Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Aug 16, 2022; 10(23): 8375-8383
Published online Aug 16, 2022. doi: 10.12998/wjcc.v10.i23.8375
Infant biliary cirrhosis secondary to a biliary inflammatory myofibroblastic tumor: A case report and review of literature
Yuan Huang, Sai-Nan Shu, Hua Zhou, Ling-Ling Liu, Feng Fang
Yuan Huang, Sai-Nan Shu, Hua Zhou, Ling-Ling Liu, Feng Fang, Department of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei Province, China
Author contributions: Fang F, Shu SN and Zhou H conceptualized and designed the study; Huang Y collected the clinical and scientific findings and wrote the manuscript; Liu LL reviewed, and revised the manuscript; all authors discussed the results and contributed to the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images
Conflict-of-interest statement: All authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and 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 NonCommercial (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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Feng Fang, PhD, Professor, Department of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, No. 1095 Jiefang Avenue, Qiaokou District, Wuhan 430030, Hubei Province, China. ffang56@163.com
Received: March 30, 2022
Peer-review started: March 30, 2022
First decision: June 16, 2022
Revised: June 25, 2022
Accepted: July 6, 2022
Article in press: July 6, 2022
Published online: August 16, 2022
Processing time: 123 Days and 18.1 Hours
Abstract
BACKGROUND

A biliary inflammatory myofibroblastic tumor (IMT) is a rare type of mesenchymoma that, although it has a broad age spectrum, usually occurs in adults. Diagnosis is difficult because biliary IMTs often exhibit nonspecific clinical symptoms and imaging features, resulting in delayed or inappropriate treatment. Although most IMTs are benign, some show malignant properties such as infiltration, recurrence, and metastasis.

CASE SUMMARY

Here, we retrospectively describe a 10-month-old infant who was admitted to our hospital due to stubborn jaundice. The patient responded poorly to routine medical treatment and his clinical manifestations and laboratory tests lacked specificity, so we turned to repeated ultrasound scans and other imaging examinations. As both hepatosplenic ultrasonography and diffusion-weighted magnetic resonance imaging demonstrated a space-occupying lesion, an exploratory laparotomy was performed. The final diagnosis made over two mo after the disease onset was infant biliary cirrhosis caused by a biliary IMT, which partially infiltrated into the liver. This infant is the youngest case of biliary IMTs that has been reported till now. The patient underwent an incomplete resection of the mass and Kasai Portoenterostomy. However, because of cirrhosis, he also received a paternal liver transplant. Since some IMTs show malignant properties, we proceeded with a three-year of follow-up; however, no recurrence or metastasis has been noted.

CONCLUSION

Neoplastic disease such as IMTs should be considered when routine medical treatment of obstructive jaundice is not successful. Observation of dynamic imaging changes is helpful for diagnosis. Periodic follow-up is necessary for IMTs.

Keywords: Inflammatory myofibroblastic tumor; Inflammatory pseudotumor; Obstructive jaundice; Biliary cirrhosis; mesenchymoma; Case report

Core Tip: Biliary inflammatory myofibroblastic tumor (IMT) is a rare type of mesenchymoma. Diagnosis is difficult because IMTs often exhibit nonspecific clinical symptoms. We describe a biliary IMT in a 10-month-old male patient who manifested as stubborn obstructive jaundice. This is the youngest case of biliary IMTs that have been reported till now. This case highlights that neoplastic disease should be considered when routine medical treatment of obstructive jaundice is not successful. Observation of dynamic imaging changes is helpful to find out occupying lesions. Timely diagnosis and treatment are crucial and periodic follow-up is necessary due to the malignant properties of IMTs.