Korean J Intern Med.
2023 Mar;38(2):176-185. 10.3904/kjim.2022.208.
Cancer risk in patients with Peutz-Jeghers syndrome in Korea: a retrospective multi-center study
- Kim SH1
- Kim ER2
- Park JJ3
- Kim ES4
- Goong HJ5
- Kim KO6
- Nam JH7
- Park Y8
- Lee SP9
- Jang HJ
9 - KSGE1
- Research Group of Capsule Endoscopy and Artificial Intelligence in Imaging1
- Affiliations
-
- 1Department of Internal Medicine, Seoul Metropolitan Government Seoul National University Boramae Medical Center, Seoul National University College of Medicine, Seoul, Korea
- 2Division of Gastroenterology, Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
- 3Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
- 4Department of Gastroenterology, Korea University College of Medicine, Seoul, Korea
- 5Digestive Disease Center and Research Institute, Department of Internal Medicine, Soonchunhyang University Bucheon Hospital, Soonchunhyang University College of Medicine, Bucheon, Korea
- 6Division of Gastroenterology and Hepatology, Department of Internal Medicine, Yeungnam University College of Medicine, Daegu, Korea
- 7Department of Internal Medicine, Dongguk University Ilsan Hospital, Dongguk University College of Medicine, Goyang, Korea
- 8Department of Internal Medicine, Ewha Womans University Seoul Hospital, Seoul, Korea
- 9Division of Gastroenterology, Department of Internal Medicine, Hallym University Dongtan Sacred Heart Hospital, Hallym University College of Medicine, Hwaseong, Korea
- KMID: 2539970
- DOI: http://doi.org/10.3904/kjim.2022.208
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.
