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Hepatic epithelioid angiomyolipoma: magnetic resonance imaging characteristics

Abdominal Radiology volume 48, pages 913–924 (2023)Cite this article

Abstract

Purpose

The aim of the study was to analyze MR imaging features of hepatic epithelioid angiomyolipoma (HEAML).

Methods

This study included 113 patients with 122 pathologically confirmed HEAML who underwent enhanced MRI scanning before partial hepatectomy. MR images were retrospectively reviewed and correlated with pathological findings.

Results

The mean age of 113 patients was 48.12 ± 11.77 years old, and the male to female ratio was 1:3.35 with 87 females (76.99%). 107 (94.69%) patients presented as single tumor, and 96 patients (84.96%) were asymptomatic. 122 HEAML lesions were diagnosed pathologically in 113 patients, with the average of 4.47 ± 3.26 cm. 109 lesions (89.34%) and 108 lesions (88.52%) showed regular and well-defined boundary. On T1WI, 121 lesions (99.18%) mainly presented hypointensity. On T2WI, 118 lesions (96.72%) and 109 lesions (89.34%) mainly showed hyperintensity and heterogeneous signals. Most of the lesions (97.46%) showed hyperintensity on DWI. 118 lesions (96.72%) manifested as severe hyperenhancement and 106 lesions (86.89%) showed heterogeneity during the arterial phase. As for the lesion enhancement pattern, 73 lesions (59.84%) presented as persistent enhancement, 37 lesions (30.33%) as wash out, 8 lesions (6.56%) as degressive enhancement, and 4 lesions (3.28%) as poor blood supply. Additionally, 96 lesions (78.69%) with intra-tumor vessels and 85 lesions (69.67%) with outer rim were confirmed during the delayed phase.

Conclusion

At enhanced MRI, hypointensity on T1WI, hyperintensity and heterogeneous signals on T2WI, hyperintensity on DWI, little or no fat component, heterogenous hyperenhancement, persistent enhancement, intra-tumor vessels, and outer rim would be helpful to diagnose HEAML.

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Abbreviations

AML:

Angiomyolipoma

EAML:

Epithelioid angiomyolipoma

HEAML:

Hepatic epithelioid angiomyolipoma

PEComas:

Perivascular epithelioid cell tumors

HMB-45:

Human melanoma black 45

SMA:

Smooth muscle actin

TSC:

Tuberous sclerosis syndrome

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Acknowledgements

I would like to thank Dr. Xu Pengju for providing the means and encouragement to write and publish this paper. Dr. Xu Pengju also made a host of important suggestions included in this study. Another special shoutout goes to Dr. Shao Xiali, who contributed to the methods in this study aimed at explaining the differences between hemorrhage, necrosis, cystic degeneration, and microscopic fat. While my understanding of the thesis writing process has undeniably benefitted from my work advising medical students, I owe particular thanks to Wang Zhe and Ma Jing who inspired my writing. Finally, I thank Cao Yiran, not only for her continuous moral and spiritual support to this text, but also for the example she sets to PhD students.

Funding

This work was supported by the Shanghai Municipal Key Clinical Specialty (shslczdzk03202).

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Authors and Affiliations

  1. Department of Radiology, Shanghai Geriatric Medical Center, Zhongshan Hospital, Fudan University, Shanghai, China

    Li Junhao, Guo Jiajun, Gao Shanshan, Zeng Mengsu & Wang Mingliang

  2. Department of Interventional Radiology, Zhongshan Hospital, Fudan University, Shanghai, China

    Li Junhao & Li Jianke

  3. Department of Radiology, Taizhou Clinical Medical School of Nanjing Medical University (Taizhou People’s Hospital), Nanjing, Jiangsu Province, China

    Zhang Hongxia

  4. Department of Plastic and Reconstructive Surgery, Zhongshan Hospital, Fudan University, Shanghai, China

    Ikram Ahmad

  5. Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai, China

    Chen Lingli & Ji Yuan

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  1. Li Junhao
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Correspondence to Wang Mingliang.

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The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Ethical approval

The study was performed in accordance with the ethical standards as laid down in the 1964.

Declaration of Helsinki and its later amendments or comparable ethical standards. Zhongshan hospital review board approved this retrospective study and waived the requirement for informed consent.

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Junhao, L., Hongxia, Z., Jiajun, G. et al. Hepatic epithelioid angiomyolipoma: magnetic resonance imaging characteristics. Abdom Radiol 48, 913–924 (2023). https://doi.org/10.1007/s00261-023-03818-z

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