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

被引：6

作者：

Kim, Su Hwan ^{[1
]}

Kim, Eun Ran ^{[2
]}

Park, Jae Jun ^{[3
]}

Kim, Eun Sun ^{[4
]}

Goong, Hyeon Jeong ^{[5
,6
]}

Kim, Kyeong Ok ^{[7
]}

Nam, Ji Hyung ^{[8
]}

Park, Yehyun ^{[9
]}

Lee, Sang Pyo ^{[10
]}

Jang, Hyun Joo ^{[10
]}

机构：

[1] Seoul Natl Univ Coll Med, Seoul Metropolitan Govt Seoul Natl Univ Boramae M, Dept Internal Med, Seoul, South Korea

[2] Sungkyunkwan Univ, Samsung Med Ctr, Dept Internal Med, Div Gastroenterol,Sch Med, Seoul, South Korea

[3] Yonsei Univ, Severance Hosp, Dept Internal Med, Coll Med, Seoul, South Korea

[4] Korea Univ, Dept Gastroenterol, Coll Med, Seoul, South Korea

[5] Soonchunhyang Univ, Coll Med, Bucheon Hosp, Digest Dis Ctr, Bucheon, South Korea

[6] Soonchunhyang Univ, Coll Med, Bucheon Hosp, Res Inst,Dept Internal Med, Bucheon, South Korea

[7] Yeungnam Univ, Dept Internal Med, Div Gastroenterol & Hepatol, Coll Med, Daegu, South Korea

[8] Dongguk Univ, Coll Med, Dept Internal Med, Ilsan Hosp, Goyang, South Korea

[9] Ewha Womans Univ, Dept Internal Med, Seoul Hosp, Seoul, South Korea

[10] Hallym Univ, Coll Med, Dept Internal Med, Div Gastroenterol,Dongtan Sacred Heart Hosp, 7 Keunjaebong Gil, Hwaseong 18450, South Korea

来源：

KOREAN JOURNAL OF INTERNAL MEDICINE | 2023年 / 38卷 / 02期

关键词：

Neoplasms; Peutz-Jeghers syndrome; Surveillance; THYROID-CARCINOMA; SURVEILLANCE; MANAGEMENT; FREQUENCY; UPDATE;

D O I：

10.3904/kjim.2022.208

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：176 / +

页数：11

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