AJR 2004; 183:171-173
© American Roentgen Ray Society
MRI of Hepatic Sarcoidosis: Large Confluent Lesions Mimicking Malignancy
Gregor Jung1,
Nicole Brill2,
Ludger Wilhelm Poll1,
Jens Albrecht Koch1 and
Mathias Wettstein2
1 Institute for Diagnostic Radiology, Heinrich Heine University Düsseldorf,
Moorenstraße 5, Düsseldorf 40225, Germany.
2 Department of Hepatology, Gastroenterology and Infectious Diseases, Heinrich
Heine University Düsseldorf, Düsseldorf 40225, Germany.
Received September 2, 2003;
accepted after revision November 12, 2003.
Address correspondence to G. Jung.
Introduction
Sarcoidosis is a multisystemic granulomatous disease of unknown origin that
typically affects the thoracic lymph nodes and the lungs
[1]. The skin, eyes, liver, and
spleen can also be involved. Liver biopsy reveals granulomatous involvement in
40–70% of cases
[1–3].
CT generally shows an apparently normal liver or homogeneous hepatomegaly.
Small nodular lesions in the liver and spleen have also been described as
manifestations of sarcoidosis seen on CT and MRI
[4].
We describe the course of a histologically confirmed case of sarcoidosis
observed on MRI for 1 year. To our knowledge, large (> 4.5 cm) confluent
lesions have not been previously reported as a manifestation of sarcoidosis in
the liver. We present the diagnostic characteristics and the differential
diagnosis.
Case Report
A 63-year-old woman was referred to the hepatology department for
assessment of liver lesions that had recently appeared. According to the
patient's history, she had been complaining for approximately 3 months of
physical exhaustion, recurrent bouts of fever, and dryness of the eyes. In
addition, cutaneous sarcoidosis had been confirmed in a subcutaneous nodule on
her right upper arm.
She had multiple subcutaneous nodules on both upper arms, but physical
examination was otherwise unremarkable. Laboratory tests showed calcium and
-glutamyl transpeptidase values outside the normal range, with calcium
concentration at 2.81 mmol/L (range of reference values, 2.15–2.55
mmol/L) and -glutamyl transpeptidase concentration at 28 U/L (reference
value, < 18 U/L). The angiotensin-converting enzyme concentration was also
clearly elevated at 93 U/L (range of reference values, 18–60 U/L).
CT of the chest and abdomen showed enlarged lymph nodes in the hilar region
of both lungs, with a maximum diameter of 1.5 cm; smaller nonenlarged lymph
nodes in the mediastinum; and unremarkable abdominal lymph nodes. Multiple
hypodense lesions were observed in the liver that showed homogeneous contrast
enhancement in the portal venous perfusion phase (Figs.
1A and
1B).
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Fig. 1A. —63-year-old woman with focal liver lesions in sarcoidosis.
Arterial phase CT scan shows lesions slightly hypodense relative to
surrounding tissue in right liver lobe. Branches of right hepatic artery are
not occluded or displaced.
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Fig. 1B. —63-year-old woman with focal liver lesions in sarcoidosis.
Portal venous phase CT scan depicts lesions more clearly. Branches of right
portal vein are not affected by sarcoidosis lesions.
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MRI of the liver showed multiple confluent lesions, particularly in the
right lobes, with a maximum diameter of 6 cm (Figs.
1C,
1D,
1E). Some individually
demarcated lesions had diameters as large as 4.5 cm. The most striking
morphologic characteristic was the completely intact vascular architecture in
the region of the lesions and the perivascular location of the lesions around
the portal veins. Sonographically guided liver biopsy using a 16-gauge Tru-Cut
needle biopsy system (Baxter Healthcare) showed typical sarcoidosis with
granulomatous epithelioid cell inflammation and no evidence of caseating
granuloma or acid-fast bacilli.
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Fig. 1D. —63-year-old woman with focal liver lesions in sarcoidosis.
T2-weighted turbo spin-echo MR image (2,500/90) shows lesions slightly
hyperintense relative to surrounding hepatic parenchyma.
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Fig. 1E. —63-year-old woman with focal liver lesions in sarcoidosis.
Superparamagnetic iron oxide–enhanced T2-weighted MR image (2,500/90)
shows increase of contrast between lesions and liver tissue indicating absence
of reticuloendothelial system cells in lesions.
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Treatment was begun with 40 mg of prednisolone by mouth. A follow-up
examination 4 months later showed that the lesions had been reduced to a
minimal perivascular remnant. Similarly, the angiotensin-converting enzyme
level had completely normalized and remained in the normal range during the
next year. MRI examination 12 months later showed healthy liver
(Fig. 1F).
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Fig. 1F. —63-year-old woman with focal liver lesions in sarcoidosis.
T1-weighted gradient-echo MR image obtained during follow-up 12 months after
E shows normal liver tissue after treatment with prednisolone.
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Discussion
Sarcoidosis is a systemic disease characterized histopathologically by
noncaseating granulomas [1].
Although biopsy shows involvement of the liver in as many as 70% of cases,
Warshauer et al. [5] found in a
CT study of 59 patients with confirmed sarcoidosis that hepatomegaly was
present in 29% of cases, but focal lesions could only be seen in 5%. When
focal involvement of the liver can be shown, the typical CT appearance is that
of multiple small hypodense nodular lesions
[3–6].
In five patients who were examined on both CT and MRI, the size of the focal
liver lesions was between 0.5 and 1.5 cm
[4]. In another CT study of 32
patients with nodular sarcoidosis of the liver and spleen, the mean size and
standard deviation of the liver lesions measured was 0.6 ± 0.4 cm
(range, 0.2–1.9 cm)
[7].
In our patient, confluent lesions as long as 6 cm were found in the liver.
Histologically, the diameter of granulomatous lesions in the liver is
generally less than 2 mm [6].
Warshauer et al. [5] postulated
that the appearance of the larger lesions was caused by a coalescence of small
granulomas, although the reason for this aggregation remains unclear. It is
also unclear why some patients with marked hepatosplenomegaly appear to have
no lesions, but nodular lesions can be seen in other patients who have only
slight enlargement of the liver and spleen. Differences in immunologic
reactions have been suggested as a possible reason for the difference.
On the basis of the unusual size of the lesions in our patient, the
differential diagnosis would suggest metastases and include lymphoma,
tuberculosis, or an atypical myobacterial infection. The decisive morphologic
characteristic with respect to the differential diagnosis was the intact
vascular architecture around the lesions. We would have expected occlusion or
displacement of the blood vessels near these space-occupying lesions if they
were malignant.
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