Cancer risk in patients with Peutz–Jeghers syndrome: A retrospective cohort study of 336 cases
Peutz–Jeghers syndrome is a rare autosomal dominant inherited disorder characterized by mucocutaneous pigmentation and hamartomatous gastrointestinal polyposis. A growing body of evidence has shown that Peutz–Jeghers syndrome could cause an increased risk of various cancers, yet the range of cancer...
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| Format: | Article |
| Language: | English |
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SAGE Publishing
2017-06-01
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| Series: | Tumor Biology |
| Online Access: | https://doi.org/10.1177/1010428317705131 |
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| author | Hong-Yu Chen Xiao-Wei Jin Bai-Rong Li Ming Zhu Jing Li Gao-Ping Mao Ya-Fei Zhang Shou-Bin Ning |
| author_facet | Hong-Yu Chen Xiao-Wei Jin Bai-Rong Li Ming Zhu Jing Li Gao-Ping Mao Ya-Fei Zhang Shou-Bin Ning |
| author_sort | Hong-Yu Chen |
| collection | DOAJ |
| description | Peutz–Jeghers syndrome is a rare autosomal dominant inherited disorder characterized by mucocutaneous pigmentation and hamartomatous gastrointestinal polyposis. A growing body of evidence has shown that Peutz–Jeghers syndrome could cause an increased risk of various cancers, yet the range of cancer risk estimates was wide among different studies. In this retrospective cohort study, 336 patients with Peutz–Jeghers syndrome in China were enrolled. The clinical characteristics, cancer spectrum, relative cancer risks, and cumulative cancer risks were analyzed. In total, 52 patients were diagnosed of cancer in the follow-up period, at a median age of 41 years (range: 21–67). The relative risk for cancer in Peutz–Jeghers syndrome patients was 63.858 (confidence interval: 47.514–85.823), and the cumulative cancer risk at the age of 60 years was 55%. Colorectal cancer was the most common cancer for Peutz–Jeghers syndrome patients (relative risk: 237.918, confidence interval: 154.417–366.572) and the cumulative cancer risk at the age of 60 years was 28%. There was a statistically significant difference in the cumulative cancer risk between patients with family history and those without family history, as well as between patients living in rural area and those living in urban areas ( p < 0.05), while no significant effects of gender and intussusception history on the cumulative cancer risk was found ( p > 0.05). Hopefully, our study may contribute to the management of this rare disorder and establishment of related surveillance projects, especially in China. |
| format | Article |
| id | doaj-art-1a53dab67e78461ba1eb3e4d8f7e70cf |
| institution | DOAJ |
| issn | 1423-0380 |
| language | English |
| publishDate | 2017-06-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | Tumor Biology |
| spelling | doaj-art-1a53dab67e78461ba1eb3e4d8f7e70cf2025-08-20T03:15:10ZengSAGE PublishingTumor Biology1423-03802017-06-013910.1177/1010428317705131Cancer risk in patients with Peutz–Jeghers syndrome: A retrospective cohort study of 336 casesHong-Yu Chen0Xiao-Wei Jin1Bai-Rong Li2Ming Zhu3Jing Li4Gao-Ping Mao5Ya-Fei Zhang6Shou-Bin Ning7Department of Gastroenterology, Clinical College of Air Force General Hospital, Anhui Medical University, Beijing, ChinaDepartment of Gastroenterology, Clinical College of Air Force General Hospital, Anhui Medical University, Beijing, ChinaDepartment of Gastroenterology, Clinical College of Air Force General Hospital, Anhui Medical University, Beijing, ChinaDepartment of Gastroenterology, Clinical College of Air Force General Hospital, Anhui Medical University, Beijing, ChinaDepartment of Gastroenterology, Clinical College of Air Force General Hospital, Anhui Medical University, Beijing, ChinaDepartment of Gastroenterology, Clinical College of Air Force General Hospital, Anhui Medical University, Beijing, ChinaDepartment of Gastroenterology, Zhongnan Hospital of Wuhan University, Wuhan, ChinaDepartment of Gastroenterology, Clinical College of Air Force General Hospital, Anhui Medical University, Beijing, ChinaPeutz–Jeghers syndrome is a rare autosomal dominant inherited disorder characterized by mucocutaneous pigmentation and hamartomatous gastrointestinal polyposis. A growing body of evidence has shown that Peutz–Jeghers syndrome could cause an increased risk of various cancers, yet the range of cancer risk estimates was wide among different studies. In this retrospective cohort study, 336 patients with Peutz–Jeghers syndrome in China were enrolled. The clinical characteristics, cancer spectrum, relative cancer risks, and cumulative cancer risks were analyzed. In total, 52 patients were diagnosed of cancer in the follow-up period, at a median age of 41 years (range: 21–67). The relative risk for cancer in Peutz–Jeghers syndrome patients was 63.858 (confidence interval: 47.514–85.823), and the cumulative cancer risk at the age of 60 years was 55%. Colorectal cancer was the most common cancer for Peutz–Jeghers syndrome patients (relative risk: 237.918, confidence interval: 154.417–366.572) and the cumulative cancer risk at the age of 60 years was 28%. There was a statistically significant difference in the cumulative cancer risk between patients with family history and those without family history, as well as between patients living in rural area and those living in urban areas ( p < 0.05), while no significant effects of gender and intussusception history on the cumulative cancer risk was found ( p > 0.05). Hopefully, our study may contribute to the management of this rare disorder and establishment of related surveillance projects, especially in China.https://doi.org/10.1177/1010428317705131 |
| spellingShingle | Hong-Yu Chen Xiao-Wei Jin Bai-Rong Li Ming Zhu Jing Li Gao-Ping Mao Ya-Fei Zhang Shou-Bin Ning Cancer risk in patients with Peutz–Jeghers syndrome: A retrospective cohort study of 336 cases Tumor Biology |
| title | Cancer risk in patients with Peutz–Jeghers syndrome: A retrospective cohort study of 336 cases |
| title_full | Cancer risk in patients with Peutz–Jeghers syndrome: A retrospective cohort study of 336 cases |
| title_fullStr | Cancer risk in patients with Peutz–Jeghers syndrome: A retrospective cohort study of 336 cases |
| title_full_unstemmed | Cancer risk in patients with Peutz–Jeghers syndrome: A retrospective cohort study of 336 cases |
| title_short | Cancer risk in patients with Peutz–Jeghers syndrome: A retrospective cohort study of 336 cases |
| title_sort | cancer risk in patients with peutz jeghers syndrome a retrospective cohort study of 336 cases |
| url | https://doi.org/10.1177/1010428317705131 |
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