Hepatic epithelioid hemangioendothelioma after thirteen years' follow-up: A case report and review of literature

Abstract

Background: Hepatic epithelioid hemangioendothelioma (EHE) is a rare vascular endothelial cell tumor of the liver, consisting of epithelioid and histiocyte-like vascular endothelial cells in mucus or a fibrotic matrix. Immunohistochemistry is usually positive for vascular markers, such as factor VIII-related antigen, CD31, and CD34. Hepatic EHE can have a varied clinical course; treatment includes liver transplantation, liver resection, chemotherapy, and radiation therapy.

Case summary: A 46-year-old woman with abdominal discomfort and elevated serum carcinoembryonic antigen was found to have multiple low-density lesions in the liver and lung on computed tomography (CT) evaluation. An ultrasound-guided fine needle aspiration biopsy revealed a fibrous stroma with dendritic cells, containing intracellular vacuoles. Immunohistochemical staining found that the tumor cells were positive for CD34, CD31, and factor VIII-related antigen. The patient received four courses of combined chemotherapy and was followed-up for 13 years, at which time the patient was in stable condition without disease progression and a confined neoplasm, as evidenced by CT scans.

Conclusion: The histology and immunohistochemical characteristics of hepatic EHE are well described. Chemotherapy may be effective in patients with extrahepatic lesions.

Keywords: Antineoplastic combined chemotherapy protocols; Case report; Epithelioid hemangioendothelioma; Immunohistochemistry; Liver neoplasm; Treatment.

Figure 1

Contrast-enhanced computed tomography. Multiple low-density lesions (black arrow) with mild-moderate peripheral enhancement are seen in the right lobe of the liver.

Figure 2

Magnetic resonance weighted image. A: T1-weighted image shows low signal ovoid lesions in the right lobe of liver; B: The lesions have a heterogeneous high signal in the T2-weighted image; C: The largest lesion in the right lobe is mildly heterogeneous with peripheral enhancement and an arterial contrast enhancement pattern; D: Peripheral enhancement of lesions is increased in spots visible in a venous contrast enhancement pattern; E: Lesions show diffusion restriction on a diffusion-weighted image.

Figure 3

Liver biopsy. A: Mildly heteromorphic spindle cells (dendritic cells) with interdigitating processes (hematoxylin and eosin, 200 ×); B: Intracellular vascular lumina containing a red blood cell (black arrow) (hematoxylin and eosin, 400 ×).

Figure 4

Histopathology, immunostaining of tumor cells. A: Anti-CD31+ (200 ×); B: Anti-CD34+ (200 ×); C: Anti-factor VIII-related antigen+ (200 ×); D: Anti-CK− (Pan) (200 ×).

Figure 5

Contrast-enhanced follow-up computed tomography scans. A: After 4 years, diameter of the low-density lesion was 16 mm; B: After 12 years, diameter of the low-density lesion was 17 mm.