Retrospective Study
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jan 26, 2020; 8(2): 264-275
Published online Jan 26, 2020. doi: 10.12998/wjcc.v8.i2.264
Malignant tumors associated with Peutz-Jeghers syndrome: Five cases from a single surgical unit
Zhi Zheng, Rui Xu, Jie Yin, Jun Cai, Guang-Yong Chen, Jun Zhang, Zhong-Tao Zhang
Zhi Zheng, Rui Xu, Jie Yin, Jun Cai, Guang-Yong Chen, Jun Zhang, Zhong-Tao Zhang, Department of General Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China
Author contributions: Zheng Z and Xu R contributed equally to this work; Zheng Z and Xu R carried out the studies, participated in collecting the data, and drafted the manuscript; Yin J and Cai J performed the statistical analysis and participated in its design; Zhang J, Chen GY, and Zhang ZT helped to draft the manuscript; all authors read and approved the final manuscript.
Supported by the Beijing Health System of High Level Health Technical Personal Training Project, No. 2013-3-067; and Beijing Municipal Science and Technology Commission, No. D171100006517003.
Institutional review board statement: This study was approved by the Ethics Committee of Beijing Friendship Hospital, Capital Medical University.
Informed consent statement: All participants provided written informed consent.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
Data sharing statement: The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Jun Zhang, MD, Professor, Department of General Surgery, Beijing Friendship Hospital, Capital Medical University, 95 Yongan Road, Xicheng District, Beijing 100050, China. zhangjun5986@ccmu.edu.cn
Received: November 11, 2019
Peer-review started: November 11, 2019
First decision: November 22, 2019
Revised: December 15, 2019
Accepted: January 1, 2020
Article in press: January 1, 2020
Published online: January 26, 2020
Processing time: 67 Days and 0.4 Hours
ARTICLE HIGHLIGHTS
Research background

Peutz-Jeghers syndrome (PJS) is a rare inherited disease, which can result in gastrointestinal and other organs malignant tumors. However, we do not have consistent diagnosis, treatment, and follow-up plans.

Research motivation

The research motivation is that many scholars do not realize the risk of PJS associated malignancy. Meanwhile, there are also disputes about diagnosing and treating for PJS.

Research objectives

Through evaluating the clinical symptoms, diagnosis, and treatment, we aimed to present our methods for treating malignant change secondary to PJS.

Research methods

We reviewed 46 PJS patients from 2014 to 2017. Among these patients, five developed malignant tumors. Hence, we collected their clinical, imaging, and pathological data for further analysis. In addition, we followed these five patients after they were discharged from hospital.

Research results

The PJS patients were first diagnosed with malignant tumor at about 43.6 years old. Among them, three patients received operations and pathology confirmed adenocarcinoma. One patient underwent genetic testing, which showed an STK11 mutation. Three patients received XELOX chemotherapy, while one patient die from tumor progression after 6 months. The other two patients received endoscopic resection; postoperative pathology confirmed high grade intraepithelial neoplasia. The surviving patients had no recurrence.

Research conclusions

If PJS patients are suspected to have secondary malignant changes, endoscopy and imageological examination are often needed to evaluate the tumor. For patients suited for endoscopic resection, we can perform the ESD. Otherwise, radical operation should be considered. After surgery, we need to conduct pathological examination and gene detection. For advanced tumor, patients need to receive adjuvant chemotherapy. Meanwhile, we also need to pay attention to the prevention in PJS patients.

Research perspectives

PJS patients do not always have typical symptoms. Compared with common people, they have higher tumor susceptibility. Therefore, they need to regularly check-up the body and take measures to prevent associated tumor complications and improve prognosis.