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Case Reports
. 2021 Apr;28(3):217-221.
doi: 10.1159/000509192. Epub 2020 Oct 5.

A Rare Case of PEComa of the Liver

Abigail Attard  1 , Naomi Piscopo  1 , John Schembri  1 , Tiffany Buhagiar  2 , Kelvin Cortis  3 , Pierre Ellul  1
Affiliations
Case Reports

A Rare Case of PEComa of the Liver

Abigail Attard et al. GE Port J Gastroenterol. 2021 Apr.
No abstract available

Keywords: Differential diagnosis; Hepatic angiomyolipoma; PEComa.

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Conflict of interest statement

The authors have o conflicts of interest to disclose.

Figures

Fig. 1
Fig. 1
CT image of PEComa in segment VI of the liver.
Fig. 2
Fig. 2
Coronal T2-weighted image showing a well-defined heterogeneously hyperintense lesion in the medial aspect of the right liver lobe.
Fig. 3
Fig. 3
Post-contrast 3D. T1-weighted axial fat-suppressed images showing a perfusion anomaly surrounding this lesion. This lesion remains hypovascular on arterial phase (a) and portal/late venous phase (b, c) imaging. No uptake of hepatobiliary contrast media is seen on 20-min delayed hepatobiliary phase imaging (d).
Fig. 4
Fig. 4
Diffusion-weighted imaging. Top image: b = 50 (a). Middle image: b = 800 (b). Bottom image: ADC map (c). The lesion is evident on b = 50 and slightly brighter on b = 800. This is compatible with mild restriction of diffusion, as confirmed on the ADC map.
Fig. 5
Fig. 5
Pathological findings of the PEComa. a H&E. Low-power view (×100). b H&E. High-power view (×400). A neoplastic proliferation composed of spindle and polygonal cells with clear to pale eosinophilic cytoplasm, arranged in a haphazard pattern around small vascular spaces.
Fig. 6
Fig. 6
Immunohistochemistry staining of PEComa. The neoplastic cells express Melan-A (a), HMB-45 (b), vimentin (c), and SMA (d).
Fig. 7
Fig. 7
PET-CT showing increased uptake in segment VI of liver.
See this image and copyright information in PMC

References

    1. Du S, Li Y, Mao Y, Sang X, Lu X, Wang W, et al. Diagnosis and treatment of hepatic angiomyolipoma. Hepatobiliary Surg Nutr. 2012 Dec;1((1)):19–24. - PMC - PubMed
    1. Horvat N, Nikolovski I, Long N, Gerst S, Zheng J, Pak LM, et al. Imaging features of hepatocellular carcinoma compared to intrahepatic cholangiocarcinoma and combined tumor on MRI using liver imaging and data system (LI-RADS) version 2014. Abdom Radiol (NY) 2018 Jan;43((1)):169–78. - PMC - PubMed
    1. Kubo H, Yamazaki H, Okada T, Takahashi Y, Nishi Y, Yokomori H. Primary hepatic angiomyolipoma: immunohistochemistry and electron microscopic observations: a case report. J Med Case Reports. 2017 Mar;11((1)):76. - PMC - PubMed
    1. Mann SA, Saxena R. Differential diagnosis of epithelioid and clear cell tumors in the liver. Semin Diagn Pathol. 2017 Mar;34((2)):183–91. - PubMed
    1. Yang X, Li A, Wu M. Hepatic angiomyolipoma: clinical, imaging and pathological features in 178 cases. Med Oncol. 2013 Mar;30((1)):416. - PubMed

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