AJR 2005; 184:1578-1580
© American Roentgen Ray Society
Delayed Enhancement Pattern in a Localized Fibrous Tumor of the Liver
Thomas Moser1,
Tereza S. Nogueira1,
Agnès Neuville2,
Sophie Riehm1,
Gerlinde Averous2,
Jean-Christophe Weber3 and
Francis Veillon1
1 Department of Radiology, Hôpital de Hautepierre, CHU Strasbourg, Avenue
Molière, Strasbourg 67000, France.
2 Department of Pathology, Hôpital de Hautepierre, Strasbourg 67000,
France.
3 Department of Digestive Surgery, Hôpital de Hautepierre, Strasbourg
67000, France.
Received February 3, 2004;
accepted after revision July 22, 2004.
Address correspondence to T. Moser
(moser_th{at}yahoo.fr).
Introduction
Originally described as pleural neoplasms, localized fibrous tumors are
currently known to occur in numerous different locations, including the liver
[1,
2]. Localized fibrous tumors
are mesenchymal tumors composed of spindle cells interspersed with collagen
bundles and containing a variable amount of vessels. Definitive diagnosis
should be made only after immunohistochemistry, with CD34 being the most
consistently positive marker
[1–6].
Hepatic localized fibrous tumors are exceedingly rare, with all
descriptions based on one or a few case reports that are not amenable to
statistical analysis
[3–5].
Fuksbrumer et al. [3] published
the most extensive imaging description of hepatic localized fibrous tumors, to
our knowledge. In their three-case series, hepatic localized fibrous tumor was
characterized as a large solitary lesion appearing heterogeneous on
sonography, CT, and MRI.
We present a case of localized fibrous tumor occurring in the liver with
radiologic–pathologic correlation and emphasis on the delayed
enhancement of the tumor on CT and MRI.
Case Report
A 73-year-old woman was brought to the emergency service of our institution
in hypoglycemic coma. She was neither diabetic nor under hypoglycemic
medications.
On admission, blood glucose level was 0.22 g/L (normal range,
0.70–1.1 g/L). Physical examination revealed a huge firm and rubbery
mass in the right upper abdominal quadrant. Results of liver function tests
were unremarkable. Blood insulin, C peptide, insulin-like growth factor 1,
growth hormone, and cortisol were all within normal ranges.
During hospitalization, glycemia was kept to normal through continuous IV
infusion of 10% glucose solution and subcutaneous administration of
glucagon.
Abdominal sonography (Sonoline Elegra, Siemens) confirmed the presence of a
huge mass in the right upper quadrant. It was hyperechoic to the liver
parenchyma and contained numerous cystic foci.
Helical CT images (ProSpeed SX Advantage, GE Healthcare) were obtained
before (Fig. 1A) and 30 sec
(Fig. 1B), 1 min
(Fig. 1C), and five min
(Fig. 1D) after IV contrast
injection. These images showed a well-demarcated, hypodense lesion containing
numerous prominent cystic areas. Portions of the lesion enhanced faintly
during both the arterial (30 sec) and portal (1 min) phases. However, marked
contrast enhancement was seen 5 min after injection. Cystic areas remained
unchanged throughout the whole examination. The adjacent right liver lobe was
displaced to the left mimicking an extrahepatic origin. However, a single tiny
area of the tumor appeared in continuity with segment VII, which favored the
hypothesis of a pedunculated hepatic mass.
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Fig. 1A. —73-year-old woman with hepatic localized fibrous tumor. Axial
CT scan obtained through upper liver without IV contrast injection shows large
well-limited hypodense mass (arrow) exophytic from right liver and
containing numerous cystic areas.
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Fig. 1B. —73-year-old woman with hepatic localized fibrous tumor. Axial
CT scan obtained at same level as A 30 sec after IV contrast injection
(arterial phase) discloses faint enhancement of lesion (arrow).
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Fig. 1C. —73-year-old woman with hepatic localized fibrous tumor. Axial
CT scan obtained at same level as A 1 min after IV contrast injection
(portal phase) discloses faint enhancement of lesion (arrow), which
remains hypodense to liver.
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Fig. 1D. —73-year-old woman with hepatic localized fibrous tumor. Axial
CT scan obtained at same level as A 5 min after IV contrast injection
(delayed phase) shows marked enhancement of lesion, and only cystic areas
remain hypodense to liver.
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Preoperative workup also included MRI, with MR venography of the inferior
vena cava on a 1.5-T scan (Magneton, Siemens). Spoiled gradient-echo
T1-weighted sequences with fat suppression were obtained 30 sec, 1 min, and 8
min (Fig. 1E) after injection
of gadolinium chelates. In addition, to rule out inferior cava vein
obstruction, which would have influenced the surgical procedure, we obtained
multiplanar images; these images reinforced the hypothesis of a pedunculated
liver mass. Furthermore, the delayed enhancement pattern seen on CT was more
conspicuously reproduced.
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Fig. 1E. —73-year-old woman with hepatic localized fibrous tumor.
Sagittal gradient-echo fat-suppressed T1-weighted MR image (TR/TE, 156/2.3;
alpha, 30°) obtained 8 min after IV gadolinium chelates injection shows
intense enhancement of lesion (arrow) during delayed phase.
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The aforementioned clinical and imaging data suggested a mesenchymal
hepatic tumor, and the hypothesis of a fibrosarcoma was raised.
The patient underwent surgery, and the tumor was removed together with part
of the segment VII. The hepatic origin was then confirmed, and involvement of
other abdominal structures was ruled out. The lesion measured 35 x 20
x 15 cm, weighed 3,900 g, and was partially surrounded by the liver
capsule. On cut section, it was whitish and contained numerous cystic
foci.
On histology (Fig. 1F), the
tumor was found to be a mesenchymal tumor composed of spindle cells disposed
haphazardly and interspersed with collagen bundles. No conspicuous vessels
were found throughout the tumor. Immunohistochemistry was positive for
vimentin, bcl-2, and CD34 and was negative for vascular (CD31, factor
VIII), epithelial (keratin, epithelial membrane antigen), nervous (S-100
protein), and muscular (desmin and 
-actin) markers. Proliferation index
(MIB-1 cell proliferation marker) was low, ranging from 1% to 2%. Altogether,
these features were diagnostic of a localized fibrous tumor of the liver
[1–6].
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Fig. 1F. —73-year-old woman with hepatic localized fibrous tumor.
Photomicrograph of histologic specimen shows spindle cells interspersed with
collagen bundles in haphazard pattern without conspicuous vascular structures.
(H and E, x200)
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Immediate follow-up after surgery was uneventful, and glycemia returned to
normal range levels.
Discussion
Localized fibrous tumors occur more frequently in the pleura. However, even
at this site it is an uncommon neoplasm, representing less than 5% of all
pleural tumors. Extrapleural localized fibrous tumors have been described
almost everywhere, including the liver, and their morphologic features greatly
mirror those of their pleural counterpart, which is consequently considered as
the prototype of this entity
[1–6].
Abdominal localized fibrous tumors generally present with vague abdominal
complaints or even mild liver function test abnormalities
[3–5].
Some patients may even be asymptomatic and the tumor incidentally discovered.
Hypoglycemia has been described as a paraneoplastic manifestation for intra-
and extraabdominal localized fibrous tumors and can manifest as an inaugural
coma as reported here and also by Chithriki et al.
[7]. Its mechanism may involve
excessive glucose consumption by the tumor, overexpression of insulin
receptors, or tumoral production of hypoglycemic factors
[1–7].
In our patient, blood insulin and hypoglycemic factor levels were within
normal ranges, which favors excessive consumption.
The few reported hepatic localized fibrous tumors reproduced the classic
histologic pattern described for the pleural variant
[1–5].
It consists of spindle cells tightly packed together and intermingled with
collagen fascicles. Most commonly, the cells are disposed haphazardly in an
arrangement known as the "patternless" pattern. Furthermore, a
varying cellularity is characteristic: It means there are alternating hyper-
and hypocellular areas in the same tumor. Staghorn branching and dilated
vessels are seen admixed with the cellular proliferation, an aspect known as
the hemangiopericytoma-like vascular pattern
[1–6].
The latter is rather frequent and was seen in all nine cases reported by Moran
et al. [4], who described the
largest series of hepatic localized fibrous tumor to date. However, the
hemangiopericytoma-like vascular pattern was absent in the present case.
Rather, it was a poorly vascularized tumor with an interstitium that was
almost exclusively composed by collagen fibers.
Previously reported hepatic and extrahepatic localized fibrous tumors
showed early contrast uptake that was correlated with the prominent vascular
structures
[1–6].
Fuksbrumer et al. [3] found
heterogeneous enhancement with areas of differential uptake and washout, which
they believed were possibly related to the varying cellularity. By contrast,
the present case showed—on both CT and MRI—a progressive
enhancement during the arterial and portal phases that became marked on
delayed images. As previously stated, our case lacked the prominent vessels.
Its enhancement dynamic is in accordance with a collagen-rich interstitium
with a poor vascular network, as has already been shown in the literature for
a wide range of fiber-containing lesions
[8].
Hepatic localized fibrous tumor should be included in the differential
diagnosis of other rare mesenchymal tumors occurring in the liver. When poorly
vascularized, as in the present case, localized fibrous tumor may be confused
with fibrous tissue–containing sarcomas such as fibrosarcoma and
malignant fibrous histiocytoma
[1,
9]. Hepatic fibrosarcoma is a
rare neoplasm that may attain large size and can also be revealed by
hypoglycemia [9]. It is a
spindle cell tumor with a collagen-rich stroma that bears closest morphologic
and microscopic resemblance to the localized fibrous tumor variant presented
herein. However, the lack of herringbone cellular arrangement and the
positivity for CD34 seen in our case precluded the diagnosis of fibrosarcoma
[9].
In conclusion, localized fibrous tumor is a rare but well-established
entity that should be considered in the differential diagnosis for a large,
well-demarcated hepatic mass, particularly if clinical manifestations of
hypoglycemia are associated. Visualization of progressive contrast enhancement
is particularly useful to assess the presence of fibrous tissue, and delayed
images should be included in the imaging protocol of such cases.
Acknowledgments
We thank A. Mataoanu for her editorial assistance.
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Introduction
Case Report
