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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