Duodenal adenomatosis in familial adenomatous polyposis

S Bülow; J Björk; I J Christensen; O Fausa; H Järvinen; F Moesgaard; H F A Vasen

doi:10.1136/gut.2003.027771

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Colon cancer

Duodenal adenomatosis in familial adenomatous polyposis

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Authors reply to Drs. Latchford and Phillips
Steffen Bülow
Published on: 18 April 2016
Duodenal adenoma and cancer in FAP
Andrew R Latchford
Published on: 18 April 2016
Authors reply to Dr Biasco et al.
Steffen Bülow
Published on: 18 April 2016
The risk of duodenal cancer in patients with Familial Adenomatous Polyposis
Guido Biasco
Published on: 18 April 2016

Published on: 18 April 2016
Authors reply to Drs. Latchford and Phillips
- Steffen Bülow, Consultant surgeon
- Other Contributors:
  Jan Björk, Ib J. Christensen, Olav Fausa, Heikki Järvinen, Flemming Moesgaard, and Hans F.A. Vasen
Dear Editor
This letter is in response to the letter by Latchford and Phillips.[1]
We agree that the ideal upper endoscopy for evaluation of duodenal adenomatosis in FAP should be performed with a side viewing as well as a forward viewing endoscope, but as pointed out by Latchford & Phillips we underlined and discussed this problem in our article.[2] Our Nordic- Dutch study was planned in the late 1...
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Dear Editor
This letter is in response to the letter by Latchford and Phillips.[1]
We agree that the ideal upper endoscopy for evaluation of duodenal adenomatosis in FAP should be performed with a side viewing as well as a forward viewing endoscope, but as pointed out by Latchford & Phillips we underlined and discussed this problem in our article.[2] Our Nordic- Dutch study was planned in the late 1980s and at that time we found that the use of side viewing endoscopy was unrealistic in a five nation multicenter study, as the majority of local endoscopists responsible for the FAP study were not routinely performing ERCP. We have clearly stated in the discussion that this may have led to an underestimation of the stage of adenomatosis.
We also agree about the possibility of bias caused by older patients with less severe disease and being included in the calculation of cancer incidence with no adjustment for age. However, the fact that there is an increased risk of Spigelman stage IV and cancer with age does not preclude that these patients are representative. Anyhow, all major series in the literature have the same problem, as a large cohort of patients followed from young age with regular upper gastrointestinal endoscopic surveillance has not yet been published.
We do not agree that the 12 patients treated with open duodenotomy and polypectomy may have biased the natural incidence of duodenal adenoma, as the adenoma recurrence rate is known to be very high after this procedure. It may, however, have had an influence on the staging of adenomatosis, but we consider this to be minimal.
Sulindac has not been used very often for FAP in the Nordic countries or the Netherlands. In the present study we have information of its use in short periods in 15 patients and therefore we consider the potential bias to be neligible.
The "take home message" of our article is that adult FAP patients should be offered regular upper endoscopy due to the risk of carcinoma development, which is increasing with age. We are convinced that this is in total agreement with the attitude of our colleagues at St. Mark's Hospital.
References
1. Latchford AR, Phillips PKS. Duodenal adenoma and cancer in FAP (electronic response to Bülow et al. Duodenal adenomatosis in familial adenomatous polyposis)
2. Bülow S, Björk J, Christensen IJ, Fausa O, Moesgaard F, and Vasen HFA. Duodenal adenomatosis in familial adenomatous polyposis. Gut 2004; 53: 381-6.
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Conflict of Interest:
None declared.
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Published on: 18 April 2016
Duodenal adenoma and cancer in FAP
- Andrew R Latchford, Clinical Research Fellow
- Other Contributors:
  Robin KS Phillips
Dear Editor
We congratulate the authors on gathering this large cohort of patients in this important area in FAP but would like to express some reservations with regards the study. Our first concern relates to the means of endoscopic assessment. Standard forward viewing endoscopy was used, whereas in clinical practice side viewing endoscopy is recommended since duodenal polyposis in FAP is more severe in the peria...
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Dear Editor
We congratulate the authors on gathering this large cohort of patients in this important area in FAP but would like to express some reservations with regards the study. Our first concern relates to the means of endoscopic assessment. Standard forward viewing endoscopy was used, whereas in clinical practice side viewing endoscopy is recommended since duodenal polyposis in FAP is more severe in the periampullary region[1] and this is likely to be missed with standard endoscopy. This will therefore underestimate both adenoma staging and frequency. This matter is raised in their discussion where they describe side-viewing endoscopy as unrealistic. We however feel this is unreasonable in an era where ERCP services are available in most hospitals, at least in the UK.
Furthermore the need for appropriate endoscopy technique and biopsy protocols has been highlighted in a recent study,[2] which revealed understaging of duodenal disease when comparing biopsy specimens and resected specimens, in addition to the finding of invasive cancer in a number of specimens resected for “severe duodenal adenomatosis” (ie Spigelman stage 3 with high grade dysplasia or stage 4). The need to operate before biopsy proven carcinoma is demonstrated by the high mortality rates from metastatic disease in those with duodenal carcinoma. Accurate staging and assessment for endoscopic or surgical intervention is, in our opinion, not possible by standard forward viewing endoscopy.
Our other concern relates to the quoted cancer incidence, which we feel must be biased. They do not have a cohort which has been followed up since they were young, but rather have a cohort whose age range at first endoscopy is 20-81 years. Both the authors' own data and others have shown that there is an increased risk of stage 4 disease and cancer with increasing age. As such those older patients in this group we would expect to be self selected to have less severe disease. Those who were destined to develop severe duodenal disease or cancer may well have developed it prior to screening. Of note the median age of those developing cancer was 52 (range 26-58).
In addition those 12 patients undergoing open duodenotomy and polypectomy are likely to be those with the most advanced disease and highest risk of malignant transformation, thus once again biasing the likely natural incidence of duodenal carcinoma.
There are also few details with regards medical intervention, which may affect duodenal staging and disease progression. In the discussion the authors mention that a few patients may have been on periodic sulindac but feel that this will have a negligible impact on their analysis. In the present era of selective Cox 2 inhibition,[3] our current clinical practice is to consider celecoxib treatment in those with stage 3 or 4 disease as well as those who have undergone surgical intervention. If this practice were followed using sulindac in the centres involved in this trial, then up to 91 (24%) patients in this cohort could have been exposed to NSAIDs.
References
1. Spigelman AD, Williams CB, Talbot IC, Domizio P, Phillips RKS. Upper gastrointestinal cancer in patients with familial adenomatous polyposis. Lancet 1989;2:783-785.
2. Bulow S, Bjork J, Christensen IJ, Fausa O, Jarvinen H, Moesgaard F, et al. Duodenal adenomatosis in familial adenomatous polyposis. Gut 2004;53(3):381-6.
3. Phillips RK, Wallace MH, Lynch PM, Hawk E, Gordon GB, Saunders BP, et al. A randomised, double blind, placebo controlled study of celecoxib, a selective cyclooxygenase 2 inhibitor, on duodenal polyposis in familial adenomatous polyposis. Gut. 2002;50(6):857-60.
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Conflict of Interest:
None declared.
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Published on: 18 April 2016
Authors reply to Dr Biasco et al.
- Steffen Bülow
Dear Editor
This letter is in response to the letter by Biasco et al.[1]
In our study [2] of 368 patients with a median follow-up of 91 months the cumulative incidence of duodenal adenomatosis Spigelman stage IV was 52% at age 70, which is in fact almost the same life time risk as the 50% found by Saurin et al.[3] and 20-30% in the Swedish and Finnish series.[4,5]
In conclusion, all major...
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Dear Editor
This letter is in response to the letter by Biasco et al.[1]
In our study [2] of 368 patients with a median follow-up of 91 months the cumulative incidence of duodenal adenomatosis Spigelman stage IV was 52% at age 70, which is in fact almost the same life time risk as the 50% found by Saurin et al.[3] and 20-30% in the Swedish and Finnish series.[4,5]
In conclusion, all major studies find that the risk of advanced duodenal adenomatosis is increasing with age to a high level, thereby indicating an increasing risk of duodenal carcinoma and justification of regular endoscopic surveillance.
References
1. Biasco G. The risk of duodenal cancer in patients with Familial Adenomatous Polyposis [electronic response to Bülow et al. Duodenal adenomatosis in familial adenomatous polyposis] gutjnl.com 2004http://gut.bmjjournals.com/cgi/eletters/53/3/381#305
2. S Bülow, J Björk, I J Christensen, O Fausa, H Järvinen, F Moesgaard, and H F A Vasen. Duodenal adenomatosis in familial adenomatous polyposis. Gut 2004; 53: 381-386
3. Saurin JC et al. Surveillance of duodenal adenomas in familial adenomatous polyposis reveals high cumulative risk of advanced disease. J Clin Oncol 2004; 22: 493-8.
4. Björk J et al. Periampullary adenomas and adenocarcinomas in familial adenomatous polyposis: cumulative risks and APC gene mutations. Gastroenterology 2001; 121: 1127-35.
5. Heiskanen I et al. Management of duodenal adenomas in 98 patients with familial adenomatous polyposis. Endoscopy 1999; 31: 412-6.
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Conflict of Interest:
None declared.
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Published on: 18 April 2016
The risk of duodenal cancer in patients with Familial Adenomatous Polyposis
- Guido Biasco, Professor
- Other Contributors:
  Maria A. Pantaleo, Giulio Di Febo, Carlo Calabrese and Giovanni Brandi
Dear Editor
Bülow and coworkers published the results of a prospective multicenter study addressed to the analysis of the natural history of duodenal adenomas in Familial Adenomatous Polyposis (FAP).[1]
368 patients were examined in a mean period of 7.6 years (range 0.5-10.4). They show a significative progression of Spigelman stage over time (p< 0.0001). At the end of the study the incidence of Spigel...
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Dear Editor
Bülow and coworkers published the results of a prospective multicenter study addressed to the analysis of the natural history of duodenal adenomas in Familial Adenomatous Polyposis (FAP).[1]
368 patients were examined in a mean period of 7.6 years (range 0.5-10.4). They show a significative progression of Spigelman stage over time (p< 0.0001). At the end of the study the incidence of Spigelman stage IV was 7.0%. These results are lower than those reported in a similar study recently published on JCO (35% incidence of Spigelman stage IV).[2] This discrepancy is not a case. The available literature on this issue reports not homogenous data. Our own experience is somewhat different. On 30 FAP patients, who entered in a study of biological markers some years ago,[3] the development of most severe Spigelman stage was negligible after 7-18 years of surveillance (only one case from stage II to stage III). We have never found patients with stage IV or cancer. It probably could be due to different selection of the patients. For example, in both the Gut and JCO papers data about colorectal surgery are not reported. Proctocolectomy affects the bile acids metabolisms and circulation.[4] Bile acids are involved in the development of duodenal neoplasia.[5]
All the patients we have under surveillance had a proctocolectomy with J- pouch ileo-anal anastomosis (IAA). Probably they have an impairment of the bile acids pool, thus leading to a less risk of duodenal neoplasia. In the Bülow‘s group a separate analysis on operated and not operated patients may be very informative in this concerns.
References
1. Bülow S, Björk J, Christensen IJ, and the DAF Study Group. Duodenal adenomatosis in familial adenomatous polyposis. Gut 2004; 53: 381-386.
2. Saurin J-C, Gutnknecht C, Napoleon B, et al. Surveillance of duodenal adenomas in Familial Adenomatous Polyposis reveals high cumulative risk of advanced disease. J Clin Oncol 2004; 22: 493-98.
3. Santucci R, Volpe L, Zannoni U, et al. Cell Proliferation of the Duodenal Mucosa in Patients Affected by Familial Adenomatous Polyposis. Gastroenterology 1997; 113: 1159-1162.
4. Pereira SP, Bain IM, Kumar D, et al. Bile composition in inflammatory bowel disease: ileal disease colectomy, but not colitis, induce lithogenic bile. Aliment Pharmacol Ther 2003; 17: 923-933.
5. Phillips RKS, Wallace MH, et al. A randomized, double blind, placebo controlled study of celecoxib, a selective cyclooxygenase 2 inhibitor, on duodenal polyposis in familial adenomatous polyposis. Gut 2002; 50: 857- 860.
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Conflict of Interest:
None declared.
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