Hepatic epithelioid hemangioendothelioma: Dilemma and challenges in the preoperative diagnosis

Abstract

Hepatic epithelioid hemangioendothelioma (HEHE) is a rare category of vascular tumor with uncertain malignant potential. It commonly presents nonspecific and variable clinical manifestations, ranging from asymptomatic to hepatic failure. In addition, laboratory measurements and imaging features also lack specificity in the diagnosis of HEHE. The aim of the present study is to highlight the dilemma and challenges in the preoperative diagnosis of HEHE, and to enhance awareness of the range of hepatobiliary surgery available in patients with multiple hepatic nodular lesions on imaging. In these patients, HEHE should at least be considered in the differential diagnosis.

Keywords: Challenges; Diagnosis; Dilemma; Hepatic epithelioid hemangioendothelioma; Vascular tumors.

Figure 1

Abdominal contrast-enhanced computerized tomography findings. Abdominal contrast-enhanced computerized tomography (CT) revealed multiple low density nodular lesions scattered in the liver parenchyma, involving the right lobe and left medial segment, with inhomogeneous enhancement.

Figure 2

Contrast-enhanced ultrasonography findings. Contrast-enhanced ultrasonography indicated multiple hypoechoic nodules with peripheral hyper-enhancement during the arterial phase and hypo-enhancement during the portal phase.

Figure 3

PET-CT findings. PET-CT showed that the hepatic masses had low glycometabolism.

Figure 4

Pathological findings. Pathological investigations identified hepatic epithelioid hemangioendothelioma: A: Hematoxylin-eosin staining revealed abnormal hyperplasia of fibrous tissue combined with vessel-like formations, with scattered neoplastic epithelioid cells; B: Immunohistochemistry showed that the tumor was positive for CD34; C: Immunohistochemistry showed that the tumor was positive for CD31.