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Only a minority of patients at high likelihood of a gastrointestinal polyposis syndrome (GPS) are appropriately referred for workup. This proof-of-concept study evaluates a GPS screening rubric based exclusively on information in prior pathology reports and intended to facilitate pathologist engagement in GPS screening and referral. We sought to (1) identify patients who would benefit from further GPS workup, (2) assign a probable polyposis syndrome category (adenomatous, hamartomatous, serrated, or mixed), and (3) suggest a specific syndrome, such as familial adenomatous polyposis, whenever possible. We retrospectively tested the rubric against the pathology records of 108 patients (median, 6 reports/patient) with an established clinical diagnosis of GPS (adenomatous (N = 88), hamartomatous (N = 18), and mixed (N = 2) polyposis syndromes). Records were reviewed chronologically (mean, 4.4 min/patient) by a GI pathologist blinded to clinical history. Ninety-five patients (88%) had a positive GPS screen (N = 76 with an adenomatous polyposis syndrome, N = 17 with a hamartomatous polyposis syndrome, N = 2 with a mixed polyposis syndrome); all were assigned to the correct syndrome category. In a subset of cases, the histopathologic record suggested a specific syndrome (correct in 28 of 30 instances). Of 13 patients with a negative screen (failure to meet any rubric parameters), N = 6 (46.2%) had incomplete records. These findings demonstrate that when robust records are available, structured review of pathology reports is a sensitive and efficient tool for the identification of patients with a high suspicion of a GPS. While prospective studies are necessary, pathologists are indeed well positioned to play an expanded role in GPS screening.
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http://dx.doi.org/10.1007/s00428-025-04118-1 | DOI Listing |
Fam Cancer
September 2025
Cancer Genetics Service, Division of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore.
This study compares three hereditary colorectal cancer (CRC) registries-the Iranian Hereditary Colorectal Cancer Registry (IHCCR), the Singapore Polyposis Registry (SPR), and the University of Cape Town Familial CRC Registry-to illuminate diverse approaches to identification, management, and research across different healthcare systems. Each registry, while emphasizing patient diversity, employed unique strategies reflecting available resources and epidemiological contexts. The IHCCR, leveraging WES, revealed considerable genetic heterogeneity, including novel mutations.
View Article and Find Full Text PDFWorld J Clin Oncol
August 2025
Department of Pathology, Faculty of Medicine, Umm Al-Qura University, Makkah 21955, Saudi Arabia.
Background: Familial adenomatous polyposis (FAP) is an autosomal dominant syndrome that results from a germline mutation in the adenomatous polyposis coli gene. It is characterized by the early development of hundreds of adenomas in the colon during the second decade of life. If prophylactic colectomy is not performed, most patients eventually develop colorectal cancer (CRC).
View Article and Find Full Text PDFCureus
July 2025
Department of Gastroenterology, Omihachiman Community Medical Center, Omihachiman, JPN.
Cronkhite-Canada syndrome (CCS) is a rare, non-hereditary disease characterized by specific symptoms, ectodermal abnormalities, endoscopic findings, and pathological examination. We report a unique case of a 39-year-old man with suspicious CCS. Although the blood test showed iron-deficiency anemia and hypoalbuminemia, he exhibited no symptoms, including those related to CCS itself and anemia and hypoalbuminemia.
View Article and Find Full Text PDFCureus
July 2025
Gastroenterology, Sengkang General Hospital, Singapore, SGP.
Fundic gland polyps (FGPs) are the most common type of gastric polyps and are frequently discovered incidentally during upper endoscopy. Long-term use of proton pump inhibitors (PPIs) has been associated with an increased risk of developing FGPs. We present a case of PPI-induced FGP in a 78-year-old woman who had been taking omeprazole daily for 15 years and was referred for evaluation of anemia.
View Article and Find Full Text PDFHum Mutat
August 2025
Advanced Center for Translational and Genetic Medicine, Stanley Manne Children's Research Institute, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, USA.
Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant disorder hallmarked by mucocutaneous melanocytic macules and gastrointestinal hamartomatous polyposis associated with germline/somatic pathogenic variants in the tumor suppressor . PJS is clinically heterogeneous; however, the relationship between clinical phenotype and genotype remains elusive. Here, we report a family with PJS that harbors a heterozygous whole gene deletion combined with a heterozygous variant in that segregates with mucocutaneous pigmentation in the family.
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