Case Report
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Apr 26, 2019; 7(8): 972-983
Published online Apr 26, 2019. doi: 10.12998/wjcc.v7.i8.972
Two case reports and literature review for hepatic epithelioid angiomyolipoma: Pitfall of misdiagnosis
Jia-Xi Mao, Fei Teng, Cong Liu, Hang Yuan, Ke-Yan Sun, You Zou, Jia-Yong Dong, Jun-Song Ji, Jun-Feng Dong, Hong Fu, Guo-Shan Ding, Wen-Yuan Guo
Jia-Xi Mao, Fei Teng, Cong Liu, Hang Yuan, Ke-Yan Sun, You Zou, Jia-Yong Dong, Jun-Song Ji, Jun-Feng Dong, Hong Fu, Guo-Shan Ding, Wen-Yuan Guo, Department of Liver Surgery and Organ Transplantation, Changzheng Hospital, Naval Medical University, Shanghai 200003, China
Author contributions: Mao JX, Teng F, and Liu C contributed equally to this work; Mao JX and Teng F contributed to the concept and design of the study; Mao JX, Teng F, and Liu C drafted the manuscript; Yuan H, Sun KY, Zou Y, and Dong JF contributed to treatment of patients; Dong JY and Ji JS were involved in the acquisition, analysis, and interpretation of clinical data; Fu H, Ding GS, and Guo WY performed analysis and interpretation of pathologic data and were responsible for critical revision of the manuscript; Guo WY and Ding GS are the guarantors; All authors read and approved the final manuscript.
Supported by the National Science Foundation of China under Grant numbers, No. 81702923; and Outstanding Postgraduate Seedling Cultivation Fund of Naval Medical University.
Informed consent statement: Written informed consents were obtained from the two patients.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2013), 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 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: Wen-Yuan Guo, MD, PhD, Associate Professor, Department of Liver Surgery and Organ Transplantation, Changzheng Hospital, Naval Medical University, 415 Fengyang Road, Huangpu District, Shanghai 200003, China. guowenyuan@smmu.edu.cn
Telephone: +86-21- 63276788 Fax: +86-21-63276788
Received: December 18, 2018
Peer-review started: December 19, 2018
First decision: January 18, 2019
Revised: February 10, 2019
Accepted: February 26, 2019
Article in press: February 26, 2019
Published online: April 26, 2019
Processing time: 129 Days and 23.7 Hours
Abstract
BACKGROUND

Hepatic epithelioid angiomyolipoma (HEAML) is a rare liver disease and is easily misdiagnosed. Enhanced recognition of HEAML is beneficial to the differential diagnosis of rare liver diseases.

CASE SUMMARY

We presented two cases of HEAML in Changzheng Hospital, Naval Medical University, and then collected and analyzed all reports about HEAML recorded in PubMed, MEDLINE, China Science Periodical Database, and VIP database from January 2000 to March 2018. A total of 409 cases of HEAML in 97 reports were collected, with a ratio of men to women of 1:4.84 and an age range from 12 years to 80 years (median 44 years). Among the patients with clinical symptoms mentioned, 61.93% (205/331) were asymptomatic, 34.74% (115/331) showed upper or right upper quadrant abdomen discomfort, while a few of them showed abdominal mass, gastrointestinal symptoms, low fever, or weight loss. The misdiagnosis rate of HEAML was as high as 40.34% (165/409) due to its nonspecific imaging findings. Most of the tumors were solitary and round in morphology, with clear boundaries. Ultrasound scan indicated low echo with internal nonuniformity and rich blood supply in most cases. Computer tomography/magnetic resonance imaging enhanced scan showed varied characteristics. The ratio of fast wash-in and fast wash-out, fast wash-in and slow wash-out, and delayed enhancement was roughly 4:5:1. A definite diagnosis of HEAML depended on the pathological findings of the epithelioid cells in lesions and the expression of human melanoma black 45, smooth muscle actin, melanoma antigen, and actin by immunohistochemical staining. HEAML had a relatively low malignant rate of 3.91%. However, surgical resection was the main treatment for HEAML, due to the difficulty diagnosing before operation.

CONCLUSION

HEAML is a rare and easily misdiagnosed disease, and it should be diagnosed carefully, taking into account clinical course, imaging, pathological ,and immunohistochemical findings.

Keywords: Hepatic epithelioid angiomyolipoma; Imaging; Pathology; Misdiagnosis; Potentially malignant; Case report

Core tip: Hepatic epithelioid angiomyolipoma (HEAML) is a rare and easily misdiagnosed disease, with a misdiagnosis rate as high as 40.34% and a relatively low malignant rate of 3.91%. In this work, we presented two cases of primary and secondary HEAML and analyzed the 409 cases of HEAML recorded in PubMed, MEDLINE, China Science Periodical Database, and VIP database from January 2000 to March 2018. This pooled analysis of HEAML, in view of clinical course, imaging, pathological and immunohistochemical findings, may provide a better understanding of HEAML.