DOI:10.2214/AJR.05.0167
AJR 2006; 187:W43-W52
© American Roentgen Ray Society
Peliosis Hepatis: Spectrum of Imaging Findings
Riccardo Iannaccone1,2,
Michael P. Federle3,
Giuseppe Brancatelli4,5,
Osamu Matsui6,
Elliot K. Fishman7,
Vamsidar R. Narra8,
Luigi Grazioli9,
Shirley M. McCarthy10,
Francesca Piacentini2,
Luigi Maruzzelli2,
Roberto Passariello1 and
Valerie Vilgrain2
1 Department of Radiological Sciences, University of Rome, La Sapienza, 324
Viale Regina Elena, Rome, Italy 00161.
2 Hôpital Beaujon, Clichy, France.
3 University of Pittsburgh Medical Center, Pittsburgh, PA.
4 Istituto di Scienze Radiologiche, Università di Palermo, Palermo,
Italy.
5 Ospedale Specializzato in Gastroenterologia "Saverio de Bellis,"
IRCCS, Bari, Italy.
6 Kanazawa University Postgraduate School of Medical Science, Kanazawa,
Japan.
7 Johns Hopkins University, Baltimore, MD.
8 Washington University, St. Louis, MO.
9 University of Brescia, Brescia, Italy.
10 Yale Diagnostic Radiology, New Haven, CT.
Received February 1, 2005;
accepted after revision April 20, 2005.
Francesca Piacentini supported by a research grant from Università
Cattolica del Sacro Cuore ("Working Experience Abroad," WEA).
Address correspondence to R. Iannaccone
(r_iannaccone{at}yahoo.it).
WEB
This is a Web exclusive article.
Abstract
OBJECTIVE. It is important to recognize the imaging characteristics
of peliosis hepatis because peliotic lesions may mimic several different types
of focal hepatic lesions
CONCLUSION. We illustrate the spectrum of imaging findings of
peliosis hepatis, including sonography, CT, MR, and angiography.
Keywords: CT imaging • liver • liver disease • MRI
Introduction
Peliosis hepatis (also called hepatic peliosis) is a rare benign disorder
causing sinusoidal dilatation and the presence of multiple blood-filled
lacunar spaces within the liver
[1]. "Peliosis" is
a term derived from the Greek pelios, which means "dusky"
or "purple," referring to the color of the liver parenchyma with
peliosis. Similar blood-filled spaces may be seen in the spleen, lymph nodes,
and other organs (including the bone marrow, lungs, pleura, kidneys, adrenal
glands, stomach, and ileum)
[2]. The size of the lesions
may vary from 1 mm to several centimeters.
Cause and Pathogenesis
The cause of peliosis hepatis can be related to drugs (including anabolic
steroids, oral contraceptives, corticosteroids, tamoxifen, diethylstilbestrol,
azathioprine, 6-thioguanine, 6-mercaptopurine, and methotrexate); toxins
(polyvinyl chloride, arsenic, and thorium oxide); chronic wasting diseases
(e.g., tuberculosis, leprosy, and various malignancies, particularly
hepatocellular carcinoma); and infection in AIDS (so-called bacillary peliosis
caused by Bartonella henselae and Bartonella quintana). In
addition, several other conditions are described as associated with peliosis
hepatis, including sprue, diabetes mellitus, necrotizing vasculitis, and
hematologic disorders. Moreover, peliosis hepatis may develop after renal or
cardiac transplantation. In 20-50% of patients, no associated condition is
identified.
The pathogenesis of peliosis remains poorly understood, with various
investigators proposing that the primary event could be obstruction of hepatic
outflow at the sinusoidal level, direct breakdown of sinusoidal borders,
dilatation of the central vein of the hepatic lobule, or hepatocellular
necrosis leading to cavity formation
[1].
Pathology
The gross pathologic appearance of peliosis hepatis is that of multiple,
irregularly shaped blood-filled hepatic cavities. The lesions typically
involve the entire liver, but focal peliosis hepatis has been described. At
microscopic examination, cystic dilated sinusoids filled with RBCs and bound
by cords of liver cells can be seen (Figs.
1A,
1B,
1C,
1D,
1E,
1F, and
1G).
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Fig. 1A —51-year-old woman with history of benign ovarian tumor and
incidentally discovered hepatic mass. Transverse unenhanced CT image shows
hypoattenuating lesion (arrow) within segment VIII of liver.
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Fig. 1B —51-year-old woman with history of benign ovarian tumor and
incidentally discovered hepatic mass. On contrast-enhanced CT during hepatic
arterial phase, lesion (arrow) shows marked homogeneous contrast
enhancement.
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Fig. 1C —51-year-old woman with history of benign ovarian tumor and
incidentally discovered hepatic mass. On contrast-enhanced CT during portal
venous phase, lesion (arrow) shows washout of contrast but is still
hyperattenuating compared with liver parenchyma. Overall, lesion has
vessel-like enhancement at CT.
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Fig. 1E —51-year-old woman with history of benign ovarian tumor and
incidentally discovered hepatic mass. On T2-weighted MR sequence, lesion
(arrow) is faintly hyperintense compared with liver parenchyma.
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Fig. 1F —51-year-old woman with history of benign ovarian tumor and
incidentally discovered hepatic mass. On T1-weighted MR sequence, lesion
(arrow) is hypointense compared with liver parenchyma.
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Pathologists originally classified peliotic lesions by the presence or
absence of endothelium [1].
However, although the blood-filled cavities may have no endothelial lining,
reendothelialization probably occurs rapidly. Thus, the continuity/rupture of
the endothelial lining of the sinusoids is not a reliable criterion to define
peliosis hepatis [2]. To
distinguish peliosis hepatis from sinusoidal dilatation, lesions should show
evidence of rupture of the reticulin fibers that support the hepatocytes and
sinusoids [1]. This rupture may
follow the intrinsic weakness of the fibers of the endothelial wall (peliosis
hepatis of the phlebectatic type) or may be associated with focal hepatocyte
necrosis (peliosis hepatis of the parenchymal type)
[1].
Peliotic lesions in bacillary peliosis contain clumps of organisms (i.e.,
B. henselae or B. quintana) that stain with the
Warthin-Starry technique [1].
Patients with bacillary peliosis often have peliosis of the spleen and lymph
nodes and cutaneous angiomatous lesions.
Clinical Presentation
Peliosis hepatis is often asymptomatic and therefore is diagnosed
incidentally at autopsy [3]. In
some instances, hepatomegaly, ascites, portal hypertension, cholestasis, and
hepatic failure may be present. Severe abdominal pain may result from rupture
and intraperitoneal hemorrhage. In the case of bacillary peliosis,
lymphadenopathy with B. henselae and neurologic symptoms with B.
quintana are typical findings.
Peliosis hepatis can occur at any age. Although a fetal form exists,
peliosis hepatis usually develops in adults without regard to sex.
Natural History
The natural course of peliosis hepatis is regression after drug withdrawal,
cessation of steroid therapy, or resolution of an associated infectious
disease. A pseudotumoral and hemorrhagic evolution has also been described
[4,
5]. Complications associated
with peliosis hepatis include liver failure, portal hypertension, and liver
rupture leading to hemoperitoneum or shock. In general, if untreated, peliosis
hepatis may be rapidly fatal.
Imaging Findings
The imaging findings of peliosis hepatis are variable depending on the
pathologic patterns of disease, various stages of the blood component of the
lesions, and concomitant hepatic steatosis.
Sonographic Findings
Conventional gray-scale sonography shows homogeneous hypoechoic lesions in
patients with hepatic steatosis, hyper-echoic lesions in patients with a
healthy liver (Figs. 2A,
2B,
2C, and
2D), and heterogeneously
hypoechoic lesions if complicated by hemorrhage. Doppler studies can show
evidence of both perinodular and intranodular vascularity (Figs.
2A,
2B,
2C, and
2D).
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Fig. 2A —42-year-old woman with peliosis hepatis. Doppler sonographic
study shows 4-cm diameter slightly heterogeneously hyperechoic lesion within
healthy liver parenchyma. Notably, lesion has no mass effect on middle and
right hepatic veins.
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Fig. 2D —42-year-old woman with peliosis hepatis. In portal venous
phase, lesion enhancement is isodense to intrahepatic vessels. Note
centripetal progression of contrast enhancement, which simulates hemangioma.
Peliotic lesions have typically centrifugal progression of contrast
enhancement, but centripetal enhancement can also be observed.
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Recently, the use of a sonographic contrast agent (Levovist [Schering], a
galactose and palmitic acid compound) has been shown to provide a "fast
surge" central-echo enhancement in peliotic lesions
[6], a finding consistent with
the target sign described below.
CT Findings
On unenhanced CT, peliotic lesions usually appear as multiple areas of low
attenuation [3] (Figs.
3A,
3B,
3C, and
3D). CT findings vary with the
size of lesions, presence or absence of thrombus within the cavities, and
presence of hemorrhage. In particular, peliotic lesions may be spontaneously
hyperattenuating to liver parenchyma in certain patients (probably related to
intralesional hemorrhage). In addition, if peliotic cavities are smaller than
1 cm in diameter, CT findings may be normal
[2]. Calcifications within
peliotic lesions have also been described.
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Fig. 3A —4-year-old man with AIDS and bacillary peliosis. Transverse
contrast-enhanced CT image shows large ill-defined, hypoattenuating lesion
(white arrow) with heterogeneous peripheral enhancement within left
liver lobe. Smaller subcapsular hypoattenuating lesion (black arrow)
with ring enhancement can also be seen in right liver lobe.
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Fig. 3B —4-year-old man with AIDS and bacillary peliosis. Transverse
contrast-enhanced CT image (different scan level) shows multiple enlarged
lymph nodes, feature typically seen in bacillary peliosis.
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Fig. 3C —4-year-old man with AIDS and bacillary peliosis. Transverse
contrast-enhanced CT image obtained after 9 months shows progression of
disease with multiple hypoattenuating lesions disseminated within liver
parenchyma with multiple small accumulations of contrast material in center of
lesions (so-called target sign).
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On contrast-enhanced CT, peliotic lesions can be hypoattenuating to liver
parenchyma in the early acquisitions (Fig.
4) and tend to become progressively isoattenuating with time. In
addition, some lesions can also show areas of increased attenuation. Notably,
larger cavities communicating with sinusoids display the same attenuation of
blood vessels, whereas thrombosed cavities have the same appearance as
nonenhancing nodules [2]. More
often, during the arterial phase of contrast enhancement, peliotic lesions
typically show early globular enhancement (vessel-like enhancement) (Figs.
2A,
2B,
2C,
2D,
5A, and
5B) and multiple small
accumulations of contrast material in the center of the lesions (the so-called
target sign) [3] (Figs.
3A,
3B,
3C, and
3D). During the portal venous
phase, a centrifugal progression of enhancement without a mass effect on
hepatic vessels is usually observed
[3]; however, a centripetal
progression of enhancement can also be seen
[7] (Figs.
2A,
2B,
2C, and
2D). On the delayed phase,
late diffuse homogeneous hyperattenuation can also be seen in the phlebectatic
type of peliosis hepatis (because of the lack of hemorrhagic parenchymal
necrosis) [3]. This
accumulation of contrast material in the delayed phase can be useful in the
differential diagnosis with other focal hepatic lesions that do not show blood
pooling. In some instances, small (< 2 cm) peliotic lesions may also show
hyperattenuation on both arterial and portal venous phase images (Figs.
1A,
1B,
1C,
1D,
1E,
1F, and
1G).
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Fig. 4 —46-year-old woman with history of abdominal pain and peliosis
hepatis. Transverse contrast-enhanced CT image shows multiple hypoattenuating
lesions (arrowheads) disseminated within liver parenchyma. Multiple
similar lesions can also be seen within spleen (arrows).
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Fig. 5A —34-year-old woman with AIDS and bacillary peliosis.
Transverse contrast-enhanced CT image shows focal hyperattenuating lesion
(arrow) within caudate lobe. Lesion has central hypoattenuation.
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Fig. 5B —34-year-old woman with AIDS and bacillary peliosis.
Transverse contrast-enhanced CT image (different scan level) shows two
additional focal hyperattenuating lesions within left lobe (large
arrow) and right lobe (small arrow) of liver.
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MR Findings
The signal intensities of the lesions on MR examination largely depend on
the age and status of the blood component. On T2-weighted sequences, peliotic
lesions are usually hyperintense to liver parenchyma with multiple foci of
high signal, likely attributable to hemorrhagic necrosis
[3,
4] (Figs.
1A,
1B,
1C,
1D,
1E,
1F,
1G,
6A,
6B,
6C,
7A,
7B,
7C,
7D, and
7E). On T1-weighted sequences,
the lesions are hypointense because of the presence of subacute blood (Figs.
1A,
1B,
1C,
1D,
1E,
1F,
1G,
6A,
6B,
6C,
8A,
8B, and
8C), although isointense
(Figs. 7A,
7B,
7C,
7D, and
7E) and hyperintense foci are
also described in the literature
[5]. On T1-weighted images
after contrast material injection, peliotic lesions usually show enhancement
(Figs. 6A,
6B,
6C,
7A,
7B,
7C,
7D,
7E,
8A,
8B, and
8C). Similar to CT, the
enhancement is typically centrifugal (from the center to the periphery of the
lesion); however, a recent report described an unusual centripetal enhancement
pattern that may be confused with that of a hemangioma
[7]. Cystic cavities may reveal
an enhancing rim that represents a hematoma. In addition, on fat-suppressed
T1-weighted images in the delayed phase after administration of gadobenate
dimeglumine, strong contrast enhancement with a branching appearance can also
be observed because of the vascular component of the lesion
[4] (Figs.
8A,
8B, and
8C).
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Fig. 6A —57-year-old man affected by non-Hodgkin's lymphoma and
peliosis hepatis caused by multiple chemotherapy treatments. Transverse
T2-weighted MR image faintly depicts multiple ill-defined lesions
(arrowheads) with heterogeneous signal hyperintensity in both liver
lobes.
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Fig. 6B —57-year-old man affected by non-Hodgkin's lymphoma and
peliosis hepatis caused by multiple chemotherapy treatments. Transverse
T1-weighted MR image before contrast material injection faintly depicts
multiple ill-defined, hypointense lesions (arrowheads) in both liver
lobes.
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Fig. 6C —57-year-old man affected by non-Hodgkin's lymphoma and
peliosis hepatis caused by multiple chemotherapy treatments. On transverse
T1-weighted MR image obtained after contrast material administration, lesions
(arrowheads) show peripheral or complete contrast enhancement.
Percutaneous biopsy (not shown) at two different sites showed peliosis
hepatis. At follow-up after 1 year (not shown), lesions were still present but
slightly smaller.
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Fig. 7A —33-year-old woman with peliosis hepatis and history of oral
contraceptive use. Transverse fat-suppressed T2-weighted image shows
ill-defined, heterogeneously hyperintense lesion (arrow) within
segment VI of liver.
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Fig. 7B —33-year-old woman with peliosis hepatis and history of oral
contraceptive use. On transverse fat-suppressed T1-weighted image before
contrast material injection, lesion (arrow) is heterogeneously
hypointense to liver parenchyma.
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Fig. 7C —33-year-old woman with peliosis hepatis and history of oral
contraceptive use. After gadolinium injection, lesion (arrow) shows
progressive enhancement from arterial (C) to portal venous (D)
and delayed (E) phases, with "branching" appearance.
Because of progressive fill-in of contrast enhancement, findings were
initially thought to be consistent with hemangioma. Percutaneous biopsy of
lesion (not shown) was consistent with hepatocellular adenoma. However,
histologic examination performed on resected surgical specimen showed peliosis
hepatis. Centripetal enhancement in peliotic lesions is rather uncommon
finding; branching appearance of contrast enhancement in delayed phase is
useful sign that may help in diagnosis.
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Fig. 7D —33-year-old woman with peliosis hepatis and history of oral
contraceptive use. After gadolinium injection, lesion (arrow) shows
progressive enhancement from arterial (C) to portal venous (D)
and delayed (E) phases, with "branching" appearance.
Because of progressive fill-in of contrast enhancement, findings were
initially thought to be consistent with hemangioma. Percutaneous biopsy of
lesion (not shown) was consistent with hepatocellular adenoma. However,
histologic examination performed on resected surgical specimen showed peliosis
hepatis. Centripetal enhancement in peliotic lesions is rather uncommon
finding; branching appearance of contrast enhancement in delayed phase is
useful sign that may help in diagnosis.
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Fig. 7E —33-year-old woman with peliosis hepatis and history of oral
contraceptive use. After gadolinium injection, lesion (arrow) shows
progressive enhancement from arterial (C) to portal venous (D)
and delayed (E) phases, with "branching" appearance.
Because of progressive fill-in of contrast enhancement, findings were
initially thought to be consistent with hemangioma. Percutaneous biopsy of
lesion (not shown) was consistent with hepatocellular adenoma. However,
histologic examination performed on resected surgical specimen showed peliosis
hepatis. Centripetal enhancement in peliotic lesions is rather uncommon
finding; branching appearance of contrast enhancement in delayed phase is
useful sign that may help in diagnosis.
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Fig. 8A —45-year-old woman with right upper quadrant pain, history of
oral contraceptive use, and peliosis hepatis. Transverse fat-suppressed
T2-weighted MR image shows ill-defined mass with heterogeneous signal
hyperintensity in right liver lobe. Lesion with same characteristics is also
evident within left liver lobe.
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Fig. 8B —45-year-old woman with right upper quadrant pain, history of
oral contraceptive use, and peliosis hepatis. Transverse fat-suppressed
T1-weighted MR image before contrast material injection does not show any
significant abnormality of liver parenchyma.
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Fig. 8C —45-year-old woman with right upper quadrant pain, history of
oral contraceptive use, and peliosis hepatis. After contrast material
administration, transverse fat-suppressed T1-weighted MR image shows
heterogeneous contrast enhancement of lesions seen on T2-weighted
sequence.
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Angiographic Findings
On angiography, peliotic lesions appear as multiple vascular nodules (i.e.,
accumulations of contrast material) during the late arterial phase. The
enhancement of peliotic lesions typically is more distinct during the
parenchymal phase and persists during the portal venous phase
[8] (Figs.
1A,
1B,
1C,
1D,
1E,
1F, and
1G).
Differential Diagnosis
Hepatic Adenoma
Similar to peliosis, hepatic adenoma might also be associated with the
long-term use of estrogens. In the case of diffuse peliosis hepatis, the
differential diagnosis is relatively easy. In addition, the presence of fat in
some adenomas is a useful sign to make a differential diagnosis. In certain
instances, however, focal peliosis can be difficult to differentiate from
adenomas. In these patients, biopsy is often required to reach a definitive
diagnosis.
Hemangioma
The typical enhancement pattern of hemangiomas (i.e., peripheral ring or
globular enhancement with centripetal progression) is opposite of peliosis
hepatis, and therefore differential diagnosis can be achieved in most
patients. In addition, hemangiomas may be rather large lesions with a mass
effect on the hepatic vessels, whereas peliotic lesions usually show no mass
effect on hepatic vessels.
Focal Nodular Hyperplasia
Focal nodular hyperplasias are typically homogeneously hyperattenuating
masses on the arterial phase and isoattenuating on the portal venous and
delayed phases. These lesions often have a central scar with low attenuation
on the arterial and portal venous phases and enhancement on the delayed phase
images. When such typical imaging characteristics of focal nodular hyperplasia
are present, the differential diagnosis with peliosis hepatis can be achieved
easily. Atypical forms of focal nodular hyperplasia may not show the
characteristic enhancement patterns and the central scar just described,
however, and thus pose some problems in the differential diagnosis with
peliosis hepatis.
Hepatic Abscess
The differential diagnosis between peliosis hepatis and hepatic abscess is
extremely important to avoid the percutaneous drainage of peliotic lesions,
which can be dangerous and even fatal
[9]. With regard to imaging
criteria, a pyogenic abscess usually presents as a mass with a multiseptated
or cluster-of-grapes appearance with nonenhancing contents.
Hypervascular Metastases
Although some hypervascular metastases with fibrotic change can show mild
hyperattenuation in the delayed phase, hypervascular metastases are usually
totally hypo- or isoattenuating in the delayed phase of contrast enhancement
because of the rapid washout of contrast material. Thus, in general, peliotic
lesions are rarely confused with hypervascular metastases.
Hepatocellular Carcinoma
Hepatocellular carcinoma is usually hyperattenuating in the arterial phase
with rapid washout in the portal venous phase and iso- or hypoattenuation in
the delayed phase. Although rare, the possibility that peliosis hepatis may
mimic the presence of hypervascular hepatocellular carcinoma has been reported
in the literature. In these patients, biopsy is often necessary to reach a
definitive diagnosis.
Treatment
The correct diagnosis of peliosis hepatis is important because withdrawal
of the offending drug or toxin can resolve the disease and prevent serious
complications such as hepatic failure or death related to intraabdominal
hemorrhage. Because of its potential complications, surgical resection of the
involved liver parenchyma should always be considered. In HIV-related peliosis
hepatis caused by B. henselae, clinical improvement has been
documented with the use of antibiotics (i.e., erythromycin).
In conclusion, peliosis hepatis is a rare clinical entity characterized by
the presence of multiple blood-filled lacunar spaces within the liver.
Awareness of the imaging findings of this disorder is important to suggest the
diagnosis. Peliosis hepatis should always be considered in the differential
diagnosis of atypical focal hepatic lesions in patients with the clinical
conditions described earlier.
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Abstract
Introduction
