CASE 16767 Published on 10.08.2020

Hepatic epithelioid hemangioendothelioma

Section

Abdominal imaging

Case Type

Clinical Cases

Authors

Tikhonova V.S., Gruzdev I.S., Kondratev E.V., Kashtanova N.Yu., Karmazanovsky G.G.

A.V. Vishnevsky National Medical Research Center of Surgery, 117997 Mosсow, Russiа, Bol. Serpuhovskаyа str., 27.

Patient

38 years, female

Categories
Area of Interest Abdomen, Liver ; Imaging Technique CT, MR
Clinical History

A 38-year-old woman with complaints of heaviness in the right hypochondrium over the course of 4 months. During the past six months decreased appetite and weight loss (up to 5 kg). A clinical blood test revealed hypochromic anaemia and thrombocytopenia.

Imaging Findings

MDCT revealed multiple hypoattenuating lesions in both hepatic lobes, which tend to form larger confluent hypoattenuating regions in a peripheral or subcapsular distribution, with a halo or target pattern of enhancement in larger lesions. Subcapsular lesions show a capsular retraction. Right hepatic vein is narrow and terminates at the edge of one of these lesions (lollipop sign). (Fig. 1,2). Abdomen MRI with contrast was performed to receive additional data about lesion structure (Figs. 3-5). MRI revealed monomorphic hypovascular target-like sections of an altered signal in both hepatic lobes. Subcapsular lesions show a capsular retraction. The structure of the lesions is heterogeneous due to the presence of central high-MR signal at T2-WI and low-MRI signal at T1-WI with less signal intensity on periphery (Fig. 3). Also, peripheral part of the lesions shows diffusion restriction (Fig. 4). CE MRI shows the ring-shaped enhancement of lesions persistent in the delayed phase (Fig. 5).

Discussion

Hepatic epithelioid haemangioendothelioma (HEH) is a rare vascular tumour of endothelial origin with low-to-intermediate grade malignancy [1]. HEH most often manifests itself as a multifocal nodular lesion, less often as solitary liver lesion. Previous studies have revealed that HEH tends to coalesce into diffuse lesions in the late stages of the disease [2]. The clinical manifestations of HEH usually are nonspecific and include right-upper quadrant pain or weight loss, but some patients may present with liver failure, Budd–Chiari syndrome, or portal hypertension; other patients may be asymptomatic.

Diagnosis of these tumours on the basis of clinical presentation is difficult because of their nonspecific signs and symptoms. They can be difficult to diagnose on the basis of biopsy results. The main contribution to differential diagnosis is made by non-invasive imaging modalities. First you have to differentiate HEH with metastatic hepatic disease, which does not have a tendency to exclusively peripheral localization, but other characteristics such as ring enhancement, peripheral diffusion restriction are the same for metastasis and HEH.

Ultrasound diagnostics is the first line method of diagnosis. Usually seen as hepatic lesions that are predominantly hypoechoic; however, hepatic lesions can also have mixed echotexture or be predominantly hyperechoic. Methods of choice are CT and MRI with contrast enhancement because they identify typical characters of  dynamic contrast enhancement for HEH. The lesions demonstrate three distinct ground of accumulation of the contrast agent [3].  In our case lesions demonstrate rim-like arterial enhancement with wash-out in the portal venous and late venous phase (PVLP). Wash-out during the PVLP is highly suspicious for malignancy [3].

The patient underwent a PET-CT. No distant metastases were found. Based on the results of biopsy and immunohistochemistry the nodular variant of epithelial hemangioendothelioma of the liver was confirmed that correlation with imaging feathers. The patient was sent to a specialized medical facility for liver transplantation.

Because of nonspecific clinical manifestations and a prolonged clinical course of the disease, the age of the patient at the time that HEH is detected may vary widely. The tumor does not respond to chemotherapy and/or radiotherapy. Complete surgical excision is recommended for resectable lesions. Orthotopic liver transplantation remains the only treatment option in selected patients with extensive liver involvement or with rapidly progressive liver failure even in patients with known extrahepatic involvement [4,5]. More familiarity with the imaging findings may allow recognition of this tumor at earlier stages.

Differential Diagnosis List
Hepatic epithelioid hemangioendothelioma
Multiple hepatic hemangiomas
Colorectal cancer metastases
Angiomyolipoma (AML) of the liver
Liver adenomas
Final Diagnosis
Hepatic epithelioid hemangioendothelioma
Case information
URL: https://www.eurorad.org/case/16767
ISSN: 1563-4086
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