Duodenal surveillance improves the prognosis after duodenal cancer in familial adenomatous polyposis

Bülow, Steffen; Christensen, I. J.; Højen, H.; Björk, Jan; Elmberg, Maria; Järvinen, Heikki; Lepistö, Anna; Nieuwenhuis, Marry H.; Vasen, Hans F. A.

doi:10.1111/j.1463-1318.2011.02844.x

Cited by 69 publications

(90 citation statements)

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“…Also similar to our results, earlier reports have suggested that severity of colorectal polyps could predict the development of severe duodenal adenomatosis. 25 Studies evaluating a genotype-phenotype correlation between specific mutation regions of the APC gene and the severity of duodenal adenomatosis have yielded inconsistent results 11,24 ; these data were not collected in this study.…”

Section: Discussionmentioning

confidence: 70%

“…2,24,26 Few studies have shown a definite improvement in outcomes in patients with advanced duodenal polyposis, a high-risk group of patients with the highest rate of duodenal cancer. 10,11 Most reports suggest the benefits and recommend endoscopic surveillance of duodenal adenomas to prevent cancer. 2,6,[10][11][12]26,27 The results of our protocol are good and will serve to give clinicians a practical and effective guide for surveillance and management of patients with duodenal adenomatosis.…”

Section: Discussionmentioning

confidence: 98%

“…Guidelines for the frequency of surveillance and management of duodenal lesions are not uniform and clinical outcome has been disappointing in some series, which have reported risk of progression to duodenal cancer in the range of 22% to 36% in patients with advanced duodenal polyposis. [10][11][12] The relative shortcomings of surveillance programs are in part due to a lack of knowledge concerning the risk of malignant progression of periampullary adenomas and controversy regarding the appropriate degree of aggressiveness necessary to eradicate periampullary polyps, thus preventing periampullary cancer. 13 The morbidity and potential mortality of endoscopic or operative management of duodenal disease must be weighed against the risk of developing duodenal adenocarcinoma, with an understanding that most interventions will not completely prevent recurrence of disease and that lifelong surveillance might be indicated for selected patients.…”

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confidence: 99%

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Progression and Management of Duodenal Neoplasia in Familial Adenomatous Polyposis

et al. 2015

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Section: Discussionmentioning

confidence: 70%

Section: Discussionmentioning

confidence: 98%

mentioning

confidence: 99%

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Progression and Management of Duodenal Neoplasia in Familial Adenomatous Polyposis

et al. 2015

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“…However, few reports have described a correlation between FAP and polyposis or carcinoma in the hepatopancreaticobiliary tract. The cumulative lifetime risk of developing severe duodenal polyposis has been estimated to be around 35% (28). Fundic gland polyps of the stomach also can occur in the majority of FAP patients that are too numerous to count.…”

Section: Resultsmentioning

confidence: 99%

The Surgical Treatment Options for Familial Adenomatous Polyposis: A Clinic Experience

Tarcan¹,

Turanlı²,

Güler³

et al. 2016

Acta Oncol Tur.

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ÖZET Amaç: Ailesel adenomatöz polipoziste (AAP) cerrahi tedavi, abdominal kolektomi-ileorektal anastomoz (IRA), restoratif proktokolektomi-ileal J poş anal anastomoz (IPAA) ve total proktokolektomi-kalıcı ileostomiyi içerir. Bu çalışma ile hangi tip cerrahi yöntemin en iyi seçenek olduğu irdelendi. Gereç ve Yöntemler: Ailesel adenomatöz polipozis tanısıyla 2004-2014 yılları arasında aynı merkezde cerrahi girişim uygulanmış 20 hasta çalışmaya alındı. Hastalar uygulanan ameliyat türüne göre 3 gruba ayrıldı. Demografik özellikler, ameliyat bulguları, patoloji sonuçları ve klinik seyirlerine ilişkin veriler geriye dönük olarak hastane kayıtlarından elde edildi. Bulgular: Yirmi hastada AAP tanısı kolonoskopi ve patoloji raporuna dayanılarak konuldu. Bunların 15'inde kanser gelişmişken 5 hastada kolonoskopik taramada kanser henüz gelişmeden tanı konuldu. Kanser gelişen ve gelişmeyen hastaların ortanca yaşı sırasıyla 37 ve 20 yaş. Dört hastaya IPAA, 4 hastaya IRA ve geri kalan 12 hastaya total proktokolektomi kalıcı ileostomi uygulandı. Hastaların hepsinde 100 ve üzerinde kolonda polip vardı. Hastalar ortanca 38 aya takip edildi. Bu takip esnasında 8 hastada (%40.0) tekrarlayan veya metastatik hastalık gelişti. Bunlar; 4 hastada uzak metastaz, 3 hastada rektum kanseri (hepsine daha önce IRA yapılmış) ve bir hastada ise desmoid tümör şeklindeydi. Ayrıca IRA yapılmış 1 hastada rektumda adenom gelişti. Ileal poş adenomu ya da ileostomide adenom gelişimi izlenmedi. Dört hasta hastalığa bağlı kaybedildi. Bunlardan 3'ü metastatik hastalık gelişimine bağlıyken kalan 1 hastada dev desmoid tümörün neden olduğu böbrek yetmezliği ölüm sebebiydi. Sonuç: Ameliyat öncesi kanser tanısı konulmuş klasik AAP hastalarında cerrahi yöntem olarak abdominal kolektomi-IRA seçimi iyi bir seçenek olmayabilir. Ancak çalışmanın az sayıda hastayı kapsadığı da göz önünde bulundurulmalıdır. Anahtar Kelimeler: Ailesel adenomatöz polipozis, kolektomi, proktokolektomi, karsinom ABSTRACT Objective: Familial adenomatous polyposis (FAP) is treated by abdominal colectomy with ileorectal anastomosis (IRA), restorative proctocolectomy with ileal J-pouch anal anastomosis (IPAA), and total proctocolectomy with ileostomy. This study investigated question is what type of surgery is better. Material and Method: Twenty FAP patients, who underwent surgery in a single hospital between 2004 and 2014, were included. Patients were classified in 3 groups according to the operation procedures. Demographic, surgical, pathological, and outcome data were analyzed from hospital records retrospectively. Results: Overall 20 patients were included, 15 index patients, and 5 relatives diagnosed by screening. Median age of patients with or without colorectal cancer was 37 and 20 years respectively. Four patients were treated by IPAA, 4 by IRA, and 12 by proctocolectomy. All patients presented with 100 or more colonic polyps. The median follow-up time was 38 months. During the follow-up period, 8 (40.0%) patients developed recurrent or metastatic disease. There were; distant metast...

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“…As a result, the mortality pattern has changed and duodenal cancer now is the leading cancer-related cause of death [3,4]. Lifetime risk of duodenal adenomas approaches 100% in patients with FAP [5], and approximately 3-7% of patients eventually develop duodenal cancer [6,7]. As duodenal cancer in patients with FAP has been associated with a poor prognosis [8,9], the clinical challenge is to identify patients with high-risk duodenal adenomas and intervene before progression to cancer occurs.…”

Section: Introductionmentioning

confidence: 99%

Duodenal mucosal risk markers in patients with familial adenomatous polyposis: effects of celecoxib/ursodeoxycholic acid co-treatment and comparison with patient controls

Heumen

Roelofs

Morsche

et al. 2013

Orphanet J Rare Dis

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BackgroundFamilial adenomatous polyposis (FAP) is a disease characterized by the development of hundreds to thousands of adenomatous polyps in the colorectum early in life. Virtually all patients with FAP will develop colorectal cancer before the age of 40 to 50 years, unless prophylactic colectomy is performed, which significantly improves their prognosis. The mortality pattern has changed and duodenal cancer now is one of the main cancer-related causes of death in these patients. Practically all patients with FAP develop premalignant duodenal adenomas, which may develop to duodenal cancer in approximately 3-7% of patients. Duodenal cancer in patients with FAP has a poor prognosis. The clinical challenge is to identify patients at high-risk for duodenal carcinoma. Chemoprevention would be desirable to avoid duodenectomy. The main goal of this study is to identify risk markers in normal duodenal mucosa of patients with FAP, that could help identify patients at increased risk for malignant transformation.MethodsMessenger RNA (mRNA) levels of glutathione S-transferase A1 (GSTA1), glutathione S-transferase P1 (GSTP1), KIAA1199, E-cadherin, peroxisome proliferative activated receptor δ (PPARδ), caspase-3, cyclin D1, β-catenin, and cyclooxygenase-2 (COX-2) were measured in duodenal mucosa, using the QuantiGene 2.0 Plex assay. Levels in normal appearing mucosa of patients with FAP (n = 37) were compared with levels in non-FAP patient controls (n = 16). In addition, levels before and after treatment with either celecoxib & ursodeoxycholic acid (UDCA, n = 14) or celecoxib & placebo (n = 13) were evaluated in patients with FAP.ResultsmRNA levels of glutathione S-transferase A1 (28.16% vs. 38.24%, p = 0.008) and caspase-3 (3.30% vs. 5.31%, p = 0.001) were significantly lower in patients with FAP vs. non-FAP patient controls, respectively. COX-2 mRNA levels in normal duodenal mucosa of patients with FAP were found to be unexpectedly low. None of the potential risk markers was influenced by celecoxib or celecoxib & UDCA.ConclusionsProtection against toxins and carcinogens (GSTA1) and apoptosis (caspase-3) is low in patients with FAP, which could contribute to increased susceptibility for malignant transformation of duodenal mucosa.Trial registrationhttp://ClinicalTrials.gov number NCT00808743

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Duodenal surveillance improves the prognosis after duodenal cancer in familial adenomatous polyposis

Abstract: The risk of duodenal cancer in familial adenomatous polyposis is considerable, and regular surveillance and cancer prophylactic surgery result in a significantly improved prognosis.

Cited by 69 publications

References 25 publications

Progression and Management of Duodenal Neoplasia in Familial Adenomatous Polyposis

Progression and Management of Duodenal Neoplasia in Familial Adenomatous Polyposis

The Surgical Treatment Options for Familial Adenomatous Polyposis: A Clinic Experience

Duodenal mucosal risk markers in patients with familial adenomatous polyposis: effects of celecoxib/ursodeoxycholic acid co-treatment and comparison with patient controls

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