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Takashi Koyama, MD Primary Hepatic

Joel G. Fletcher, MD
C. Daniel Johnson, MD Angiosarcoma: Findings at CT
Mark S. Kuo, MD
Kenji Notohara, MD
Lawrence J. Burgart, MD
and MR Imaging1
PURPOSE: To evaluate and describe cross-sectional imaging findings in patients
Index terms: with pathologically confirmed primary hepatic angiosarcoma.
Liver neoplasms, 761.322
Liver, nodules, 761.322 MATERIALS AND METHODS: Findings from imaging examinations in 13 patients
Sarcoma, 761.322 with pathologically confirmed primary hepatic angiosarcoma were retrospectively
reviewed (computed tomographic [CT] images obtained in 10 patients and mag-
Published online before print
10.1148/radiol.2223010877 netic resonance [MR] images obtained in five patients were available for review).
Radiology 2002; 222:667– 673 Two gastrointestinal radiologists evaluated lesion number, size, attenuation and
signal intensity characteristics, and the pattern and degree of contrast material
1
From the Department of Diagnostic enhancement. Medical records were reviewed for clinical features associated with
Imaging and Nuclear Medicine, School angiosarcoma.
of Medicine, Kyoto University, Japan
(T.K.); Departments of Radiology (J.G.F., RESULTS: Angiosarcoma appeared as multiple nodules (n ⫽ 6), as dominant masses
C.D.J.) and Anatomic Pathology (K.N., (n ⫽ 6), or as a diffusely infiltrating lesion (n ⫽ 1). Multiple nodules were hypoat-
L.J.B.), Mayo Clinic, 200 First St SW,
tenuating at unenhanced and contrast material– enhanced CT (six of six patients).
Mayo East 2B, Rochester, MN 55905;
and Department of Radiology, Scotts- When dominant masses were encountered at MR imaging, T2-weighted MR imag-
dale Medical Imaging, Ariz (M.S.K.). ing demonstrated heterogeneous internal architecture (four of four patients) similar
Received May 2, 2001; revision re- to that of hepatocellular carcinoma. Multiphase contrast-enhanced CT and MR
quested June 8; revision received and
accepted September 7. Address cor- images showed dominant masses to have heterogeneous and progressive enhance-
respondence to J.G.F. (e-mail: fletcher ment (three of three patients). Clinical features associated with angiosarcoma
.joel@mayo.edu). included splenic metastases (six of 13 patients), thrombocytopenia (seven of 13
© RSNA, 2002 patients), disseminated intravascular coagulation (four of 13 patients), and hemo-
lytic anemia (three of 13 patients).
CONCLUSION: Primary hepatic angiosarcoma exhibits a spectrum of appearances
that reflect its varied pathologic features.
© RSNA, 2002

While primary hepatic angiosarcoma accounts for only 2% of primary hepatic tumors, it
is the most common malignant mesenchymal tumor of the liver (1– 4). Hepatic angiosar-
coma portends a poor prognosis, and most patients die within a year of diagnosis (3).
When the lesion is confined to one lobe of the liver without any metastatic lesions,
however, it is resectable. Because of the vascularity of the lesion, percutaneous biopsy has
been reported as treacherous, complicated by massive hemorrhage in a minority of
patients (3).
Author contributions:
Early reports of hepatic angiosarcoma focused on its association with environmental
Guarantors of integrity of entire study, carcinogens, such as thorium dioxide (Thorotrast), arsenic, and vinyl chloride, but expo-
T.K., J.G.F.; study concepts and de- sure to these agents is now rare. Most of these tumors occur either in the absence of known
sign, T.K., J.G.F.; literature research, risk factors or with cirrhosis (2).
T.K.; clinical studies, T.K., J.G.F.; ex- Various appearances of hepatic angiosarcoma on computed tomographic (CT) images
perimental studies, all authors; data
acquisition and analysis/interpretation, have been described in case reports and in a few small series (5–11). On CT images,
T.K., J.G.F., C.D.J.; manuscript prepara- angiosarcoma has been known to simulate benign hemangioma or metastases (7,11). The
tion and definition of intellectual con- appearance of this tumor at magnetic resonance (MR) imaging is described in only a few
tent, all authors; manuscript editing, sporadic case reports in the English-language literature, so its MR features have not been
T.K., J.G.F., C.D.J.; manuscript revision/
review, all authors; manuscript final ver- clearly delineated (11–13). The purpose of our study was to evaluate and describe cross-
sion approval, T.K., J.G.F., C.D.J. sectional imaging findings in patients with pathologically confirmed primary hepatic
angiosarcoma.

667
MATERIALS AND METHODS intravenous administration of 10 mL of and one underwent a radiographic, met-
gadopentetate dimeglumine (Magnevist; astatic bone survey.
After receiving approval from our institu- Berlex Laboratories, Wayne, NJ) or 16 mL
tional review board, we reviewed cross- of gadoteridol (ProHance; Bracco Diag- RESULTS
referenced records from May 1984 to Jan- nostics), respectively.
uary 2001 in the departments of radiology Two gastrointestinal radiologists (T.K., Clinical Information
and pathology at the Mayo Clinic and J.G.F., C.D.J.) examined lesion size (di- Patients most frequently presented
identified 13 patients with pathologically ameter in centimeters), number, and lo- with upper-quadrant pain (n ⫽ 7), ab-
confirmed angiosarcoma. Patients were cation, as well as characteristics such as dominal discomfort (n ⫽ 7), anorexia
not individually asked for consent to be hemorrhage, necrosis, and sites of metas- (n ⫽ 4), or weight loss (n ⫽ 3). Findings of
included in this study, but each patient tasis, and formed a consensus interpreta- laboratory tests revealed that seven pa-
in the study did agree to the retrospective tion. Tumor attenuation (for the entire tients had thrombocytopenia, four of
use of medical records and images for tumor and for the foci of enhancement whom had disseminated intravascular
research purposes during treatment at within the tumor) was visually graded coagulation. All four patients with dis-
our institution. These 13 patients, in- as greater than, less than, or equal to seminated intravascular coagulation had
cluding eight men and five women, con- the attenuation of the surrounding he- elevated D-dimer and low fibrinogen lev-
stituted our study population. Patient age patic parenchyma and the aorta. En- els. Eight patients had anemia, including
ranged from 37 to 84 years (mean age, hancement patterns within tumors were three with microangiopathic hemolytic
66.7 years ⫾ 10.9 [SD]). CT images were characterized as focal regions of enhance- anemia and one with known aplastic
available in 10 patients, and MR images ment, irregularly shaped regions of en- anemia. Several patients had medical his-
were available in five patients. Two pa- hancement, or rim enhancement. On MR tories related to their development of an-
tients had both CT and MR images avail- images, the signal intensity characteris- giosarcoma. Two patients had a history
able for review. tics of the lesion were compared with of exposure to Thorotrast. One patient
All patients had biopsy-proven hepatic those of the surrounding liver paren- had a chronic organized hematoma along
angiosarcoma (nine patients underwent chyma and the muscles, and the pattern the lateral surface of the liver (a compli-
CT-guided biopsy, two underwent surgi- of enhancement was also characterized, cation of percutaneous transhepatic bili-
cal resection, and four underwent explor- as described above. Pathologic findings ary drainage 5 years prior to presenta-
atory laparotomy). CT-guided biopsy was in surgical specimens were compared tion), with a massive angiosarcoma in
unsuccessful in two patients (requiring with imaging findings (T.K., K.N., L.J.B.). the vicinity of the hematoma. Another
subsequent exploratory laparotomy), and Point-to-point comparison was not pos- patient had abused anabolic steroids and
substantial bleeding was not observed in sible, however, since only two patients acquired transfusion-induced hemochro-
the immediate postprocedure period in underwent complete surgical resection. matosis subsequent to his known aplastic
any of the patients who underwent percu- Clinical records of patients were re- anemia. Another patient had a husband
taneous biopsy. viewed (T.K., J.G.F.) regarding clinical who worked with arsenic and lead-based
Unenhanced CT images were obtained presentation, preexisting diseases, ab- pesticides. None of the patients in our
in six patients, and contrast material– en- normal findings of laboratory tests, and study had a history of exposure to vinyl
hanced CT images were obtained in eight history of exposure to environmental chloride.
patients with the use of intravenous iodin- carcinogens. Clinical histories were re-
ated contrast material. Six patients re- viewed for history of exposure to Thoro- Imaging Findings
ceived 140–150 mL of iopamidol (Isovue trast, vinyl chloride, anabolic steroids, or The number and size of hepatic tumors
300; Bracco Diagnostics, Princeton, NJ), arsenic, and for history of hemochroma- were based on CT and MR imaging find-
and two patients received 150 mL of iothal- tosis, chronic hematoma, or pyothorax. ings. Six patients had multiple small nod-
amate meglumine (Conray 60; Mallinck- The following values were obtained from ules, usually measuring less than 3 cm in
rodt Medical, St Louis, Mo). Regarding the laboratory tests and recorded at diagnosis diameter, scattered within both lobes of
contrast-enhanced examinations, one pa- of angiosarcoma (n ⫽ 12) or at presenta- the liver. Five patients had a large domi-
tient underwent imaging in the arterial tion to our institution (n ⫽ 1), when ap- nant mass measuring 8 –14 cm (three in
phase, seven underwent imaging in the plicable: hemoglobin (n ⫽ 13), hemato- the right lobe, one in the left lobe, and
portal venous phase, and two underwent crit (n ⫽ 11), platelets (n ⫽ 13), D-dimer one in both lobes). Four of these five
delayed imaging. Section thickness ranged (n ⫽ 4), and fibrinogen (n ⫽ 4). patients had other intrahepatic lesions
between 5 and 10 mm. Clinical records of patients were also that measured less than 3 cm. One pa-
Transverse T1- and T2-weighted MR reviewed for sites of metastasis. All 13 tient had two large focal lesions (one in
images were obtained in five patients. A patients underwent either chest CT (n ⫽ each lobe) measuring 7–9 cm, and one
conventional spin-echo technique was 9) or chest radiography (n ⫽ 13) to doc- patient had a diffusely infiltrating tu-
used to obtain T1-weighted images (rep- ument pulmonary metastases. Splenic mor throughout the liver.
etition time msec/echo time msec, 250 – metastases were assessed either with sur- Seven patients (54%) had metastatic
660/14 –20). Regarding T2-weighted im- gical specimens (n ⫽ 2) or with CT or MR lesions. Six (46%) had splenic metasta-
ages, a fast spin-echo technique (4,000 – imaging of the abdomen (n ⫽ 11), as ses, while three (23%) had lung metas-
8,571/105–140) was used in two patients, previously described. Bone metastases tases. Three (23%) also had bone metasta-
and a conventional spin-echo technique were noted when present on any image ses, but only eight patients underwent
(2,000 –2,500/100 –120) was used in three (CT, MR, or plain radiographic). All pa- either a metastatic bone survey or CT of
patients. Dynamic T1-weighted three-di- tients underwent MR imaging or CT of the entire chest, abdomen, and pelvis. On
mensional fast spoiled gradient-echo im- the abdomen, nine underwent CT of the unenhanced CT images, all lesions were
ages were obtained in two patients after chest, seven underwent CT of the pelvis, hypoattenuating compared with normal

668 䡠 Radiology 䡠 March 2002 Koyama et al


hancement were located predominantly size, necrosis was present in 10 of 12
in the central portion of each lesion, with (83%) specimens, and hemorrhage was
a few located peripherally. Two of these present in nine of 11 (82%) specimens
three patients had innumerable nodular (one was too small to evaluate). The pres-
masses. ence of necrosis and hemorrhage in the
On contrast-enhanced CT images, three examined samples correlated with high
patients had a large, dominant mass lesion T1-weighted signal intensity at MR imag-
in the liver. Two of these dominant masses ing in four of five tumors. Heterogeneous
demonstrated heterogeneous enhance- attenuation on unenhanced CT scans
ment that suggested central necrosis and correlated with the presence of necrosis
fibrotic change (Fig 2). One of these pa- in four of six patients and hemorrhage in
tients had a solitary tumor that arose in three of five patients. Cavernous spaces
the vicinity of a chronic organized hema- lined with neoplastic cells were present
toma, and portal- and delayed-phase im- in six of eight (75%) cases with suffi-
aging demonstrated heterogeneous and ciently large sample volume. For exam-
progressive enhancement of the tumor ple, one patient with hemochromato-
(Fig 2). The second patient had a domi- sis and a dominant mass with satellite
nant mass with multiple satellite nodules tumors at imaging had a tumor that
in the liver. The third patient had two contained numerous, blood-filled cystic
dominant masses of homogeneous hy- spaces (Fig 3). In another patient with a
poattenuation without focal enhance- multifocal tumor, images showed abun-
ment. dant cavernous spaces lined with tumor
On MR images, four patients had dom- cells (Fig 1). These cavernous vascular
inant mass lesions, and three were ac- spaces simulated those seen with cavern-
companied by satellite lesions. On T1- ous hemangioma; interestingly, these tu-
weighted images, all of the dominant mors demonstrated focal enhancement
masses were of low intensity but con- at contrast-enhanced CT (Fig 1). Another
tained focal areas of high intensity, sug- lesion, which arose at the site of a
gesting hemorrhage (Figs 2– 4). On T2- chronic hematoma, was predominantly
weighted images, these dominant masses solid and demonstrated neoplastic cells
Figure 1. Images show angiosarcoma in a 62- demonstrated predominantly increased (forming freely anastomosing vascular
year-old man. (a) Transverse contrast-enhanced T2 signal intensity compared with that of channels with few dilated vascular spac-
CT scan shows multiple hypoattenuating liver the skeletal muscle and the normal liver, es), central fibrosis, and hyalinization
lesions, some with foci of enhancement (arrow- and the images showed a heterogeneous (Fig 2).
heads), which are of decreased attenuation com- appearance with focal areas of high in- Some tumors did have unique features.
pared with the aorta. (b) Photomicrograph
shows dilated, cavernous vascular channels (ar-
tensity interspersed with septumlike re- MR images of one patient showed little
rows) lined with neoplastic cells. (Hematoxylin- gions of low intensity (Figs 2– 4). On T2- heterogeneity of signal intensity in the
eosin stain; original magnification, ⫻50.) weighted images, satellite lesions showed liver. This patient had undergone splenec-
high intensity (Fig 3) or fluid-fluid levels, tomy and wedge resection in the lateral
which are typical of intratumoral hemor- segment 10 months prior to the imaging
rhage (Fig 4). One patient did not have a study. At the time of resection, the surgeon
liver parenchyma. The lesions had attenu- focal intrahepatic mass, but rather had noted that the liver “contained a diffuse
ation similar to that of the aorta, but they diffuse heterogeneous signal intensity process, appearing vascular in nature, char-
exhibited a heterogeneous appearance be- throughout the liver on T1-weighted im- acterized by small, less-than-1-cm, reddish-
cause of the presence of focal areas of hy- ages (Fig 5). Corresponding T2-weighted purple spots.” At histologic analysis, the
perattenuation. In two patients with a his- images showed no apparent mass lesion spleen and entire wedge resection were
tory of Thorotrast exposure, multiple (Fig 5). diffusely involved with angiosarcoma,
lesions displaced the linear network of re- On dynamic contrast-enhanced MR with hyperchromatic tumor cells dif-
sidual Thorotrast, and the spleen was atro- images, the pattern of enhancement in fusely spreading along the portal tracts
phic and hyperattenuating due to prior the arterial and portal phases was mark- and sinusoidal spaces without forming
Thorotrast exposure. edly heterogeneous within the dominant macronodular lesions (Fig 5).
Contrast-enhanced CT images (n ⫽ 8) masses. Delayed imaging resulted in pro-
showed multiple tumor nodules in five gressive enhancement over time com-
patients. In these patients, tumor nod- pared with the enhancement of the sur- DISCUSSION
ules were hypoattenuating. In three pa- rounding hepatic parenchyma, except in
tients, most lesions contained focal areas central regions of hemorrhage, necrosis, Angiosarcoma is the most common pri-
of enhancement (Fig 1a). The attenua- or fibrosis. mary sarcoma in the liver (1– 4). It com-
tion of many foci of enhancement was monly affects patients 60 –70 years of age
less than that of the aorta but greater but is also known to occur in younger
Pathologic Findings
than that of the hepatic parenchyma. A patients (2,3). A strong male predomi-
few foci of enhancement were isointense All tumors were characterized micro- nance has been reported, with a male-
with the aorta. Some areas of enhance- scopically by spindle-shaped cells, which female ratio of four to one (3).
ment had irregular shapes, and others demonstrated vascular formation in var- Angiosarcoma has received attention
were ring shaped. Focal regions of en- ious patterns. In specimens of substantial in recent decades because of its frequent

Volume 222 䡠 Number 3 Primary Hepatic Angiosarcoma 䡠 669


Figure 2. Images show angiosarcoma associ-
ated with a chronic organized subcapsular he-
matoma (arrowheads) in a 76-year-old man.
(a) Transverse CT scan in the portal phase dem-
onstrates a heterogeneous enhancement pat-
tern in the lesion (arrows). At resection, the tu-
mor was found adherent to (but not invading)
the diaphragm. (b) Transverse contrast-en-
hanced dynamic delayed-phase CT scan dem-
onstrates progressive enhancement over time
(arrows). (c) Transverse T1-weighted spin-echo
(266/14) MR image shows a massive tumor (ar-
row) in the vicinity of a chronic organized sub-
capsular hematoma (arrowheads). The lesion
contains focal areas of high intensity, which
suggest hemorrhage. (d) Transverse fat-satu-
rated T2-weighted fast spin-echo (8,571/70) MR
image shows the marked heterogeneous appear-
ance of the lesion (arrows) and hematoma (ar-
rowheads). (e) Cut section of the gross specimen
shows a hemorrhagic mass with a chronic orga-
nized subcapsular hematoma (white arrow-
heads). Fibrotic scar (hyalinization), which is
whitish in color, is notable in the central area of
the lesion (black arrowhead).
association with several environmental thrombocytopenia, are frequently associ-
carcinogens, such as Thorotrast, vinyl ated (3). Most patients in our series were
chloride, and arsenic compounds (5,6). anemic (62%) and thrombocytopenic
spleen is the site of the primary neo-
Both hemochromatosis and anabolic ste- (54%), with microangiopathic hemolytic
roids have been associated with angiosar- anemia in 23%. Erythrocytes are thought plasm. It may be speculated that splenic
coma (14,15), as seen in one patient with to be traumatized in the poorly organized lesions represent simultaneous occur-
aplastic anemia in our series. Chronic in- neoplastic vessels, and platelets may be rence, which may be related to known or
flammation has also been implicated as a trapped. Occasionally, this localized co- unknown environmental carcinogens.
potential cause of the development of agulopathy results in the development of Diagnosis by means of liver biopsy has
pleural angiosarcoma in patients with systemic disseminated intravascular co- been reported as treacherous and nondi-
pyothorax (16). In one of our patients, agulation, which was seen in 31% of our agnostic (17). Our experience differs in
angiosarcoma originated in or adjacent patients. that seven of nine (78%) percutaneous
to a chronic organized hematoma. How- Most patients have metastatic lesions biopsies in our series yielded diagnostic
ever, in most patients with primary he- at the time of presentation. The most specimens without substantial bleeding.
patic angiosarcoma, cause is not appar- common site of metastases is the lung, Nevertheless, recent reports of lethal com-
ent (2,3). followed by the spleen (3). In our series, plications following image-guided percuta-
Patients usually present with nonspe- 23% of patients had pulmonary metasta- neous biopsy remain (17). Therefore, surgi-
cific symptoms, including abdominal pain, ses, but 46% had splenic metastases. The cal backup may be prudent if percutaneous
weakness, fatigue, anorexia, and weight high frequency of splenic metastases is biopsy is attempted.
loss. Hematologic abnormalities, including notable, but in some instances it is diffi- The gross appearance of angiosarcoma
microangiopathic hemolytic anemia and cult to determine whether the liver or the at pathologic evaluation is characterized

670 䡠 Radiology 䡠 March 2002 Koyama et al


findings of angiosarcoma. In the case of
Thorotrast exposure, which is well docu-
mented, tumors are hypoattenuating on
unenhanced CT scans and are inferred
by the displacement of the hyperat-
tenuating linear network of residual
Thorotrast (4 – 6). Even in patients with-
out Thorotrast exposure, unenhanced
CT images demonstrate the tumor to be
predominantly hypoattenuating com-
pared with the surrounding hepatic pa-
renchyma. On contrast-enhanced im-
ages, most lesions are hypoattenuating
compared with normal liver tissue, but
some lesions can be hyperattenuating
(8). While White et al have reported pro-
gressive centripetal enhancement similar
to that of cavernous hemangioma (10),
recent reports have demonstrated that
angiosarcoma does not resemble benign
Figure 3. MR images show angiosarcoma in a 37-year-old man with known aplastic anemia. cavernous hemangioma at biphasic im-
(a) Transverse T1-weighted spin-echo (250/17) MR image shows a diffuse lesion involving almost
the entire posterior segment of the right lobe of the liver. Numerous small nodules of high
aging (8,9,12). Peterson et al found only
intensity suggest a focal area of hemorrhage. Diffuse decrease in signal intensity in the liver, one tumor nodule in six patients that
spleen (not shown), and bone marrow is consistent with patient’s known secondary hemochro- had the typical nodular enhancement of
matosis. (b) Transverse fat-saturated T2-weighted spin-echo (2,500/120) MR image shows com- a hemangioma (8). We observed focal ar-
partmentalization within the lesion that contains numerous focal areas of high intensity. eas of enhancement in three of five (60%)
patients with multiple tumor nodules.
These lesions were different from multi-
ple cavernous hemangiomas for several
reasons: Focal areas of enhancement
showed less attenuation than the aorta,
with bizarre shapes, central enhance-
ment, or peripheral ring-shaped en-
hancement. Innumerable hepatic lesions
indicated that a malignancy was present.
Unlike the patient group of Peterson et
al, three patients in our series had large
dominant masses. None of these patients
demonstrated foci of enhancement simi-
lar to those of hemangioma.
Although there have been several re-
ports on the CT appearances of angiosar-
coma, little has been discussed on the MR
imaging features of angiosarcoma and
the role of MR imaging in preoperative
diagnosis. We found that MR imaging
Figure 4. MR images show angiosarcoma in a 65-year-old man. (a) Transverse T1-weighted demonstrated the hemorrhagic, hetero-
spin-echo (250/15) MR image shows multiple low-intensity lesions (arrows) that contain focal geneous, and hypervascular nature of all
areas of slightly high T1-weighted signal intensity. (b) Transverse fat-saturated T2-weighted the dominant masses in our series.
spin-echo (2,500/100) MR image shows heterogeneous signal intensity throughout the dominant On T1-weighted MR images, all four
mass. Fluid-fluid levels can be seen in smaller satellite lesions (arrows). dominant mass lesions contained irreg-
ular areas of high signal intensity, sug-
gesting hemorrhage. The high frequency
by the presence of remarkable necrosis lated sinusoidal or cavernous spaces that of hemorrhage in the lesions demon-
and hemorrhage and can show four types simulate cavernous hemangioma to slit- strated on T1-weighted images is in good
of growth patterns: multiple nodules, a like, freely anastomosing vascular chan- accordance with gross pathologic fea-
large dominant mass, mixed patterns of nels (2,18,19). These various vascular pat- tures. The presence of fluid-fluid levels on
a dominant mass with nodules, and, terns are usually intermingled with each T2-weighted images is another finding
rarely, a diffusely infiltrating micronodu- other, with the predominant pattern dif- that reflects the hemorrhagic nature of
lar tumor (18,19). Angiosarcoma is char- fering in each patient. Fibrosis and dep- angiosarcoma, but this finding is also of-
acterized microscopically by spindle- osition of hemosiderin are frequently en- ten seen with hypervascular metastases.
shaped cells that form vascular channels. countered in solid portions of the tumor All four dominant mass lesions that
These channels show a wide spectrum of (18,19). underwent T2-weighted MR imaging also
patterns, ranging from excessively di- There have been several reports on CT showed a markedly heterogeneous archi-

Volume 222 䡠 Number 3 Primary Hepatic Angiosarcoma 䡠 671


tecture, with focal areas of high intensity
along with septumlike or rounded areas
of low intensity on T2-weighted images.
This appearance suggests compartmen-
talization within the tumor, and it is sim-
ilar to that observed with hepatocellular
carcinoma. Areas of low signal intensity
on T2-weighted images may reflect he-
mosiderin, fibrous solid portions, or fresh
hemorrhage, on occasion, while areas of
high intensity may represent hemor-
rhage or necrosis. Unlike results in earlier
case reports, no tumors in our series
showed a central area of low intensity or
a few internal septa that could have been
confused with hemangioma (11,20,21).
On dynamic contrast-enhanced MR
images, each lesion showed heteroge-
neous enhancement on the arterial- and
portal-phase images. At delayed imaging,
however, there was progressive enhance-
ment of the lesion compared with that of
early-phase images. The heterogeneous
enhancement pattern on contrast-en-
hanced images was similar to that ob-
served with CT and likely represents the
heterogeneity of microscopic vascular
patterns within each tumor. The areas
with abundant, freely anastomosing vas-
cular channels may enhance quickly,
while dilated cavernous vascular spaces
may show slowly progressive enhance-
ment.
In one patient with diffuse signal het-
erogeneity throughout the liver on T1-
weighted MR images, pathologic findings Figure 5. Images show angiosarcoma that diffusely involves the entire liver in a 64-year-old-
were surprising, as tumor cells diffusely man. (a) Transverse T1-weighted spin-echo (500/15) MR image shows slightly heterogeneous
signal intensity of the liver and ascites. (b) Transverse T2-weighted spin-echo (2,000/100) MR
infiltrated the liver parenchyma along
image shows little abnormality. (c) Photomicrograph with a low-power view (hematoxylin-eosin
the portal tracts. This micronodular pat- stain; original magnification, ⫻25) shows hyperchromatic cells diffusely involving the liver
tern is an unusual feature of angiosar- parenchyma along the portal tracts without forming macronodular lesions.
coma (18).
Since angiosarcoma commonly ap-
pears as multiple masses or as a heteroge-
neous dominant mass, it often cannot be abnormalities, including thrombocytope- MR images can demonstrate its hemor-
readily distinguished from hypervascular nia, disseminated intravascular coagula- rhagic and heterogeneous appearance.
metastases (such as neuroendocrine tu- tion, and hemolytic anemia, are more fre- Dynamic enhancement of a dominant
mors) and hepatocellular carcinoma. All quently associated with angiosarcoma. mass at CT or MR imaging may show
of these tumors may demonstrate in- Our study had several weaknesses— heterogeneous enhancement on the early-
ternal hemorrhage and heterogeneity, in predominantly, small sample size and phase images and progressive enhance-
addition to early and heterogeneous lack of consistent imaging parameters, ment on delayed images. T2-weighted
enhancement (22,23). In contrast to hep- due to the rarity of the tumor and the MR images demonstrate a correspond-
atocellular carcinoma, however, angio- length of time in which cases were col- ing heterogeneous or compartmental-
sarcoma demonstrates continuing, pro- lected. Not all patients received intrave- ized appearance of the dominant mass.
gressive enhancement on delayed-phase nous contrast material or underwent When angiosarcoma appears as multi-
images. Splenic metastases and lack of dynamic imaging. Only two patients ple nodular lesions at CT, most lesions
cirrhosis may also suggest angiosarcoma underwent complete surgical resection, are hypoattenuating, and foci of en-
instead of hepatocellular carcinoma. which prohibited point-to-point radio- hancement may be present, but they are
Clinical findings may also help to distin- logic-pathologic correlation. Neverthe- clearly distinguishable from the nodular
guish angiosarcoma from other hypervas- less, we believe that some important enhancement in benign hemangiomas.
cular lesions. Hepatocellular carcinoma is and consistent patterns emerged. Such nodular enhancement may be less
usually associated with chronic hepatitis or Angiosarcoma has various appearances than that of the aorta. It is often bizarre
liver cirrhosis caused by chronic virus in- at CT or MR imaging that reflect its var- in shape, with ring enhancement seen in
fections, and it may be associated with el- ied histologic composition. When an an- some nodules. Splenic metastases are also
evated ␣-fetoprotein levels. Hematologic giosarcoma appears as a massive lesion, common with angiosarcoma. In a patient

672 䡠 Radiology 䡠 March 2002 Koyama et al


with a hypervascular and hemorrhagic osition and Thorotrast-induced angiosar- sis in comparison to matched control pa-
dominant mass or multiple liver lesions, coma of the liver. J Comput Assist To- tients with non-iron-related chronic liver
mogr 1983; 7:655– 658. disease. Hepatology 2001; 33:647– 651.
characteristic hematologic abnormalities 7. Vasile N, Larde D, Zafrani ES, Berard H, 16. Myoui A, Aozasa K, Iuchi K, et al. Soft
may suggest a diagnosis of primary he- Mathieu D. Hepatic angiosarcoma. J Com- tissue sarcoma of the pleural cavity. Can-
patic angiosarcoma. put Assist Tomogr 1983; 7:899–901. cer 1991; 68:1550 –1554.
8. Peterson MS, Baron RL, Rankin SC. He- 17. Drinkovic I, Brkljacic B. Two cases of le-
Acknowledgments: We thank professors Yuji patic angiosarcoma: findings on mul- thal complications following ultrasound-
Itai and David H. Stephens for their thoughtful tiphasic contrast-enhanced helical CT do guided percutaneous fine-needle biopsy
mentorship and encouragement of young radi- not mimic hepatic hemangioma. AJR of the liver. Cardiovasc Intervent Radiol
ologists, and for introducing the authors. We Am J Roentgenol 2000; 175:165–170. 1996; 19:360 –363.
also thank Dr Itai for stimulating our interest in 9. Rademaker J, Widjaja A, Galanski M. He- 18. Kojiro M, Nakashima T, Ito Y, Ikezaki H,
primary hepatic angiosarcoma with his insight- patic hemangiosarcoma: imaging find- Mori T, Kido C. Thorium dioxide-related
ful observations. ings and differential diagnosis. Eur Radiol angiosarcoma of the liver: pathomorpho-
2000; 10:129 –133. logic study of 29 autopsy cases. Arch
10. White PG, Adams H, Smith PM. The com- Pathol Lab Med 1985; 109:853– 857.
References puted tomographic appearances of angio- 19. Ludwig J, Hoffman HN. Hemangiosar-
1. Alrenga DP. Primary angiosarcoma of the sarcoma of the liver. Clin Radiol 1993; coma of the liver. Spectrum of morpho-
liver: review article. Int Surg 1975; 60: 48:321–325. logic changes and clinical findings. Mayo
198 –203. 11. Itai Y, Teraoka T. Angiosarcoma of the Clin Proc 1975; 50:255–263.
2. Ishak KG. Mesenchymal tumor of the liver mimicking cavernous hemangioma 20. Ohtomo K, Araki T, Itai Y, et al. MR im-
liver. In: Okuda K, Peters RL, eds. Hepa- on dynamic CT. J Comput Assist Tomogr aging of malignant mesenchymal tumors
tocellular carcinoma. New York, NY: 1989; 13:910 –912. of the liver. Gastrointest Radiol 1992; 17:
Wiley, 1976; 247–308. 12. Ohtomo K, Araki T, Itai Y, et al. MR im- 58 – 62.
3. Locker GY, Doroshow JH, Zwelling LA, aging of malignant mesenchymal tumors 21. Ros PR, Lubbers PR, Olmsted WW, Mo-
Chabner BA. The clinical features of he- of the liver. Gastrointest Radiol 1992; 17: rillo G. Hemangioma of the liver: hetero-
patic angiosarcoma: a report of four cases 58 – 62. geneous appearance on T2-weighted im-
and a review of the English literature. 13. Worawattanakul S, Semelka RC, Kelekis ages. AJR Am J Roentgenol 1987; 149:
Medicine 1979; 58:48 – 64. NL, Woosley JT. Angiosarcoma of the liv- 1167–1170.
4. Buetow PC, Buck JL, Ros PR, Goodman er: MR imaging pre- and post-chemother- 22. Onaya H, Itai Y. MR imaging of hepato-
ZD. Malignant vascular tumors of the liv- apy. Magn Reson Imaging 1997; 15:613– cellular carcinoma. Magn Reson Imaging
er: radiologic-pathologic correlation. Ra- 617. Clin N Am 2000; 8:757–768.
dioGraphics 1994; 14:153–156. 14. Falk H, Thomas LB, Popper H, Ishak KG. 23. Stevens WR, Gulino SP, Batts KP, Ste-
5. Levy DW, Rindsberg S, Friedman AC, et al. Hepatic angiosarcoma associated with phens DH, Johnson CD. Mosaic pattern
Thorotrast-induced hepatosplenic neopla- androgenic-anabolic steroids. Lancet 1979; of hepatocellular carcinoma: histologic
sia: CT identification. AJR Am J Roentgenol 2:1120–1123. basis for a characteristic CT appearance.
1986; 146:997–1004. 15. Fracanzani AL, Conte D, Fraquelli M, et J Comput Assist Tomogr 1996; 20:337–
6. Silverman PM, Ram PC, Korobkin M. CT al. Increased cancer risk in a cohort of 230 342.
appearance of abdominal thorotrast dep- patients with hereditary hemochromato-

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