Progression of duodenal adenomatosis in familial adenomatous polyposis: due to ageing of subjects and advances in technology

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

Hyperplastic polyposis syndrome: a pilot study for the differentiation of polyps by using high-resolution endoscopy, autofluorescence imaging, and narrow-band imaging

BACKGROUND: Endoscopic differentiation and removal of potentially premalignant sessile serrated adenomas (SSAs) may be important steps in preventing the development of colorectal cancer in hyperplastic polyposis syndrome (HPS). OBJECTIVE: To assess the value of high-resolution endoscopy, autofluorescence imaging (AFI), and narrow-band imaging (NBI) for differentiating polyps in HPS. DESIGN: A prospective polyp series. SETTING: Single tertiary referral center. PATIENTS AND INTERVENTIONS: Seven patients with HPS underwent colonoscopy with endoscopic trimodal imaging, which incorporates high-resolution endoscopy, AFI, and NBI in 1 system. All detected polyps were analyzed with AFI for color and with NBI for Kudo pit pattern and vascular pattern intensity. MAIN OUTCOME MEASUREMENTS: The accuracy, sensitivity, and specificity of AFI and NBI in differentiating detected polyps were determined by using histology as the criterion standard. RESULTS: A total of 19 hyperplastic polyps (HPs), 32 SSAs, and 15 adenomas were detected. For differentiating SSAs from HPs, AFI color, Kudo pit pattern, and vascular pattern intensity resulted in a diagnostic accuracy of 55%, 55%, and 52%, respectively. For differentiating adenomas from HPs, the accuracy was 65%, 94%, and 90%, respectively. Macroscopically, the combination of a size of 3 mm or larger and a proximal location resulted in the highest accuracy (76%) for differentiating SSAs from HPs. LIMITATION: Small sample size. CONCLUSION: Endoscopic differentiation between HPs and SSAs by using endoscopic trimodal imaging proved unsatisfactory. Differentiation of adenomas from HPs was possible with NBI but not with AF

The long-term outcomes and natural disease course of serrated polyposis syndrome: over 10 years of prospective follow-up in a specialized center

Background and Aims: Serrated polyposis syndrome (SPS) is the most prevalent colonic polyposis syndrome known and is associated with a high risk of colorectal cancer (CRC) if left untreated. Treatment consists of clearance of the initial polyp burden, followed by lifelong stringent endoscopic surveillance. However, the long-term safety and efficacy of surveillance and the natural disease course after initial clearance have not been described in detail. Methods: We analyzed a single-center cohort of patients with SPS with over 10 years of prospective follow-up. Outcome measures were (1) CRC incidence, (2) postcolonoscopy adverse event rates, and (3) trends in polyp recurrence during endoscopic surveillance. Results: The cohort included 142 patients who underwent a median of 6 colonoscopies with a median of 47 months of prospective follow-up after initial polyp clearance. During surveillance (every 1-2 years), 1 case of CRC occurred (5-year CRC incidence, 1.0%; 95% confidence interval, 0%-2.9%). During 447 surveillance colonoscopies with 1308 polypectomies, 1 episode of postpolypectomy bleeding, 1 postpolypectomy syndrome, and no perforations occurred (adverse event rate, 0.45% per colonoscopy). During up to 9 rounds of surveillance, no upward or downward trend in polyp recurrence was observed. Conclusions: In this prospective cohort with over 10 years of follow-up, endoscopic surveillance was effective and safe, with a low risk of CRC and colonoscopy-related adverse events. Furthermore, we show that the disease course of SPS is such that the polyp burden remains more or less equal during long-term surveillance, which advocates lifelong adherence to (personalized) surveillance guidelines and discourages de-intensifying surveillance intervals after multiple rounds of surveillance

Yield of Screening Colonoscopy in First-degree Relatives of Patients With Serrated Polyposis Syndrome

Goals: We aimed to evaluate the diagnostic yield of screening colonoscopies in first-degree relatives (FDRs) of patients with serrated polyposis syndrome (SPS). Background: Patients with SPS are at an increased risk for colorectal cancer. Although inheritance patterns are unknown, FDRs of these patients have an increased risk for both colorectal cancer and SPS. Prospective studies evaluating the yield of screening colonoscopies in this group are however scarce. This information would be useful to evaluate a possible mode of inheritance and to investigate whether screening colonoscopies are justified in this group. Study: FDR of patients with SPS were invited to undergo colonoscopy. The diagnostic yield was expressed by the number of FDRs with at least 1 significant polyp relative to the total number of included FDRs. Significant polyps were defined adenomas, traditional serrated adenomas, sessile serrated adenoma/ polyp, or proximal hyperplastic polyp. Tissue specimens were reviewed by one expert pathologist. Results: Seventy-seven FDRs underwent colonoscopy (median age 52 y; interquartile range, 41 to 60). Colorectal cancer was not diagnosed. One or more significant polyps were detected in 43% of FDRs. No differences based on age, gender, or familial relationship were observed in the detection of polyps. Seven first-degree (9%) relatives had multiple polyps (>= 5). Eleven (14%) FDRs fulfilled SPS WHO-criterion 2, of whom 1 sibling also met SPS WHO-criterion 3. Conclusions: The yield of a single screening colonoscopy in FDRs of patients with serrated polyposis is substantial, warranting a colonoscopy screening program for these individuals

Hyperplastic polyps and sessile serrated adenomas as a phenotypic expression of MYH-associated polyposis

BACKGROUND & AIMS: MYH-associated polyposis (MAP) is a disorder caused by a bi-allelic germline MYH mutation, characterized by multiple colorectal adenomas. These adenomas typically harbor G:C-->T:A transversions in the APC and K-ras genes caused by MYH deficiency. Occasional hyperplastic polyps (HPs) have been described in MAP patients but a causal relationship has never been investigated. We examined the presence of HPs and sessile serrated adenomas (SSAs) in 17 MAP patients and studied the occurrence of G:C-->T:A transversions in the APC and K-ras gene in these polyps. METHODS: MAP patients were analyzed for the presence of HPs/SSAs. APC-mutation cluster region and K-ras codon 12 mutation analysis was performed in adenomas (n = 22), HPs (n = 63), and SSAs (n = 10) from these patients and from a control group of sporadic adenomas (n = 17), HPs (n = 24), and SSAs (n = 17). RESULTS: HPs/SSAs were detected in 8 of 17 (47%) MAP patients, of whom 3 (18%) met the criteria for hyperplastic polyposis syndrome. APC mutations were detected only in adenomas and comprised exclusively G:C-->T:A transversions. K-ras mutations were detected in 51 of 73 (70%) HPs/SSAs in MAP patients, compared with 7 of 41 (17%) sporadic HPs/SSAs in the control group (P T:A transversions, compared with 2 of 7 (29%) sporadic HPs/SSAs in the control group (P T:A transversions in the K-ras gene of HPs/SSAs strongly suggests that these polyps are related causally to MYH deficiency. This implies that distinct pathways, that is, APC-gene related in adenomas and nonrelated in HPS/SSAs, appear to be operational in MA