Risk of Colorectal Cancer in Juvenile Polyposis

Overview

Journal Gut

Specialty Gastroenterology

Date 2007 Feb 17

PMID 17303595

Citations 90

Authors

Lodewijk A A Brosens

Arnout van Hattem

Linda M Hylind

Christine Iacobuzio-Donahue

Katharine E Romans

Jennifer Axilbund

Marcia Cruz-Correa

Anne C Tersmette

G Johan A Offerhaus

Francis M Giardiello

Affiliations

Soon will be listed here.

Abstract

Background: Juvenile polyposis (JP) is an autosomal-dominant syndrome characterised by the development of hamartomatous gastrointestinal polyps and is associated with colorectal cancer. However, the relative and absolute risk of colorectal malignancy in these patients is not known.

Methods: The incidence rates of colorectal cancer in patients with JP were compared with that of the general population through person-year analysis with adjustment for demographics.

Results: In patients with JP, the RR (95% CI) of colorectal cancer was 34.0 (14.4 to 65.7). Similar risks were noted in both males (30.0, 9.6 to 68.6) and females (43.7, 8.8 to 125). The cumulative life-time risk for colorectal cancer was 38.7%. The mean (SD) age of diagnosis of colorectal cancer was 43.9 (10.4) years. Other gastrointestinal malignancies were not noted in this cohort.

Conclusion: Patients with JP have a markedly increased RR and absolute risk for colorectal cancer and require vigilant colorectal surveillance starting at young age. A low threshold for recommending surgery with consideration for removal of the entire colorectum seems warranted.

Citing Articles

Ulcerative Colitis or Not? A Case of Dysplasia, Gastrointestinal Bleeding, and Juvenile Polyposis in a 27-Year-Old Man.

She T, Ren S, He H, Symer M, Katz S ACG Case Rep J. 2024; 11(7):e01450.

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Peutz-Jeghers Syndrome: A Comprehensive Review of Genetics, Clinical Features, and Management Approaches.

Amru R, Dhok A Cureus. 2024; 16(4):e58887.

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Sporadic gastric juvenile polyposis with a novel SMAD4 nonsense mutation in a mosaic pattern.

Matsuyama S, Fukuda A, Matsumoto A, Eguchi H, Ueo T, Ohana M Clin J Gastroenterol. 2023; 17(1):23-28.

PMID: 37950802 DOI: 10.1007/s12328-023-01884-w.

Genotype-phenotype correlation of BMPR1a disease causing variants in juvenile polyposis syndrome.

Papadopulos M, Plazzer J, Macrae F Hered Cancer Clin Pract. 2023; 21(1):12.

PMID: 37400896 PMC: 10316536. DOI: 10.1186/s13053-023-00255-3.

Genome-wide CRISPR Screen Reveals RAB10 as a Synthetic Lethal Gene in Colorectal and Pancreatic Cancers Carrying SMAD4 Loss.

Erasimus H, Kolnik V, Lacroix F, Sidhu S, DAgostino S, Lemaitre O Cancer Res Commun. 2023; 3(5):780-792.

PMID: 37377893 PMC: 10158796. DOI: 10.1158/2767-9764.CRC-22-0301.

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