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Hepatic Epithelioid Hemangioendothelioma: Sonographic, CT, and MR Imaging Appearances

¹ Department of Radiology, Abdominal Division, Vancouver General Hospital and University of British Columbia, 855 W. 12th Ave., B. C., Canada.
² Present address: Department of Radiology, Cheltenham General Hospital, Sandford Rd., Cheltenham, Gloucestershire GL53 7AN, United Kingdom.
³ Department of Pathology, Vancouver General Hospital and University of British Columbia, Vancouver, B. C., Canada.
⁴ Department of Surgery, Vancouver General Hospital and the University of British Columbia, Vancouver, B. C., Canada.

Received August 19, 2002; accepted after revision November 14, 2002.

 
Address correspondence to I. D. Lyburn.

Introduction

Top Introduction Imaging Features Extrahepatic Involvement Conclusion References

 
Hepatic epithelioid hemangioendothelioma is a rare tumor of vascular origin, first defined as a specific entity by Weiss and Enzinger [1] in 1982. Clinical manifestation is variable, usually with nonspecific symptoms such as right upper quadrant pain and weight loss. Some patients present with Budd-Chiari syndrome or liver failure; others present with incidental findings [2, 3]. Diagnosis may be difficult. Many published cases are initially misdiagnosed [1, 3].

Although tumor growth may be progressive and lead to hepatic failure, extrahepatic metastases, and death, the prognosis is considered more favorable than that of other hepatic malignancies. Hepatic epithelioid hemangioendothelioma is usually defined as a low-to intermediate-grade malignancy [2, 3]. Histologically, the tumors are composed of dendritic and epithelioid cells. Tumor cells with intracytoplasmic lumina, occasionally containing RBC, appear as signet ring–like structures (Fig. 1). The stroma is fibrous with myxohyaline areas. Immunohistochemically, tumors are positive for at least one endothelial marker (factor VIII-related antigen, CD34 or CD31, or both) [2, 3] and negative for epithelial markers (cytokeratin and carcinoembryonic antigen). It is important to distinguish this entity from adenocarcinoma and angiosarcoma, because long-term survival is possible. Treatment modalities include hepatic resection; orthotopic liver transplantation, even in cases with known metastases; radiotherapy; chemotherapy with Adriamycin ([doxorubicin hydrochloride] Pharmacia and Upjohn, Peapack, NJ); and interferon alpha-2 [2, 3].

View larger version (177K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1. —Hepatic epithelioid hemangioendothelioma. Photomicrograph shows epithelioid cells with signet ring–like structure (arrow). (H and E, x900)

The aim of this pictorial essay is to present the cross-sectional imaging features of hepatic epithelioid hemangioendothelioma. Most lesions are of nodular configuration, with a tendency to coalesce. The number of lesions and the amount of liver involved are variable.

Imaging Features

Top Introduction Imaging Features Extrahepatic Involvement Conclusion References

 
Abnormalities are usually multifocal, involving both lobes of the liver. Parenchymal calcification may occur, being sufficiently dense in some cases for depiction on abdominal radiographs [3, 4]. The uninvolved portions of hepatic parenchyma may undergo hypertrophy. On scintigraphy, decreased perfusion of involved areas with increased blood flow to uninvolved areas of liver may be seen [4]. It has been postulated that the shunting of blood can be explained by tumor growth within portal veins, thus reducing flow [4].

Tumors may appear as discrete nodules ranging from 0.5 to 12 cm in diameter or as complex confluent masses with a tendency to coalesce [4, 5]. Two forms of hepatic epithelioid hemangioendothelioma have been described, multifocal and diffuse. A multifocal nodular pattern of infiltration is observed in the early stage. Later, the lesions increase in size and coalesce, forming a diffuse pattern [4]. Many lesions are peripheral in location, extending to the capsule. Flattening or capsular retraction of the liver capsule due to fibrosis and compensatory hypertrophy of the unaffected liver segments may be observed. Other findings include focal hepatic calcification and signs of portal hypertension such as splenomegaly.

Sonography
On sonography, discrete nodules may be seen (Fig. 2A), or the liver may have a diffusely heterogeneous echotexture in regions of extensive diffuse involvement [4, 5] (Fig. 2B). Echogenicity of individual lesions is variable. Most frequently, the lesions are hypoechoic relative to adjacent hepatic parenchyma [4] (Fig. 3A), but masses may be hyperechoic and isoechoic relative to background liver. The internal architecture of a nodule may be complex and heterogeneous (Fig. 3B). There is no correlation between echogenicity and size of the tumor masses [4].

View larger version (141K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2A. —49-year-old woman, previously well, who presented with right upper quadrant pain. Percutaneous core biopsy (not shown) revealed hepatic epithelioid hemangioendothelioma. Transverse sonograms of liver show multiple hypoechoic nodules (arrows, A) and diffusely heterogeneous regions (B).

View larger version (134K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2B. —49-year-old woman, previously well, who presented with right upper quadrant pain. Percutaneous core biopsy (not shown) revealed hepatic epithelioid hemangioendothelioma. Transverse sonograms of liver show multiple hypoechoic nodules (arrows, A) and diffusely heterogeneous regions (B).

View larger version (124K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3A. —54-year-old woman with symptoms of general malaise and minimally elevated results of serum liver function tests. Subsequent laparoscopic biopsy showed hepatic epithelioid hemangioendothelioma. Transverse sonogram of liver shows hypoechoic nodules (arrows).

View larger version (138K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3B. —54-year-old woman with symptoms of general malaise and minimally elevated results of serum liver function tests. Subsequent laparoscopic biopsy showed hepatic epithelioid hemangioendothelioma. Sonogram shows detailed view of nodule, which is predominantly hypoechoic but also contains components isoechoic to background liver.

CT
Most of the lesions are peripheral, extending to the capsular margin. Capsular retraction adjacent to the mass is seen in fewer than 25% of patients [5] (Figs. 4 and 5). Hepatic parenchymal calcification may be seen [4] (Figs. 2C and 5). Tumor involvement can be widespread with extensive confluent masses and few traceable signs of portal or hepatic veins (Figs. 2E and 6). After administration of IV contrast material, some tumor nodules display marginal enhancement during the arterial phase [5] (Fig. 2D). On contrast-enhanced scans, the tumor nodules may become isodense to liver parenchyma. The extent of involvement may be better defined on unenhanced images [4].

View larger version (127K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4. —38-year-old woman with hepatic epithelioid hemangioendothelioma. Axial contrast-enhanced CT scan of liver shows peripheral coalescing masses with capsular retraction (arrows).

View larger version (134K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5. —44-year-old man with hepatic epithelioid hemangioendothelioma. Axial contrast-enhanced CT scan of liver shows multiple peripheral masses with capsular retraction and more confluent lesions centrally that contain calcification.

View larger version (123K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2C. —49-year-old woman, previously well, who presented with right upper quadrant pain. Percutaneous core biopsy (not shown) revealed hepatic epithelioid hemangioendothelioma. Unenhanced axial CT scan of liver shows multiple discrete low-attenuation lesions and calcification.

View larger version (134K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2E. —49-year-old woman, previously well, who presented with right upper quadrant pain. Percutaneous core biopsy (not shown) revealed hepatic epithelioid hemangioendothelioma. Portal venous phase of contrast-enhanced CT scan obtained at same level as C shows limited traceable signs of veins.

View larger version (124K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6. —38-year-old woman with hepatic epithelioid hemangioendothelioma. Axial contrast-enhanced CT scan of liver shows widespread confluent masses occupying most of hepatic parenchyma.

View larger version (127K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2D. —49-year-old woman, previously well, who presented with right upper quadrant pain. Percutaneous core biopsy (not shown) revealed hepatic epithelioid hemangioendothelioma. Arterial phase of contrast-enhanced CT scan obtained at same level as C shows marginal enhancement of some lesions.

MR Imaging
MR imaging shows the lesions on T1-weighted images as being of low intensity relative to uninvolved liver parenchyma, with some lesions containing central areas of lower signal intensity than the remainder of the tumor [5] (Fig. 2F). On T2-weighted images, lesions tend to be of heterogeneously increased signal [6, 7] (Fig. 2G). Some lesions may have a target appearance due to the presence of a central sclerotic zone and a peripheral region of cellular proliferation [7]. Central areas of reduced signal may correspond to areas of hemorrhage, coagulation necrosis, and calcification; peripheral high signal intensity corresponds to edematous connective tissue and viable tumor [6]. After IV contrast administration of gadopentetate dimeglumine, peripheral enhancement occurs with a thin nonenhancing rim corresponding to a narrow avascular zone between normal liver parenchyma and the nodules [6]. Ferumoxides-enhanced T1-weighted images more clearly define the extent of tumor than other images [6], but distinction between normal liver and tumor may be difficult on all sequences.

View larger version (88K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2F. —49-year-old woman, previously well, who presented with right upper quadrant pain. Percutaneous core biopsy (not shown) revealed hepatic epithelioid hemangioendothelioma. Axial T1-weighted spin-echo MR image (TR/TE, 416/11) of liver shows multiple lesions of low signal intensity.

View larger version (96K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2G. —49-year-old woman, previously well, who presented with right upper quadrant pain. Percutaneous core biopsy (not shown) revealed hepatic epithelioid hemangioendothelioma. Axial T2-weighted MR image (6000/135) of liver shows multiple lesions of high signal intensity.

Extrahepatic Involvement

Top Introduction Imaging Features Extrahepatic Involvement Conclusion References

 
Extrahepatic involvement may include the peritoneal lymph nodes (Fig. 3C), omentum, and mesentery [3] (Fig. 3D) and may sometimes be associated with calcification. Thoracic disease may be intrapulmonary or pleural [2, 3] (Fig. 7A). Extraperitoneal adenopathy may be seen (Fig. 7B). Metastases may be cutaneous or intramuscular (Fig. 7C). The mortality rates in patients with metastatic disease are greater than 60% [3].

View larger version (117K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3C. —54-year-old woman with symptoms of general malaise and minimally elevated results of serum liver function tests. Subsequent laparoscopic biopsy showed hepatic epithelioid hemangioendothelioma. Unenhanced axial CT scan of liver shows calcified periportal adenopathy (arrow).

View larger version (126K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3D. —54-year-old woman with symptoms of general malaise and minimally elevated results of serum liver function tests. Subsequent laparoscopic biopsy showed hepatic epithelioid hemangioendothelioma. Axial contrast-enhanced CT scan of pelvis shows calcified mesenteric mass (arrow).

View larger version (148K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7A. —Metastases in 58-year-old woman with hepatic epithelioid hemangioendothelioma diagnosed 4 years previously. Axial contrast-enhanced CT scan of lower thorax and upper abdomen shows multiple low-attenuation lesions in liver, with metastatic disease in thorax, as evidenced by left pleural thickening and pleural effusion.

View larger version (109K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7B. —Metastases in 58-year-old woman with hepatic epithelioid hemangioendothelioma diagnosed 4 years previously. Axial contrast-enhanced CT scan of inguinal region shows enhancing metastatic adenopathy (arrow).

View larger version (112K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7C. —Metastases in 58-year-old woman with hepatic epithelioid hemangioendothelioma diagnosed 4 years previously. Axial contrast-enhanced CT scan at L3–L4 level shows low-attenuation primary lesions in inferior aspect of liver and intramuscular metastasis (arrow) in right erector spinae muscle.

Conclusion

Top Introduction Imaging Features Extrahepatic Involvement Conclusion References

 
Because of nonspecific clinical manifestations and a prolonged clinical course of the disease, the age of the patient at the time that hepatic epithelioid hemangioendothelioma is detected may vary widely. More familiarity with the imaging findings may allow recognition of this tumor at earlier stages. Most lesions are hypoechoic on sonography, hypodense on CT, hypointense on T1-weighted MR imaging, and hyperintense on T2-weighted MR imaging. Enhancing margins and capsular retraction may be seen [4, 5, 6, 7].

Although multiple liver lesions in a predominantly peripheral distribution are rarely shown, the diagnosis of hepatic epithelioid hemangioendothelioma should be considered. Hepatic epithelioid hemangioendothelioma is one of the less aggressive hepatic tumors, and liver transplantation is now considered the treatment of choice, even in patients with known extrahepatic involvement [2, 8].

References

Top Introduction Imaging Features Extrahepatic Involvement Conclusion References

 

1. Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer 1982;50:970 –981[Medline]
2. Uchimura K, Nakamuta M, Osoegawa M, et al. Hepatic epithelioid hemangioendothelioma. J Clin Gastroenterol 2001;32:431 –434[Medline]
3. Makhlouf HR, Ishak KG, Goodman ZD. Epithelioid hemangioendothelioma of the liver: a clinicopatholgic study of 137 cases. Cancer 1999;85:562 –582[Medline]
4. Radin DR, Craig JR, Colletti PM, Ralls PW, Halls JM. Hepatic epithelioid hemangioendothelioma. Radiology 1988;169:145 –148[Abstract/Free Full Text]
5. Miller WJ, Dodd GD III, Federle MP, Baron RL. Epithelioid hemangioendothelioma of the liver: imaging findings with pathologic correlation. AJR 1992;159:53 –57[Abstract/Free Full Text]
6. Kehagias DT, Moulopoulos LA, Antoniou A, Psychogios V, Vourtsi A, Vlahos LJ. Hepatic epithelioid hemangioendothelioma: MR imaging findings. Hepatogastroenterology 2000;47:1711 –1713[Medline]
7. Ros LH, Fernandez L, Villacampa VM, Ros PR. Epithelioid hemangioendothelioma of the liver: characteristics on magnetic resonance imaging: case report. Can Assoc Radiol J 1999;50:387 –389[Medline]
8. Madariaga JR, Marino IR, Karavias DD, et al. Long-term results after liver transplantation for primary hepatic hemangioendothelioma. Ann Surg Oncol 1995;2:483 –487[Medline]

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