PMC5615861_01
Imaging of a hepatic perivascular epithelioid cell tumor. (a) Contrast-enhanced arterial phase of computed tomography reveals a poorly defined mass with marked enhancement and inferior vena cava invasion. (b) Coronal reconstruction of venous phase contrast-enhanced computed tomography shows relatively washout of mass with unclear borders. Tumor thrombus in inferior vena cava is also seen clearly in coronal multidetector computed tomography image.
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F1
MDCT examination reveals a liver mass with 52 mm x 43 mm diameters located in segment VIII and caudate lobe of the liver [Figure 1].
Axial T1-weighted (a), T2-weighted (b), and fat-suppressed T2-weighted (c) magnetic resonance imaging images demonstrate T1-isointense, T2 and fat-suppressed T2-hyperintense mass component of liver perivascular epithelioid cell tumor in vena cava inferior.
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F2
T1-weighted images and heterogeneously hyperintense on T2-weighted images relative to the liver parenchyma [Figure 2]. There was not seen any fat component in the lesion.
Postgadolinium fat-suppressed axial arterial phase (a), and coronal venous phase images demonstrate a large liver perivascular epithelioid cell tumor that marked heterogeneous enhancement in arterial phase, and washout in venous phase with prominent invasion of inferior vena cava. Tumor thrombus is markedly seen in late venous phase (b).
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F3
Inferior vena cava (IVC) invasion with tumor thrombus into the lumen is also demonstrated, especially coronal Gd-enhanced images [Figure 3].
Diffusion-weighted image of perivascular epithelioid cell tumor (black arrow). Marked diffusion restriction of malign perivascular epithelioid cell tumor is seen as hypointense signal change on apparent diffusion coefficient map.
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F4
There was also marked diffusion restriction of lesion on diffusion-weighted images as totally hypointense signal in apparent diffusion coefficient maps [Figure 4].
Histopathologic findings of malign perivascular epithelioid cell tumor; photomicrograph (H and E, x400) shows specimen is composed of nests of large epithelioid cells with clear-to-granular eosinophilic cytoplasm. There is also seen severe nuclear atypia that indicates of malign tumor.
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F5
The patient underwent biopsy of the liver mass, and pathology revealed a hepatic perivascular epithelioid cell tumor or "PEComa." Histologic findings were a mixture of large epithelioid cells and clear-to-eosinophilic granular cytoplasm [Figure 5].