyposis
Gastroenterology, 2009Co-Authors: Stefanie Vogt, Hans F. A. Vasen, Christoph Engel, Natalie Jones, Daria Christian, Maartje Nielsen, Astrid Kaufmann, Verena Steinke, Peter Propping, Julian R SampsonAbstract:Background & Aims MUTYH -associated polyposis (MAP) is characterized by a lifetime risk of colorectal Cancer of up to 100%. However, no systematic evaluation of extracolonic manifestations has been reported. Methods A large cohort of MAP patients was recruited from a European multicenter study. Data were collected on 276 cases from 181 unrelated families. Information on extracolonic tumor spectrum and incidence were evaluated to determine cumulative lifetime risk, which was compared with that of the general population to obtain standardized incidence ratios (SIRs). Results Duodenal polyposis occurred in 17% of cases; the relative risk (SIR) of Duodenal Cancer was 129 (95% confidence interval [CI]: 16–466), whereas the lifetime risk was 4%. The incidence of extraintestinal malignancies among cases was almost twice that of the general population (SIR: 1.9; 95% CI: 1.4–2.5), with a lifetime risk of 38%. We observed a significant increase in the incidence of ovarian, bladder, and skin Cancers (SIR: 5.7, 7.2, and 2.8, respectively) and a trend of increased risk of breast Cancer among cases. The median ages of onset of these 4 malignancies ranged from 51 to 61 years. In contrast to familial adenomatous polyposis, no desmoid tumors were observed, but sebaceous gland tumors, characteristic of the Muir-Torre variant of Lynch syndrome, occurred in 5 patients. Conclusions The relative risks for several extraintestinal malignancies increased in patients with MAP, but based on the spectrum of Cancers (which overlaps with that of Lynch syndrome) and the relatively advanced age at onset, intensive surveillance measures other than frequent endoscopy are unlikely to be helpful to patients with MAP.
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worldwide survey among polyposis registries of surgical management of severe Duodenal adenomatosis in familial adenomatous polyposis
British Journal of Surgery, 2003Co-Authors: W H De Vos Tot Nederveen Cappel, Heikki Järvinen, Jan Björk, T Berk, G Griffioen, Hans F. A. VasenAbstract:Background: The lifetime risk of developing Duodenal Cancer in familial adenomatous polyposis (FAP) is about 5 per cent. When and to what extent surgical intervention should be undertaken to prevent death from invasive carcinoma is controversial. The aim of this study was to determine the effectiveness of various surgical treatments for Cancer and severe Duodenal adenomatosis. Methods: A questionnaire was mailed to the members of the Leeds Castle Polyposis Group to obtain data on patients with FAP, treated for Duodenal Cancer or severe Duodenal adenomatosis after 1990. Results: Sixty-nine patients were included. The indication for surgery was invasive Cancer in 13 patients, of whom six died from metastatic disease. Fifty-six patients were initially treated for severe Duodenal adenomatosis, five (9 per cent) of whom died from metastatic disease (P = 0.002). In surviving patients, adenomas recurred after ampullectomy (six of eight, at mean follow-up of 11 months), after duodenotomy with polypectomy (17 of 21, at mean 29 months) and after pancreatoduodenectomy (six of 25, at mean 47 months). None of six patients who underwent a pancreas-sparing duodenectomy had recurrence of adenoma (mean follow-up 11 months). Conclusion: Surgery for Duodenal adenomatosis should take place before endoscopic biopsy reveals invasive Cancer. Even after extensive surgical procedures, small bowel adenomas may occur, emphasizing the need for chemoprevention.
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Decision analysis in the management of Duodenal adenomatosis in familial adenomatous polyposis.
Gut, 1997Co-Authors: Hans F. A. Vasen, G Griffioen, Lisbeth Mathus-vliegen, Fokko M. Nagengast, Steffen Bülow, Torben Myrhøj, Erik Buskens, B. G. Taal, J.f.m. Slors, P.e. De RuiterAbstract:BACKGROUND: Patients with familial adenomatous polyposis are not only at high risk of developing adenomas in the colorectum but a substantial number of patients also develop polyps in the duodenum. Because treatment of Duodenal polyps is extremely difficult and it is unknown how many patients ultimately develop Duodenal Cancer, the value of surveillance of the upper digestive tract is uncertain. AIMS: (1) To assess the cumulative risk of Duodenal Cancer in a large series of polyposis patients. (2) To develop a decision model to establish whether surveillance would lead to increased life expectancy. METHODS: Risk analysis was performed in 155 Dutch polyposis families including 601 polyposis patients, and 142 Danish families including 376 patients. Observation time was from birth until date of last contact, death, diagnosis of Duodenal Cancer, or closing date of the study. RESULTS: Seven Dutch and five Danish patients developed Duodenal Cancer. The lifetime risk of developing this Cancer by the age of 70 was 4% (95% confidence interval 1-7%) in the Dutch series and 3% (95% confidence interval 0-6%) in the Danish series. Decision analysis showed that surveillance led to an increase in life expectancy by seven months. CONCLUSIONS: Surveillance of the upper digestive tract led to a moderate gain in life expectancy. Future studies should evaluate whether this increase in life expectancy outweighs the morbidity of endoscopic examination and proximal pancreaticoduodenectomy.
Evelien Dekker - One of the best experts on this subject based on the ideXlab platform.
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Duodenal adenomas in patients with multiple colorectal adenomas without germline apc or mutyh mutations
Diseases of The Colon & Rectum, 2017Co-Authors: Frank G J Kallenberg, Sk Clark, Barbara A J Bastiaansen, Andrew Latchford, Nikki C Lips, Cora M Aalfs, Evelien DekkerAbstract:BACKGROUND:Patients with genetic adenomatous polyposis syndromes have an increased risk for Duodenal Cancer, and clear surveillance recommendations exist for this group. However, limited data are available on the Duodenal phenotype of patients with multiple colorectal adenomas (10–99) without a germ
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frequency and features of Duodenal adenomas in patients with mutyh associated polyposis
Clinical Gastroenterology and Hepatology, 2016Co-Authors: Sarahjane Walton, Sk Clark, Evelien Dekker, Frank G J Kallenberg, Andrew LatchfordAbstract:Background & Aims MUTYH-associated polyposis (MAP) is similar to familial adenomatous polyposis (FAP), in that it increases the risk for Duodenal adenomas and Cancer. Almost all patients with FAP develop Duodenal adenomas and 5% develop Duodenal Cancer. Little is known about the prevalence of Duodenal adenomas and Cancer in patients with MAP, but current surveillance recommendations are the same for patients with FAP—they should begin surveillance when they are 25 years old. We aimed to assess the prevalence, extent, and progression of Duodenal adenomas in patients with MAP and evaluate upper gastrointestinal tract surveillance recommendations. Methods In a retrospective study, we collected data on all patients (n = 92) with MAP undergoing surveillance esophagogastroduodenoscopy from registries at St Mark's Hospital (London, UK) and the Academic Medical Center (Amsterdam, The Netherlands) from 2002 through 2014. We collected information on adenoma development, age at adenoma detection, interventions, and disease progression. Results Duodenal adenomas were detected in 31 patients (34%), at a median age of 50 years. When Duodenal polyposis first was detected, it was Spigelman stages I or II in 84% of patients; most had few small polyps, without high-grade dysplasia or villous features. Subsequent esophagogastroduodenoscopy evaluation of 18 of these patients found that 14 (78%) had Spigelman stages 0 to II disease (median follow-up period, 7.8 y). Disease progressed in stage in 6 patients, over 9.5 years, because of lesion size or villous features (2 reached stage IV disease). Adenomas were down-staged in 8 patients after biopsy or polypectomy analyses, and were unchanged for 3 patients. Conclusions In a data analysis from 92 patients with MAP, Duodenal polyposis seemed to develop less frequently than in patients with FAP, and developed at a later age. Increasing lesion size and villous change appear to promote adenoma progression, rather than polyp number or dysplasia. It may be time to consider a new staging system for patients with MAP, to better determine disease severity and surveillance strategies.
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Surgical management for advanced Duodenal adenomatosis and Duodenal Cancer in Dutch patients with familial adenomatous polyposis: A nationwide retrospective cohort study
Surgery, 2012Co-Authors: Bjorn W. H. Van Heumen, Hans F. A. Vasen, Jan Dees, Marry H Nieuwenhuis, Harry Van Goor, Lisbeth Mathus-vliegen, Evelien Dekker, Dirk J. Gouma, Casper H.j. Van Eijck, Fokko M. NagengastAbstract:BACKGROUND: Duodenal Cancer is a major cause of mortality in patients with familial adenomatous polyposis (FAP). The clinical challenge is to perform duodenectomy before Cancer develops; however, procedures are associated with complications. Our aim was to gain insight into the pros and cons of prophylactic duodenectomy. METHODS: Patients with FAP from the nationwide Dutch polyposis registry who underwent prophylactic duodenectomy or were diagnosed with Duodenal Cancer were identified and classified as having benign disease or Cancer at preoperative endoscopy. Surveillance, clinical presentation, surgical management, outcome, survival, and recurrence were compared. RESULTS: Of 1,066 patients with FAP in the registry, 52 (5%; 25 males) were included: 36 with benign adenomatosis (median: 48 years old; including two (6%) Cancer cases diagnosed after operation), and 16 with Cancer (median: 53 years old). Cancer cases had been diagnosed with colorectal Cancer more often (6% vs 44%; P < .01). Forty-three patients underwent duodenectomy (35 benign/eight Cancer): 30-day mortality was 4.7% (n = 2), and in-hospital morbidity occurred in 21 patients (49%), without differences between patients with benign adenomatosis and Cancer. Adenomas recurred in reconstructed proximal small bowel in 14 of 28 patients (50%, median time to recurrence: 75 months), and one patient developed Cancer. Median survival of all 18 Cancer cases in the registry (1.7%; 12 ampullary/six Duodenal) was 11 months. CONCLUSION: Prognosis of Duodenal Cancer in patients with FAP is poor, which justifies an aggressive approach to advanced benign adenomatosis. Strict adherence to recommended surveillance intervals is essential for a well-timed intervention. Given the substantial morbidity and mortality of duodenectomy, patients' individual characteristics are to be critically evaluated preoperatively. As adenomas recur, postoperative endoscopic surveillance is mandatory.
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progression of Duodenal adenomatosis in familial adenomatous polyposis due to ageing of subjects and advances in technology
Familial Cancer, 2011Co-Authors: E M H Mathusvliegen, Karam S. Boparai, Evelien Dekker, Nan Van GelovenAbstract:Familial adenomatous polyposis patients are at risk of Duodenal Cancer. Surveillance is indicated and the extent of Duodenal polyposis is quantified by the Spigelman staging system. We noticed an impressive increase in high Spigelman stages over the years and therefore decided to investigate whether this increase might be due to the time-lapse since the inception of surveillance or related to improvements in endoscopic imaging and/or changes in dysplasia-reporting. Patients who were investigated by the same endoscopist since 1980 in at least 2 different episodes of technical improvements were eligible. The period 1980–2009 was divided into 4 episodes using the following landmarks: replacement of fibre-endoscopes by video-endoscopes in 1987, change in processors in 1995, change in image resolution in 2000, and change in dysplasia-reporting in 2006. An increase in Spigelman stages from low stages (0–II 100%) to high stages (III 28.1%, IV 43.8%) was seen (median follow-up: 19.5 years). In patients who progressed, a median of 4 years elapsed before progression by one stage occurred and 7 years to progress by two stages. In a mixed-model analysis, both time-lapse and technical improvements were determinant factors for Duodenal disease progression. When both factors were introduced in the model, the time-lapse as well as the change in image resolution and dysplasia-ranking contributed consistently in increasing Spigelman scores and stages. The impressive increase in severity of Duodenal polyposis is determined by time-lapse, technological advances and change in dysplasia-reporting. These results might call for a revised Spigelman classification.
Andrew Latchford - One of the best experts on this subject based on the ideXlab platform.
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Duodenal adenomas in patients with multiple colorectal adenomas without germline apc or mutyh mutations
Diseases of The Colon & Rectum, 2017Co-Authors: Frank G J Kallenberg, Sk Clark, Barbara A J Bastiaansen, Andrew Latchford, Nikki C Lips, Cora M Aalfs, Evelien DekkerAbstract:BACKGROUND:Patients with genetic adenomatous polyposis syndromes have an increased risk for Duodenal Cancer, and clear surveillance recommendations exist for this group. However, limited data are available on the Duodenal phenotype of patients with multiple colorectal adenomas (10–99) without a germ
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frequency and features of Duodenal adenomas in patients with mutyh associated polyposis
Clinical Gastroenterology and Hepatology, 2016Co-Authors: Sarahjane Walton, Sk Clark, Evelien Dekker, Frank G J Kallenberg, Andrew LatchfordAbstract:Background & Aims MUTYH-associated polyposis (MAP) is similar to familial adenomatous polyposis (FAP), in that it increases the risk for Duodenal adenomas and Cancer. Almost all patients with FAP develop Duodenal adenomas and 5% develop Duodenal Cancer. Little is known about the prevalence of Duodenal adenomas and Cancer in patients with MAP, but current surveillance recommendations are the same for patients with FAP—they should begin surveillance when they are 25 years old. We aimed to assess the prevalence, extent, and progression of Duodenal adenomas in patients with MAP and evaluate upper gastrointestinal tract surveillance recommendations. Methods In a retrospective study, we collected data on all patients (n = 92) with MAP undergoing surveillance esophagogastroduodenoscopy from registries at St Mark's Hospital (London, UK) and the Academic Medical Center (Amsterdam, The Netherlands) from 2002 through 2014. We collected information on adenoma development, age at adenoma detection, interventions, and disease progression. Results Duodenal adenomas were detected in 31 patients (34%), at a median age of 50 years. When Duodenal polyposis first was detected, it was Spigelman stages I or II in 84% of patients; most had few small polyps, without high-grade dysplasia or villous features. Subsequent esophagogastroduodenoscopy evaluation of 18 of these patients found that 14 (78%) had Spigelman stages 0 to II disease (median follow-up period, 7.8 y). Disease progressed in stage in 6 patients, over 9.5 years, because of lesion size or villous features (2 reached stage IV disease). Adenomas were down-staged in 8 patients after biopsy or polypectomy analyses, and were unchanged for 3 patients. Conclusions In a data analysis from 92 patients with MAP, Duodenal polyposis seemed to develop less frequently than in patients with FAP, and developed at a later age. Increasing lesion size and villous change appear to promote adenoma progression, rather than polyp number or dysplasia. It may be time to consider a new staging system for patients with MAP, to better determine disease severity and surveillance strategies.
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Features of Duodenal Cancer in Patients With Familial Adenomatous Polyposis
Clinical Gastroenterology and Hepatology, 2009Co-Authors: Andrew Latchford, K. Neale, Allan D. Spigelman, R. K. S. Phillips, Susan K. ClarkAbstract:Background & Aims Most patients with familial adenomatous polyposis (FAP) develop Duodenal adenomas; Duodenal Cancer is a major cause of mortality in this patient group. We reviewed cases of Duodenal Cancer in patients with FAP to identify factors that determine long-term Cancer risk. Methods Twenty FAP patients (12 male) were identified from a registry database search. Data from registry and medical notes and endoscopic and histopathologic reports were evaluated. Results Of the Cancers that developed in these patients, 11 were ampullary and 9 were Duodenal. The median age at Cancer diagnosis was 53 years. Seventeen patients died (median age at death, 57 y; median survival from diagnosis, 11 mo); the cause of death was metastatic or Duodenal/ampullary Cancer in 14 patients. Fifteen patients presented symptomatically (including 3 interval Cancers while on surveillance). Two were diagnosed at surveillance and 3 were diagnosed during surgery performed for endoscopic features of advanced benign disease. Duodenal Cancers were associated with a significantly lower mean colonic polyp count than ampullary Cancers (496 ± 282 vs 1322 ± 735; P = .025); there appeared to be familial clustering of this Cancer. When endoscopic data were available (n = 11 of 20), all ampullary Cancers arose from ampullas greater than 1 cm. The Spigelman stage did not predict risk of ampullary Cancer but did predict Duodenal Cancer (median stage 2 vs stage 4 for Duodenal Cancer). Conclusions Once Cancer arises in patients with FAP, prognosis is poor, so Cancer prevention should be the main goal. Surveillance intervals should reflect both Spigelman staging and ampullary disease.
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a 10 year review of surgery for desmoid disease associated with familial adenomatous polyposis
British Journal of Surgery, 2006Co-Authors: Andrew Latchford, K. Neale, N J H Sturt, P A Rogers, R K S PhillipsAbstract:Background: Desmoid tumours affect 10–25 per cent of patients with familial adenomatous polyposis and represent a major cause of morbidity and mortality. Surgery for intra-abdominal desmoids has traditionally been used as a last resort or to manage obstructive complications. The aim was to review 10 years of desmoid surgery in patients with familial adenomatous polyposis from a single centre. Methods: Patients who had surgery for desmoid disease between 1994 and 2004 were identified from the Polyposis Registry database and their hospital notes reviewed. Results: Twenty patients had surgery to remove 32 desmoid tumours (16 intra-abdominal, 12 abdominal wall, four extra-abdominal). Complete clearance was achieved in 19 tumours and, of these, clinically significant recurrence occurred in eight. There was no difference in recurrence rates for site or sex. There was no operative mortality. Intra-abdominal desmoid resection was associated with a mean resection of 45·55 (range 10–200) cm of small bowel. One patient required long-term parenteral feeding. Median follow-up was 5 (range 0·6–10) years. During this period, one patient died (metastatic Duodenal Cancer); there was no mortality from desmoid disease. Conclusion: Surgery for intra-abdominal desmoids in selected patients is less hazardous than previously reported. Surgery for abdominal wall and extra-abdominal tumours is safe. However, disease recurrence remains a major problem. Copyright © 2006 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd.
Russell D. Brown - One of the best experts on this subject based on the ideXlab platform.
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Self-expandable metal stents for malignant gastric outlet obstruction: a modified technique.
Endoscopy, 1998Co-Authors: Rama P. Venu, Bennett J. Pastika, M. Kini, D. Chua, R. Christian, J. Schlais, Russell D. BrownAbstract:Background and study aims Endoscopic palliative treatment may be effective in the management of malignant gastric outlet obstruction. However, experience in this area is limited, and the techniques vary widely. In this retrospective study, a uniform technique using nearly identical self-expandable metal stents was employed to assess technical feasibility, safety, and outcome. Patients and methods Eight patients presenting with clinical findings of gastric outlet obstruction confirmed by upper gastrointestinal radiography underwent endoscopic placement of expandable metal stents. All patients had primary or metastatic malignancy involving the pylorus or duodenum. Endoscopic and Gastrografin-enhanced upper gastrointestinal radiographic evaluations were carried out immediately after stent placement. Complications and clinical outcomes were assessed in each patient. Results Five patients had extrinsic compression of the descending duodenum due to pancreatic Cancer, two had pyloric stenosis from metastatic Cancer, and one patient had primary Duodenal Cancer. Stent placement was successful in all patients, and was followed by clinical improvement. There was one death within 30 days, related to pneumonia. Conclusion Endoscopic self-expandable stent placement appears to be a reasonable therapeutic alternative in patients with malignant gastric outlet obstruction.
Heikki Järvinen - One of the best experts on this subject based on the ideXlab platform.
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Duodenal surveillance improves the prognosis after Duodenal Cancer in familial adenomatous polyposis
Colorectal Disease, 2012Co-Authors: S Bulow, Heikki Järvinen, Jan Björk, A Lepistö, Ib Jarle Christensen, H Hojen, M Elmberg, Marry H Nieuwenhuis, Hans F. A. VasenAbstract:Aim Duodenal adenomatosis in familial adenomatous polyposis results in a Cancer risk that increases with age. Endoscopic surveillance has been recommended, but the effect has not yet been documented. The aim of this study was to present the results of long-term Duodenal surveillance and to evaluate the risk of Cancer development. Method Follow up of patients in a previous study with gastroduodenoscopy in 1990–2010. Statistical analysis included the χ2 test, actuarial method and Kaplan–Meier analysis. Results Among 304 patients, 261 (86%) had more than one endoscopy. The median follow up was 14 (interquartile range, 9–17) years. The cumulative lifetime risk of Duodenal adenomatosis was 88% (95% CI, 84–93), and of Spigelman stage IV was 35% (95% CI, 25–45). The Spigelman stage improved in 32 (12%) patients, remained unchanged in 88 (34%) and worsened in 116 (44%). Twenty (7%) patients had Duodenal Cancer at a median age of 56 (range, 44–82) years. The cumulative Cancer incidence was 18% at 75 years of age (95% CI, 8–28) and increased with increasing Spigelman stage at the index endoscopy to 33% in Spigelman stage IV (P < 0.0001). The median overall survival was 6.4 years (95% CI, 1.7 to not estimated): 8 years after a screen-detected Cancer vs 0.8 years (95% CI, 0.03–1.7) after a symptomatic Cancer (P < 0.0001). The location of the mutation in the APC gene did not influence the risk of developing Spigelman stage IV (P = 0.46) or Duodenal Cancer (P = 0.83). Conclusion The risk of Duodenal Cancer in familial adenomatous polyposis is considerable, and regular surveillance and Cancer prophylactic surgery result in a significantly improved prognosis.
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Surveillance and treatment of Duodenal adenomatosis in familial adenomatous polyposis.
Endoscopy, 2009Co-Authors: A Lepistö, T Kiviluoto, J Halttunen, Heikki JärvinenAbstract:BACKGROUND AND STUDY AIMS: Patients with familial adenomatous polyposis (FAP) are at increased risk for Duodenal Cancer whereas colorectal Cancer is largely prevented by prophylactic colectomy. We analyzed the results of endoscopic surveillance and different treatment modalities of Duodenal adenomatosis in patients with FAP. PATIENTS AND METHODS: Data on endoscopies, histopathological examinations, and surgical therapies were collected from the medical histories of 129 patients with FAP. The cumulative incidences of Duodenal adenomatosis and severe dysplasia and Cancer were calculated using Kaplan-Meier analysis. RESULTS: By the age of 60 years, the cumulative incidence was 80 % for any adenomatosis and 23 % for severe dysplasia or Cancer. Duodenal Cancer was observed in six patients (4.7 %). Fifteen endoscopic excisions in 14 patients, and 19 open duodenotomies in 17 patients were carried out. Later, pancreaticoduodenectomy was undertaken in six (35.3 %) of these 17 patients. Altogether, 12 patients (9.3 %) underwent pancreaticoduodenectomy. Except for one patient, the indication for surgery was based on follow-up endoscopies, and none of these patients died of Duodenal Cancer. No postoperative deaths occurred. Seven patients (58.3 %) had major complications, four (33.3 %) of which were surgical. CONCLUSIONS: The high incidence of severe dysplasia and Cancer in Duodenal polyps suggests that endoscopic surveillance is essential. Endoscopic polypectomies under sedation anesthesia have partly replaced open duodenotomies. High-risk patients with Spigelman IV adenomatosis or adenomas with persisting severe dysplasia should undergo surgery with pylorus-preserving pancreaticoduodenectomy before invasive Cancer develops.
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worldwide survey among polyposis registries of surgical management of severe Duodenal adenomatosis in familial adenomatous polyposis
British Journal of Surgery, 2003Co-Authors: W H De Vos Tot Nederveen Cappel, Heikki Järvinen, Jan Björk, T Berk, G Griffioen, Hans F. A. VasenAbstract:Background: The lifetime risk of developing Duodenal Cancer in familial adenomatous polyposis (FAP) is about 5 per cent. When and to what extent surgical intervention should be undertaken to prevent death from invasive carcinoma is controversial. The aim of this study was to determine the effectiveness of various surgical treatments for Cancer and severe Duodenal adenomatosis. Methods: A questionnaire was mailed to the members of the Leeds Castle Polyposis Group to obtain data on patients with FAP, treated for Duodenal Cancer or severe Duodenal adenomatosis after 1990. Results: Sixty-nine patients were included. The indication for surgery was invasive Cancer in 13 patients, of whom six died from metastatic disease. Fifty-six patients were initially treated for severe Duodenal adenomatosis, five (9 per cent) of whom died from metastatic disease (P = 0.002). In surviving patients, adenomas recurred after ampullectomy (six of eight, at mean follow-up of 11 months), after duodenotomy with polypectomy (17 of 21, at mean 29 months) and after pancreatoduodenectomy (six of 25, at mean 47 months). None of six patients who underwent a pancreas-sparing duodenectomy had recurrence of adenoma (mean follow-up 11 months). Conclusion: Surgery for Duodenal adenomatosis should take place before endoscopic biopsy reveals invasive Cancer. Even after extensive surgical procedures, small bowel adenomas may occur, emphasizing the need for chemoprevention.
