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Cancer risk in patients with Peutz-Jeghers syndrome in Korea: a retrospective multi-center studyopen accessCancer risk in patients with Peutz-Jeghers syndrome in Korea: a retrospective multi-center study

Other Titles
Cancer risk in patients with Peutz-Jeghers syndrome in Korea: a retrospective multi-center study
Authors
Kim, Su HwanKim, Eun RanPark, Jae JunKim, Eun SunGoong, Hyeon JeongKim, Kyeong OkNam, Ji HyungPark, YehyunLee, Sang PyoJang, Hyun JooResearch Group of Capsule Endoscopy and Artificial Intelligence in Imaging
Issue Date
Mar-2023
Publisher
대한내과학회
Keywords
Neoplasms; Peutz-Jeghers syndrome; Surveillance
Citation
The Korean Journal of Internal Medicine, v.38, no.2, pp 176 - 185
Pages
10
Indexed
SCIE
SCOPUS
KCI
Journal Title
The Korean Journal of Internal Medicine
Volume
38
Number
2
Start Page
176
End Page
185
URI
https://scholarworks.korea.ac.kr/kumedicine/handle/2021.sw.kumedicine/62301
DOI
10.3904/kjim.2022.208
ISSN
1226-3303
2005-6648
Abstract
Background/Aims There have been little research on the cancer risks of patients with Peutz-Jeghers syndrome (PJS) in Korea. We aimed to investigate the clinical features of PJS patients and their cancer incidence rate. Methods Patients with PJS from nine medical centers were enrolled. In those patients diagnosed with cancer, data obtained included the date of cancer diagnosis, the tumor location, and the cancer stage. The cumulative risks of gastrointestinal cancers and extra-gastrointestinal cancers were calculated using the Kaplan-Meier method. Results A total of 96 PJS patients were included. The median age at diagnosis of PJS was 23.4 years. Cancer developed in 21 of the 96 patients (21.9%). The age of PJS diagnosis was widely distributed (0.9 to 72.4 years). The most common cancers were gastrointestinal cancer (n = 12) followed by breast cancer (n = 6). The cumulative lifetime cancer risk was calculated to be 62.1% at age 60. The cumulative lifetime gastrointestinal cancer risk was 47.1% at age 70. The cumulative lifetime extra-gastrointestinal cancer risk was 40.3% at age 60. Conclusions PJS onset may occur at any age and the risks of gastrointestinal and extra-gastrointestinal cancer are high. Thorough surveillance of PJS patients for malignancies is vital.
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Kim, Eun Sun
Anam Hospital (Department of Gastroenterology and Hepatology, Anam Hospital)
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