Korean J Intern Med. 2023;38(2):176-185.

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

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제1저자 김수환, 김은란 · 교신저자 장현주

PMID 36517957 PMC9993101 DOI 10.3904/kjim.2022.208 IF 2.2 소화기내과

소속 (9)

Department of Internal Medicine, Seoul Metropolitan Government Seoul National University Boramae Medical Center, Seoul National University College of Medicine, Seoul, Korea.
Division of Gastroenterology, Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
Department of Gastroenterology, Korea University College of Medicine, Seoul, Korea.
Digestive Disease Center and Research Institute, Department of Internal Medicine, Soonchunhyang University Bucheon Hospital, Soonchunhyang University College of Medicine, Bucheon, Korea.
Division of Gastroenterology and Hepatology, Department of Internal Medicine, Yeungnam University College of Medicine, Daegu, Korea.
Department of Internal Medicine, Dongguk University Ilsan Hospital, Dongguk University College of Medicine, Goyang, Korea.
Department of Internal Medicine, Ewha Womans University Seoul Hospital, Seoul, Korea.
Division of Gastroenterology, Department of Internal Medicine, Hallym University Dongtan Sacred Heart Hospital, Hallym University College of Medicine, Hwaseong, Korea.

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

NeoplasmsPeutz-Jeghers syndromeSurveillance

Figures

**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** 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** 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.

그림 출처: PubMed Central 공개 논문 (PMC9993101)

Conflict of interest statement

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

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