{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T22:31:56Z","timestamp":1792621916716,"version":"4.5.0"},"reference-count":25,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2018,6,6]],"date-time":"2018-06-06T00:00:00Z","timestamp":1528243200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Lynch syndrome (LS) and familial adenomatous polyposis (FAP) are autosomal dominant hereditary diseases caused by germline mutations leading to the development of colorectal cancer. Moreover, these mutations result in the development of a spectrum of different tumors, including gastric cancers (GCs). Since the clinical characteristics of GCs associated with LS and FAP are not well known, we investigated clinical and molecular features of GCs occurring in patients with LS and FAP attending our Institution. The Hereditary Tumor Registry was established in 1994 at the Department of Oncologic Gastroenterology, CRO Aviano National Cancer Institute, Italy. It includes 139 patients with LS and 86 patients with FAP. Patients were recruited locally for prospective surveillance. Out of 139 LS patients, 4 developed GC\u20143 in the presence of helicobacter pylori infection and 1 on the background of autoimmune diseases. All GCs displayed a high microsatellite instability (MSI-H) and loss of related mismatch repair (MMR) protein. One of the FAP patients developed a flat adenoma, displaying low-grade dysplasia at the gastric body, and another poorly differentiated adenocarcinoma with signet ring cells like Krukenberg without HP infection. LS carriers displayed a risk of GC. The recognition of HP infection and autoimmune diseases would indicate those at higher risk for an endoscopic surveillance. Regarding FAP, the data suggested the need of suitable endoscopic surveillance in long survivals with diffuse fundic gland polyps.<\/jats:p>","DOI":"10.3390\/ijms19061682","type":"journal-article","created":{"date-parts":[[2018,6,6]],"date-time":"2018-06-06T07:38:15Z","timestamp":1528270695000},"page":"1682","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":34,"title":["Molecular and Pathological Features of Gastric Cancer in Lynch Syndrome and Familial Adenomatous Polyposis"],"prefix":"10.3390","volume":"19","author":[{"given":"Mara","family":"Fornasarig","sequence":"first","affiliation":[{"name":"SOC di Gastroenterologia Oncologica, Centro di Riferimento Oncologico IRCSS, 33081 Aviano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raffaella","family":"Magris","sequence":"additional","affiliation":[{"name":"SOC di Gastroenterologia Oncologica, Centro di Riferimento Oncologico IRCSS, 33081 Aviano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6100-9373","authenticated-orcid":false,"given":"Valli","family":"De Re","sequence":"additional","affiliation":[{"name":"SOSD Immunopatologia e biomarcatori Oncologico, Centro di Riferimento Oncologico IRCSS, 33081 Aviano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ettore","family":"Bidoli","sequence":"additional","affiliation":[{"name":"SOC di Epidemiologia, Centro di Riferimento Oncologico IRCSS, 33081 Aviano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6010-0976","authenticated-orcid":false,"given":"Vincenzo","family":"Canzonieri","sequence":"additional","affiliation":[{"name":"SOSD di Anatomia Patologica, Centro di Riferimento Oncologico IRCSS, 33081 Aviano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefania","family":"Maiero","sequence":"additional","affiliation":[{"name":"SOC di Gastroenterologia Oncologica, Centro di Riferimento Oncologico IRCSS, 33081 Aviano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandra","family":"Viel","sequence":"additional","affiliation":[{"name":"SOSD Oncogenetica e Oncogenomica Funzionale, Centro di Riferimento Oncologico IRCSS, 33081 Aviano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2020-222X","authenticated-orcid":false,"given":"Renato","family":"Cannizzaro","sequence":"additional","affiliation":[{"name":"SOC di Gastroenterologia Oncologica, Centro di Riferimento Oncologico IRCSS, 33081 Aviano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2018,6,6]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"M\u00f8ller, P., Sepp\u00e4l\u00e4, T.T., Bernstein, I., Holinski-Feder, E., Sala, P., Evans, D.G., Lindblom, A., Macrae, F., Blanco, I., and Sijmons, R.H. 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