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Multicenter Study
. 2023 Mar;38(2):176-185.
doi: 10.3904/kjim.2022.208. Epub 2022 Dec 15.

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

Su Hwan Kim  1 , Eun Ran Kim  2 , Jae Jun Park  3 , Eun Sun Kim  4 , Hyeon Jeong Goong  5 , Kyeong Ok Kim  6 , Ji Hyung Nam  7 , Yehyun Park  8 , Sang Pyo Lee  9 , Hyun Joo Jang  9 ; KSGE; Research Group of Capsule Endoscopy and Artificial Intelligence in Imaging
Affiliations
Multicenter Study

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

Su Hwan Kim et al. Korean J Intern Med. 2023 Mar.

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.

Conclusion: 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.

Keywords: Neoplasms; Peutz-Jeghers syndrome; Surveillance.

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Conflict of interest statement

No potential conflict of interest relevant to this article was reported.

Figures

Figure 1
Figure 1
Cumulative incidence rates of cancer. (A) Cumulative incidence rates of cancer in total patients with Peutz-Jeghers synsdome. (B) Comparision analysis by gender. There was no difference in cumulative cancer risk between male and female patients.
Figure 2
Figure 2
Cumulative incidence rates of gastrointestinal cancer. (A) Cumulative incidence rates of gastrointestinal cancer in total patients with Peutz-Jeghers syndrome. (B) Comparison analysis by gender. There is no difference between male and female patients.
Figure 3
Figure 3
Cumulative incidence rates of extragastrointestinal cancer. (A) Cumulative incidence rates of extragastrointestinal cancers in total patients with Peutz-Jeghers syndrome (B) Comparison analysis by gender. There is no difference between male and female patients.
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