Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Feb 6, 2020; 8(3): 577-586
Published online Feb 6, 2020. doi: 10.12998/wjcc.v8.i3.577
Peutz-Jeghers syndrome with mesenteric fibromatosis: A case report and review of literature
Huai-Jie Cai, Han Wang, Nan Cao, Wei Wang, Xi-Xi Sun, Bin Huang
Huai-Jie Cai, Han Wang, Nan Cao, Wei Wang, Xi-Xi Sun, The Fourth Clinical Medicine College, Zhejiang Chinese Medical University, Hangzhou 310053, Zhejiang Province, China
Bin Huang, Department of Ultrasound, Zhejiang Hospital, Hangzhou 310013, Zhejiang Province, China
Author contributions: Cai HJ participated in the design of the report, analyzed the data, and wrote the paper; Wang W and Sun XX collected the medical imaging materials; Wang H, Cao N, and Huang B designed the report and performed the preliminary revision of the manuscript.
Informed consent statement: Consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
CARE Checklist (2016) statement: The manuscript was prepared and revised according to the CARE Checklist (2016).
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: Bin Huang, MB, Chief Physician, Department of Ultrasound, Zhejiang Hospital, No.1229 Gudun Road, Hangzhou 310013, Zhejiang Province, China. hb2k@163.com
Received: October 12, 2019
Peer-review started: October 12, 2019
First decision: December 23, 2019
Revised: January 7, 2020
Accepted: January 11, 2020
Article in press: January 11, 2020
Published online: February 6, 2020
Processing time: 116 Days and 22.5 Hours
Abstract
BACKGROUND

Peutz-Jeghers syndrome (PJS) and mesenteric fibromatosis (MF) are rare diseases, and PJS accompanying MF has not been previously reported. Here, we report a case of a 36-year-old man with both PJS and MF, who underwent total colectomy and MF surgical excision without regular follow-up. Two years later, he sought treatment for recurrent acute abdominal pain. Emergency computed tomography showed multiple soft tissue masses in the abdominal and pelvic cavity, and adhesions in the small bowel and peritoneum. Partial intestinal resection and excision of the recurrent MF were performed to relieve the symptoms.

CASE SUMMARY

A 36-year-old male patient underwent total colectomy for PJS with MF. No regular reexamination was performed after the operation. Two years later, due to intestinal obstruction caused by MF enveloping part of the small intestine and peritoneum, the patient came to our hospital for treatment. Extensive recurrence was observed in the abdomen and pelvic cavity. The MF had invaded the small intestine and could not be relieved intraoperatively. Finally, partial bowel resection, proximal stoma, and intravenous nutrition were performed to maintain life.

CONCLUSION

Regular detection is the primary way to prevent deterioration from PJS. Although MF is a benign tumor, it has characteristics of invasive growth and ready recurrence. Therefore, close follow-up of both the history of MF and gastrointestinal surgery are advisable. Early detection and early treatment are the main means of improving patient prognosis.

Keywords: Peutz-Jeghers syndrome; Mesenteric fibromatosis; Recurrence; Regular follow-up; Case report

Core tip: Peutz-Jeghers syndrome with mesenteric fibromatosis is extremely rare and has not been previously reported. Resection of the diseased bowel and mesenteric fibromatosis may be the best therapy. Regular review and timely intervention may improve patient prognosis. This paper analyzed the characteristics of Peutz-Jeghers syndrome and mesenteric fibromatosis in detail, and summarized this case, with the aim of providing a reference for future clinical diagnosis and treatment.