Three cases of hepatic epithelioid hemangioendothelioma evaluated using conventional and contrast-enhanced ultrasound: Case reports

Abstract

Hepatic epithelioid hemangioendothelioma (HEHE) is a very rare vascular endothelial cell tumor, which lacks typical clinical manifestations and specificity of imaging features. Whether the background of fatty liver and the difference in Contrast enhanced ultrasound (CEUS) characteristics between large and small lesions has not been well defined. In this case reports, we described the ultrasound image features of three patients with HEHE. These three patients with HEHE have certain similar characteristics of conventional ultrasound and CEUS. CEUS imaging features include large nodules show earlier perfusion than liver parenchyma, with rim-enhancement, nonenhancing regions in the center, while small nodules show earlier perfusion than liver parenchyma, with hyperenhancement. All nodules show faster washout than hepatic parenchyma, showing heterogeneous hypoenhancement, and more washout lesions can be found in the PVP and LP. Conventional ultrasound and CEUS not only help to improve the diagnostic confidence of HEHE of rare liver tumors, but also can guide the biopsy area, making it easier to make accurate pathological diagnosis.

Keywords: contrast enhanced ultrasound; focal liver lesions; hemangioendothelioma; hepatic neoplasm; rare tumors.

FIGURE 1

CASE 1 (A) The hypoechoic lesions (4.8 cm × 4.3 cm) was visible under the background of fatty liver, with vague boundary and irregular shape; (B) Color Doppler showed no obvious blood flow signal within the lesions. (C–E) CEUS demonstrating a rim APHE (C) with early washout (D–E). (F–G) The smaller nodules showed APHE (F) with early washout in the PVP (G). (H) HE×4. Irregular vascular‐like lacunae were found in the liver tissue, some of which were lined with flattened to cubic epithelium, and some were single‐celled lumens. Residue hepatic cord (cytoplasmic red) at upper right

FIGURE 2

CASE 2 (A) Under the background of fatty liver, a hypoechoic nodule (4.3 cm × 3.9 cm) were seen, with vague boundaries and irregular shapes. (B) Color Doppler imaging shows no obvious blood flow signal in the hypoechoic nodules, with peripheral branching vessels. (C–E) CEUS indicated that the largest nodule demonstrates rim APHE with early washout in PVP and LP. (D–E) More washout lesions (red arrow) were found during the PVP and LP. (F) CEUS demonstrated that some hypoechoic lesions showed isoenhancing in AP, PVP and LP. (G–H) HE×10. Short spindle cells and small dilated or irregular lacunae were seen in the fibrous background (G) and myxoid background (H). Red cells were seen in the lower left lacunae of Figure G

FIGURE 3

CASE 3 (A, E) The liver parenchyma presents a fatty liver background, with multiple hypoechoic lesions, some of which with vague boundaries and irregular shapes. (B) CEUS indicated that the largest nodule demonstrates rim APHE and early washout in PVP and LP. (F–G) The two small nodules lesion of similar size demonstrate homogeneous or heterogeneous APHE, and early washout in PVP and LP. (C) The structure of hepatic lobule was destroyed, irregular vessels and single‐cell lumen were infiltrated, with hepatic cord blurring and sinus disappearance (HE×40). (D) Immunohistochemical staining showed CD34 membrane positive, showing disordered vascular distribution. (H) Ki‐67 was about 2%

FIGURE 4

Pattern A: Rim APHE with early washout. Pattern B: Rim APHE with early washout and more washout lesions were appeared during the PVP and LP. Pattern C: APHE with early washout