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      Features of incident colorectal cancer in Lynch syndrome

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          Abstract

          <div class="section"> <a class="named-anchor" id="d4847599e209"> <!-- named anchor --> </a> <h5 class="section-title" id="d4847599e210">Background and objective</h5> <p id="d4847599e212">Despite intensive colonoscopic surveillance, a substantial proportion of Lynch syndrome (LS) patients develop colorectal cancer (CRC). The aim of this study was to characterize incident CRC in LS patients. </p> </div><div class="section"> <a class="named-anchor" id="d4847599e214"> <!-- named anchor --> </a> <h5 class="section-title" id="d4847599e215">Methods</h5> <p id="d4847599e217">All patients diagnosed with incident CRC after start of colonoscopic surveillance were identified in the Dutch LS Registry of 905 patients. A retrospective analysis of patient records was carried out for patient characteristics, survival, CRC characteristics and findings of previous colonoscopy. </p> </div><div class="section"> <a class="named-anchor" id="d4847599e219"> <!-- named anchor --> </a> <h5 class="section-title" id="d4847599e220">Results</h5> <p id="d4847599e222">Seventy-one patients (7.8%) were diagnosed with incident CRC. Median interval between incident CRC diagnosis and previous colonoscopy was 23.8 (range 6.7–45.6) months. Median tumor diameter was 2.5 cm, and 17% of the tumors were sessile or flat. Most patients (83%) had no lymph node metastases. There was no association between tumor size and colonoscopy interval or lymph node status. Most patients (65%) had no adenomas during previous colonoscopy. Two patients (2.8%) eventually died from metastatic CRC. </p> </div><div class="section"> <a class="named-anchor" id="d4847599e224"> <!-- named anchor --> </a> <h5 class="section-title" id="d4847599e225">Conclusion</h5> <p id="d4847599e227">The high frequency of incident CRC in LS likely results from several factors. Our findings lend support to the hypothesis of fast conversion of adenomas to CRC, as 65% of patients had no report of polyps during previous colonoscopy. High-quality colonoscopies are essential, especially as tumors and adenomas are difficult to detect because of their frequent non-polypoid appearance. Early detection due to surveillance as well as the indolent growth of CRC, as demonstrated by the lack of lymph node metastases, contributes to the excellent survival observed. </p> </div>

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          Cancer incidence and survival in Lynch syndrome patients receiving colonoscopic and gynaecological surveillance: first report from the prospective Lynch syndrome database

          Pål Møller, Toni Seppälä, Inge Bernstein … (2015)
          Objective Estimates of cancer risk and the effects of surveillance in Lynch syndrome have been subject to bias, partly through reliance on retrospective studies. We sought to establish more robust estimates in patients undergoing prospective cancer surveillance. Design We undertook a multicentre study of patients carrying Lynch syndrome-associated mutations affecting MLH1, MSH2, MSH6 or PMS2. Standardised information on surveillance, cancers and outcomes were collated in an Oracle relational database and analysed by age, sex and mutated gene. Results 1942 mutation carriers without previous cancer had follow-up including colonoscopic surveillance for 13 782 observation years. 314 patients developed cancer, mostly colorectal (n=151), endometrial (n=72) and ovarian (n=19). Cancers were detected from 25 years onwards in MLH1 and MSH2 mutation carriers, and from about 40 years in MSH6 and PMS2 carriers. Among first cancer detected in each patient the colorectal cancer cumulative incidences at 70 years by gene were 46%, 35%, 20% and 10% for MLH1, MSH2, MSH6 and PMS2 mutation carriers, respectively. The equivalent cumulative incidences for endometrial cancer were 34%, 51%, 49% and 24%; and for ovarian cancer 11%, 15%, 0% and 0%. Ten-year crude survival was 87% after any cancer, 91% if the first cancer was colorectal, 98% if endometrial and 89% if ovarian. Conclusions The four Lynch syndrome-associated genes had different penetrance and expression. Colorectal cancer occurred frequently despite colonoscopic surveillance but resulted in few deaths. Using our data, a website has been established at http://LScarisk.org enabling calculation of cumulative cancer risks as an aid to genetic counselling in Lynch syndrome.
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            Controlled 15-year trial on screening for colorectal cancer in families with hereditary nonpolyposis colorectal cancer.

            Heikki J Järvinen, Markku Aarnio, Harri Mustonen … (2000)
            Identification of the hereditary nonpolyposis colorectal cancer (HNPCC) syndrome enables prevention of colorectal cancer (CRC) by means of colonoscopy and polypectomies. We evaluated the efficacy of screening in a controlled trial over 15 years. Incidence of CRC and survival were compared in 2 cohorts of at-risk members of 22 families with HNPCC. Colonic screening at 3-year intervals was arranged for 133 subjects; 119 control subjects had no screening. Genetic testing was offered to subjects in whose families the causative mutation was known. CRC developed in 8 screened subjects (6%) compared with 19 control subjects (16 %; P = 0.014). The CRC rate was reduced by 62%. In mutation-positive subjects alone, the CRC rates were 18% in screened subjects and 41% in controls (P = 0.02). The decrease resulted from the removal of adenomas in 13 mutation-positive individuals (30%) and in 6 subjects with unknown mutation status (40%). All CRCs in the study group were local, causing no deaths, compared with 9 deaths caused by CRC in the controls. The overall death rates were 10 vs. 26 subjects in the study and control groups (P = 0.003), 4 vs. 12 in mutation-positive subjects (P = 0.05). Colonoscopic screening at 3-year intervals more than halves the risk of CRC, prevents CRC deaths, and decreases overall mortality by about 65% in HNPCC families.
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              One to 2-year surveillance intervals reduce risk of colorectal cancer in families with Lynch syndrome.

              Juda Vecht, Silvia Sanduleanu, Fokko Nagengast … (2010)
              Two percent to 4% of all cases of colorectal cancer (CRC) are associated with Lynch syndrome. Dominant clustering of CRC (non-Lynch syndrome) accounts for 1%-3% of the cases. Because carcinogenesis is accelerated in Lynch syndrome, an intensive colonoscopic surveillance program has been recommended since 1995. The aim of the study was to evaluate the effectiveness of this program. The study included 205 Lynch syndrome families with identified mutations in one of the mismatch repair genes (745 mutation carriers). We also analyzed data from non-Lynch syndrome families (46 families, 344 relatives). Patients were observed from January 1, 1995, until January 1, 2009. End points of the study were CRC or date of the last colonoscopy. After a mean follow-up of 7.2 years, 33 patients developed CRC under surveillance. The cumulative risk of CRC was 6% after the 10-year follow-up period. The risk of CRC was higher in carriers older than 40 years and in carriers of MLH1 and MSH2 mutations. After a mean follow-up of 7.0 years, 6 cases of CRC were detected among non-Lynch syndrome families. The risk of CRC was significantly higher among families with Lynch syndrome, compared with those without. With surveillance intervals of 1-2 years, members of families with Lynch syndrome have a lower risk of developing CRC than with surveillance intervals of 2-3 years. Because of the low risk of CRC in non-Lynch syndrome families, a less intensive surveillance protocol can be recommended. Copyright 2010 AGA Institute. Published by Elsevier Inc. All rights reserved.
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                Author and article information

                Journal
                Title: United European Gastroenterology Journal
                Abbreviated Title: United European Gastroenterology Journal
                Publisher: SAGE Publications
                ISSN (Print): 2050-6406
                ISSN (Electronic): 2050-6414
                Publication date Created: June 12 2018
                Publication date Created: October 2018
                Publication date (Electronic): June 11 2018
                Publication date (Print): October 2018
                Volume: 6
                Issue: 8
                Pages: 1215-1222
                Affiliations
                [1 ]Department of Gastroenterology &amp; Hepatology, Leiden University Medical Center, Leiden, The Netherlands
                [2 ]Department of Gastroenterology &amp; Hepatology, University Medical Center Groningen, Groningen, The Netherlands
                [3 ]Department of Gastroenterology &amp; Hepatology, Radboud University Medical Center, Nijmegen, The Netherlands
                [4 ]Department of Gastroenterology &amp; Hepatology, Isala Clinics, Zwolle, The Netherlands
                [5 ]Department of Gastroenterology &amp; Hepatology, Academic Medical Center, Amsterdam, The Netherlands
                [6 ]Department of Surgery, Gelre Hospitals, Apeldoorn, The Netherlands
                Article
                DOI: 10.1177/2050640618783554
                PMC ID: 6169044
                PubMed ID: 30288284
                SO-VID: d6020896-6fca-41c2-9b7e-40f4e51b180f
                Copyright © © 2018
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