AJR 2002; 179:1457-1463
© American Roentgen Ray Society
CT and MR Imaging Features of Pathologically Proven Atypical Giant Hemangiomas of the Liver
M. Coumbaras1,
D. Wendum2,
L. Monnier-Cholley1,
H. Dahan1,
J. M. Tubiana1 and
L. Arrivé1
1 Department of Radiology, Hôpital Saint-Antoine, 184 Rue du Faubourg
Saint-Antoine, 75571 Paris cedex 12, France.
2 Department of Pathology, Hôpital Saint-Antoine, 75571 Paris cedex 12,
France.
Received December 27, 2001;
accepted after revision June 3, 2002.
Address correspondence to L. Arrivé.
Introduction
Although hepatic hemangiomas are the most common benign tumors of the
liver, giant hemangiomas of the liver are markedly less common. They are
defined as lesions greater than 4 cm in diameter. However, some authors
[1,2,3]
have defined giant hemangiomas of the liver as lesions greater than 6 or 10 cm
in diameter. Liver enlargement and abdominal discomfort may be observed in
patients with giant hemangioma of the liver
[1].
In most patients, giant hemangiomas of the liver are heterogeneous with a
markedly hypoattenuating central area depicted on unenhanced CT. On dynamic
contrast-enhanced CT, complete filling of the lesion is rarely shown.
Similarly, the central area is frequently markedly hypointense on T1-weighted
MR images and markedly hyperintense on T2-weighted MR images. Pathology
usually shows thrombosis, hyalinization, and fibrosis in the central area of
the lesion [2]. These typical
imaging features of giant hemangioma of the liver are relatively well known,
but the presence of atypical features may lead to a misdiagnosis. Our purpose
is to present a broad spectrum of atypical imaging features of giant
hemangioma to help avoid diagnostic
errors.
View larger version (144K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1E. —35-year-old woman with giant hemangioma of right lobe of
liver who presented with upper abdominal pain, fever, and anemia and was
referred because of liver abscess. Photomicrograph of histopathologic specimen
shows large areas of hemorrhagic necrosis (stars) and fibrous (F)
areas. (H and E, x200) (Reprinted with permission from
[1])
|
|
A series of 25 consecutive patients underwent surgery at our university
hospital for giant hemangioma. CT and MR images of these patients were
reviewed retrospectively to select cases with atypical imaging features. We
present giant hemangiomas of the liver with unusual characteristics: atypical
histology, compression of adjacent vascular or biliary structures, rupture,
unusual contrast enhancement, and other unusual imaging features.
However, an awareness of all these atypical radiographic features might not
obviate a biopsy in some cases.
Atypical Histologic Characteristics
Spontaneous hemorrhage is rare in giant hemangioma of the liver in
comparison with hepatocellular adenoma or hepatocellular carcinoma. On
unenhanced CT, hemorrhage appears as hyperdense zones in the central area of
the lesion (Figs. 1A and
2A). These zones are
hyperintense on T1-weighted MR images (Figs.
1B,
1D, and
2B). As a result of necrosis,
calcifications may be observed in the central area of a giant hemangioma of
the liver [3]
(Fig. 3A). Calcifications shown
in the marginal portion of the lesion, related to phleboliths, may also be
observed (Fig. 4A).
View larger version (126K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1A. —35-year-old woman with giant hemangioma of right lobe of
liver who presented with upper abdominal pain, fever, and anemia and was
referred because of liver abscess. Unenhanced CT scan shows main part of tumor
is hypodense. Central area of tumor is markedly more hypodense (curved
arrow) and corresponds to central necrosis. Clump of high density
(thick straight arrow) and rim of high density (thin straight
arrows) correspond to hemorrhagic foci.
|
|
View larger version (130K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 2A. —58-year-old man with giant hemangioma of right lobe of liver
who presented with right pain, fever, and anemia. Laboratory studies showed
cholestasis, which disappeared after right hepatectomy. Unenhanced CT scan
obtained at upper part of lesion shows spontaneous hyperdense areas
(arrows) in giant hemangioma of liver.
|
|
View larger version (113K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1B. —35-year-old woman with giant hemangioma of right lobe of
liver who presented with upper abdominal pain, fever, and anemia and was
referred because of liver abscess. T1-weighted MR image shows hyperintense
areas (arrows) in tumor that represent hemorrhage.
|
|
View larger version (118K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1D. —35-year-old woman with giant hemangioma of right lobe of
liver who presented with upper abdominal pain, fever, and anemia and was
referred because of liver abscess. Delayed phase (5 min) contrast-enhanced
T1-weighted MR image shows incomplete peripheral enhancement of lesion.
|
|
View larger version (164K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 2B. —58-year-old man with giant hemangioma of right lobe of liver
who presented with right pain, fever, and anemia. Laboratory studies showed
cholestasis, which disappeared after right hepatectomy. T1-weighted MR image
shows spontaneous hyperintense areas (arrows).
|
|
View larger version (133K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 3A. —50-year-old woman with giant hemangioma of liver resulting in
inferior vena cava thrombosis that was confirmed at liver transplantation.
Unenhanced CT scan shows central calcifications (arrow) in large
hypoattenuating lesion (arrowheads).
|
|
View larger version (116K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 4A. —35-year-old woman with giant hemangioma of liver 24 hr after
sonographically guided needle biopsy. Delayed phase (3 min) contrast-enhanced
CT scan shows incomplete filling of large heterogeneous hemangioma. Peripheral
spotty calcifications (arrowheads) are shown. Lesion biopsy was
complicated by free intraperitoneal hemorrhage (arrows).
|
|
MR imaging usually provides better tissue characterization than CT. This
statement is particularly true for visualization of septa not seen on CT
(Figs. 5A and
5B) but well shown on the
T2-weighted MR images (Fig.
5C). These septa correspond at pathology to strands of cellular
fibrous tissue [4]
(Fig. 5D). Similarly, MR
imaging easily detects spontaneous hemorrhage in giant hemangiomas of the
liver (Figs. 1B,
1C, and
2B). On the other hand,
calcifications are more readily detected on CT (Figs.
3A and
4A).
View larger version (122K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 5A. —47-year-old woman admitted for traumatic rupture of giant
hemangioma of liver after traffic collision. Unenhanced CT scan shows margins
of hemangioma (arrows) of liver. Small hemoperitoneum related to
rupture of giant hemangioma is shown around spleen and liver
(arrowheads).
|
|
View larger version (123K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 5B. —47-year-old woman admitted for traumatic rupture of giant
hemangioma of liver after traffic collision. Delayed phase (3 min)
contrast-enhanced CT scan shows large heterogeneous hemangioma
(arrows).
|
|
View larger version (112K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 5C. —47-year-old woman admitted for traumatic rupture of giant
hemangioma of liver after traffic collision. T2-weighted MR image shows that
lesion is hyperintense. Note hypointense linear elements corresponding to
internal fibrotic septa (arrows) that were not shown on CT.
|
|
View larger version (151K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 5D. —47-year-old woman admitted for traumatic rupture of giant
hemangioma of liver after traffic collision. Photomicrograph of
histopathologic specimen shows septa corresponding to internal fibrotic
strands (S). Between these strands, necrotic changes (N) in giant hemangioma
of liver are shown. (H and E, x200)
|
|
View larger version (115K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1C. —35-year-old woman with giant hemangioma of right lobe of
liver who presented with upper abdominal pain, fever, and anemia and was
referred because of liver abscess. T2-weighted MR image shows marked
hyperintensity of main part of tumor. Rim of low signal intensity
(arrows) corresponding to rim of high density on CT is related to
hemosiderin deposit.
|
|
Although giant hemangiomas are well circumscribed, capsules or
pseudocapsules are uncommon. On MR imaging, a peripheral low-signal-intensity
rim may be observed corresponding at pathology to a thin pseudocapsule of
fibrous tissue (Fig. 6). This
unusual development of a pseudocapsule may be related to fibrotic changes in
the adjacent liver parenchyma
[5].
Compressive Features
Hemangioma of the liver is a soft tumor and usually does not compress
adjacent structures. However, modifications of internal components such as
thrombosis and hemorrhage may transform the lesion into a firm solid mass and
may contribute to compression of biliary and vascular structures.
Intrahepatic bile duct dilatation (Figs.
2C and
7) may sometimes be shown in
giant hemangiomas of the liver
[6].
View larger version (127K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 2C. —58-year-old man with giant hemangioma of right lobe of liver
who presented with right pain, fever, and anemia. Laboratory studies showed
cholestasis, which disappeared after right hepatectomy. Intermediate (90 sec)
contrast-enhanced T1-weighted MR image shows intense nodular enhancement
(arrow) of lesion and biliary duct dilatation (arrowheads).
(Reprinted with permission from
[6])
|
|
View larger version (113K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 7. —32-year-old woman with giant hemangioma of liver and biliary
duct dilatation. Arterial phase contrast-enhanced T1-weighted MR image shows
large lesion that displaces both right (R) and left (L) portal veins. Left
biliary duct dilatation (arrowheads) is also shown.
|
|
Compression of the inferior vena cava may be observed (Figs.
3B and
3C). Portal vein compression
may also be seen, which results in areas of perfusion abnormalities either on
CT or MR imaging (Figs. 8A and
9A).,,
View larger version (122K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 3B. —50-year-old woman with giant hemangioma of liver resulting in
inferior vena cava thrombosis that was confirmed at liver transplantation.
T1-weighted MR image shows large heterogeneous hepatic mass
(arrowheads). Large interhepatic collateral vein is shown as
hypointense rounded structure (arrow). Retrohepatic inferior vena
cava is not shown.
|
|
View larger version (149K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 3C. —50-year-old woman with giant hemangioma of liver resulting in
inferior vena cava thrombosis that was confirmed at liver transplantation.
Venacavagram shows upper inferior vena cava obstruction and opacification of
large interhepatic collateral vein arising from vena cava below obstruction
(arrow).
|
|
View larger version (155K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 8A. —70-year-old man with hyalinized giant hemangioma of liver.
Venous phase contrast-enhanced CT scan shows markedly heterogeneous uptake of
contrast material within lesion (arrows). Increased attenuation
related to perfusion abnormalities is also shown in left lobe of liver
(arrowheads).
|
|
View larger version (112K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 9A. —36-year-old woman with giant hemangioma of liver associated
with focal nodular hyperplasia. Arterial phase contrast-enhanced T1-weighted
MR image shows typical nodular enhancement of liver hemangioma (thick
arrowheads). Massive, intense enhancement of focal nodular hyperplasia
(large arrows) and unenhanced central scar are shown (small
arrow). Areas of different signal intensity related to perfusion
abnormalities are shown at periphery of lesions (thin
arrowheads).
|
|
View larger version (132K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 4B. —35-year-old woman with giant hemangioma of liver 24 hr after
sonographically guided needle biopsy. Photomicrograph of histopathologic
specimen shows that dilated vascular spaces (V) lined by flat endothelial
cells (arrowheads) are supported by connective tissue (C). (H and E,
x200)
|
|
View larger version (141K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 6B. —43-year-old woman with giant hemangioma of right lobe of
liver. Photomicrograph of histopathologic specimen shows large necrotic areas
(N). Septa of fibrosis extend to periphery of tumor, creating thin
pseudocapsule of fibrous tissue (arrows). (H and E, x50)
|
|
View larger version (124K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 8B. —70-year-old man with hyalinized giant hemangioma of liver.
Photomicrograph of histopathologic specimen shows large fibrous areas in which
only few vascular spaces are present (arrows). (H and E,
x100)
|
|
Rupture
Even in large hemangiomas, rupture is rare
[1]. Rupture of giant
hemangiomas of the liver may be observed after severe trauma (Figs.
5A and
5B). Uncommonly, hemorrhage
may also be identified in giant hemangiomas after biopsy
(Fig. 4A).
Unusual Patterns of Contrast Enhancement
The typical enhancement pattern of a giant hemangioma of the liver consists
of dense nodular enhancement of the periphery of the lesion with gradual
filling-in of the vascular spaces toward the center of the lesion over
time.
Rarely, giant hemangiomas of the liver may be observed without any contrast
enhancement. On CT, the mass is not enhanced after injection of contrast media
even on delayed phase images (Fig.
10A). On delayed T1-weighted MR imaging, no pooling of gadolinium
at the periphery of the tumor is present
(Fig. 10C). Microscopic
examination usually shows that the tumor is composed of large spaces even at
the periphery; organized thrombi may also be revealed in the vascular spaces
(Fig. 10D). Filling-in tends
to be slow because vascular flow is slower in enlarged vascular spaces than in
small vascular spaces [7].
Complete hyalinization has also been reported in nonenhancing hemangiomas
[4]. Pathologic examination
shows extensive fibrous transformation and obliteration of vascular spaces. In
other cases, heterogeneous hyalinization may result in massive heterogeneity
both before and after contrast injection
(Fig.
8A).,,
View larger version (112K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 10A. —22-year-old woman with giant hemangioma of liver. Delayed
phase (3 min) contrast-enhanced CT scan shows absence of enhancement of lesion
(arrows). Hemangioma is surrounded by branches of middle hepatic
vein.
|
|
View larger version (121K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 10C. —22-year-old woman with giant hemangioma of liver. Delayed
phase (5 min) contrast-enhanced T1-weighted MR image shows absence of
enhancement of lesion (arrows). Hemangioma is surrounded by branches
of middle hepatic vein.
|
|
View larger version (138K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 10D. —22-year-old woman with giant hemangioma of liver.
Photomicrograph of histologic specimen shows organized thrombi
(arrows) in vascular spaces and dilated vascular spaces (V) lined by
flat endothelial cells (arrowheads). (H and E, x200)
|
|
View larger version (111K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 9B. —36-year-old woman with giant hemangioma of liver associated
with focal nodular hyperplasia. T2-weighted MR image shows typical features of
giant hemangioma (H) of liver and high signal intensity of central scar of
focal nodular hyperplasia (arrow).
|
|
View larger version (145K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 11B. —32-year-old woman with giant hemangioma of liver with
capsular retraction. T1-weighted MR image similarly shows capsular retraction
(curved arrow). Margins of hemangioma are indicated by straight
arrows. G = gallbladder.
|
|
Other Unusual Imaging Features
Capsular retraction is usually associated with malignant tumors. Mild or
marked capsular retraction may sometimes be observed in cases of giant
hemangioma of the liver (Fig.
11A,11B).
View larger version (169K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 11A. —32-year-old woman with giant hemangioma of liver with
capsular retraction. Arterial phase contrast-enhanced CT scan shows early
peripheral enhancement of lower part of hemangioma (straight arrows).
Capsular retraction is shown (curved arrow). Note gallbladder (G)
containing gallstone.
|
|
Kasabach-Merritt syndrome is a rare complication of giant hemangiomas of
the liver. In adults, this syndrome is characterized by a fibrinolysis in the
hemangiomas, which results in thrombocytopenia. We observed such a case of
marked and progressive thrombocytopenia in a patient with a giant hemangioma
of the liver. Neither CT nor MR imaging showed any specific feature
(Fig. 12).
View larger version (109K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 12. —43-year-old woman with giant hemangioma (arrows) of
right lobe of liver complicated by Kasabach-Merritt syndrome. Delayed phase (5
min) contrast-enhanced T1-weighted MR image shows incomplete filling of
lesion.
|
|
Finally, giant hemangiomas may be associated with other liver lesions such
as focal nodular hyperplasia (Fig.
9A,9B).
The association of hepatic hemangioma and focal nodular hyperplasia is quite
frequent and not fortuitous
[8]. Focal nodular hyperplasia
is thought to have a vascular origin, like hemangioma. When the tumors have
typical imaging features, the diagnosis can be performed with confidence.
However, an association of a giant hemangioma of the liver with any other
hepatic lesions, whether benign or malignant, may lead to a misdiagnosis.
Acknowledgments
We thank Pascale Dono for her assistance with preparation of this
manuscript.
References
- Bouras T, Arrivé L, Monnier-Cholley L, et al. Imagerie d'un
angiome géant symptomatique. J Radiol
1996;77:1145
-1148[Medline]
- Ros PR, Lubbers PR, Olmsted WW, Morillo G. Hemangioma of the liver:
heterogeneous appearance of T2-weighted images. AJR
1987;149:1167
-1170[Abstract/Free Full Text]
- Mitsudo K, Watanabe Y, Saga T, et al. Nonenhanced hepatic cavernous
hemangioma with multiple calcifications: CT and pathologic correlation.
Abdom Imaging
1995;20:459
-461[Medline]
- Vilgrain V, Boulos L, Vullierme MP, Denys A, Terris B, Menu Y.
Imaging of atypical hemangiomas of the liver with pathologic correlation.
RadioGraphics
2000;20:379
-397[Abstract/Free Full Text]
- Takayasu K, Moriyama N, Shima Y, et al. Atypical radiographic
findings in hepatic cavernous hemangioma: correlation with histologic
features. AJR
1986;146:1149
-1153[Abstract/Free Full Text]
- Issahar-Zadeh A, Monnier-Cholley L, Tiret E, et al.
Hémangiome hépatique géant responsable d'une dilatation
des voies biliaires intrahépatiques. J Radiol
1997;78:381
-384[Medline]
- Hanafusa K, Ohashi I, Himeno Y, Suzuki S, Shibuya H. Hepatic
hemangioma: findings with two-phase CT. Radiology
1995;196:465
-469[Abstract/Free Full Text]
- Mathieu D, Zafrani ES, Anglade MC, Dhumeaux D. Association of focal
nodular hyperplasis and hepatic hemangioma.
Gastroenterology
1989;97:154
-157[Medline]
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
Top
Introduction
Atypical Histologic...
