Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Oct 26, 2021; 9(30): 9114-9121
Published online Oct 26, 2021. doi: 10.12998/wjcc.v9.i30.9114
Spontaneous rupture of a mucinous cystic neoplasm of the liver resulting in a huge biloma in a pregnant woman: A case report
Artur Kośnik, Anna Stadnik, Benedykt Szczepankiewicz, Waldemar Patkowski, Maciej Wójcicki
Artur Kośnik, Maciej Wójcicki, Liver and Internal Medicine Unit, Medical University of Warsaw, Warsaw 02-097, Poland
Anna Stadnik, Department of Radiology, Medical University of Warsaw, Warsaw 02-097, Poland
Benedykt Szczepankiewicz, Department of Pathology, Medical University of Warsaw, Warsaw 02-097, Poland
Waldemar Patkowski, Department of General, Transplant, and Liver Surgery, Medical University of Warsaw, Warsaw 02-097, Poland
Author contributions: Kośnik A designed and wrote the paper and reviewed the literature; Stadnik A designed and wrote the paper, performed imaging analysis; Szczepankiewicz B reviewed the literature and performed pathological analysis; Patkowski W performed surgery and conceptualised the paper; Wójcicki M wrote and approved the final manuscript; All authors issued final approval for the version to be submitted.
Informed consent statement: Informed written 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 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: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Maciej Wójcicki, MD, PhD, Professor, Surgeon, Liver and Internal Medicine Unit, Medical University of Warsaw, Banacha 1a, Warsaw 02-097, Poland. maciej.wojcicki@uckwum.pl
Received: March 14, 2021
Peer-review started: March 14, 2021
First decision: July 14, 2021
Revised: July 26, 2021
Accepted: September 16, 2021
Article in press: September 16, 2021
Published online: October 26, 2021
Processing time: 220 Days and 14.9 Hours
Abstract
BACKGROUND

Mucinous cystic neoplasm of the liver (MCN-L) and intraductal papillary neoplasm of the bile duct (IPN-B) are two different types of mucin-producing bile duct tumour that may complicate the course of pregnancy. To the best of our knowledge, we describe herein the first case of MCN-L with spontaneous rupture during pregnancy necessitating complex surgical treatment.

CASE SUMMARY

A 24-year-old woman was initially admitted to another hospital in October 2018 with signs of jaundice (serum bilirubin level 12 mg/dL) and upper abdominal pain radiating to the left shoulder. Initial magnetic resonance imaging (MRI) of the abdominal cavity revealed a multilocular cystic tumour of the liver hilum (37 mm × 40 mm in diameter) located between segments 3 and 4 of the left liver lobe. Six weeks later (December 2018), the patient was found to be 12 wk pregnant and was referred to our institution for further diagnostics and treatment. At admission, a soft, palpable, and tender mass in the left upper abdomen was found. It was determined via MRI (with no intravenous contrast in view of the first-trimester pregnancy) to be a large collection of fluid (19 cm × 17 cm × 10 cm) located close to the liver hilum and below the left liver lobe. The patient did not undergo any diagnostic or therapeutic procedures nor did they have any abdominal trauma in the preceding weeks. The fluid collection proved to be of biliary origin following percutaneous drainage. Therefore, we concluded this was a spontaneous rupture of an MCN-L with the formation of a biloma. The MRI study also revealed the previously found cystic tumour of the liver hilum communicating with the left hepatic duct, which, together with left hepatic duct dilatation, suggested the diagnosis of IPN-B. The follow-up MRI with intravenous gadolinium contrast performed in the second trimester of pregnancy (week 14) showed, in turn, some features of MCN-L, including enhancement of the internal septations within the cystic liver mass. A precise preoperative differential diagnosis between IPN-B and MCN-L was therefore not possible. The patient was submitted to surgery in the second trimester of pregnancy (week 18). Surgery included a cholecystectomy, left hepatectomy, and concomitant resection of the extrahepatic bile ducts followed by anastomosis of the right hepatic duct with the Roux limb of the jejunum. The post-operative period was uneventful and the patient was discharged 8 days after surgery. The histopathological examination of the resected specimen revealed a final diagnosis of MCN-L with low-grade dysplasia and epithelium surrounded by ovarian-type stromal tissue. The patient delivered a healthy baby girl and both remain well at present, after 2 years of follow-up since surgery.

CONCLUSION

The differential diagnosis and management of MCN-L and IPN-B may be very challenging, particularly in the setting of pregnancy. When indications for surgery are obvious, the final diagnosis is based on histopathological examination, with ovarian-type stroma being pathognomonic for MCN-L. We believe that the growth of this subepithelial stroma secondary to the high levels of sex hormones produced during pregnancy might have been the main causative factor leading to the tumour rupture with the formation of a biloma in our patient.

Keywords: Biliary tract neoplasms; Liver neoplasms; Hepatectomy; Anastomosis, Roux-en-Y; Pregnancy; Case report

Core Tip: The differential diagnosis and management of biliary cystic neoplasms of the liver may be very challenging, especially in the setting of pregnancy. To the best of our knowledge, we describe herein the first case of a mucinous cystic neoplasm of the liver (MCN-L) with spontaneous rupture leading to the formation of a huge biloma in a pregnant woman. This was treated with percutaneous drainage of the biloma, followed by MCN-L removal via left hepatectomy with extrahepatic bile duct resection and anastomosis of the right hepatic duct with the Roux limb of the jejunum in the second trimester of pregnancy. We believe that the growth of this subepithelial stroma due to the high levels of sex hormones during pregnancy might have been the main causative factor that led to the tumour rupture and the formation of a biloma in our patient.