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JDMXXX10.1177/8756479312461303Journ
Case Studies
Abstract
Focal nodular hyperplasia (FNH) is the second most common benign hepatic mass. The primary purpose of this article
is to provide a relevant case study and offer a review of the recent literature related to FNH with a discussion of
both the clinical and imaging findings of this classically benign hepatic lesion. Although magnetic resonance imaging is
considered the gold standard of FNH imaging, the sonographic and computed tomography appearance also is offered in
this article. Furthermore, contrast-enhanced ultrasound, which boasts a 96% success rate at differentiating FNH from
other hepatic tumors, is analyzed.The historical treatment options, including medical and possible surgical intervention,
are provided as well. Last, this article offers an analysis of the prognosis for the patient diagnosed with FNH.
Keywords
focal nodular hyperplasia, sonography, central stellate scar, liver lesions, benign hepatic mass, contrast-enhanced
ultrasound
Case Presentation
A young white woman in her early 20s entered the sonography program and was offered an educational assignment that included the sonographic evaluation of her liver.
Incidentally, during a practice scanning laboratory session, a liver mass was recognized using an ATL HDI 5000
(Philips/ATL, Philips Healthcare, Andover, Massachusetts)
C5-2 transducer. The lesion was well marginated, hyperechoic, and positioned adjacent to the inferior vena cava
within the right lobe of the liver (Figures 1 and 2). The
mass did appear to distort the hepatic architecture in a
manner that was inconsistent with a cavernous hemangioma. Color Doppler revealed blood flow around and
within the mass, thus providing another sonographic
feature that was thought to be less likely indicative of a
Corresponding Author:
Steven M. Penny, BS, RT(R), RDMS, Johnston Community College
Imaging, PO Box 2350, Smithfield, NC 27577, USA
Email: smpenny@johnstoncc.edu
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Discussion
FNH is one of the most common benign hepatic masses.
Its incidence is second only to the frequently encountered
cavernous hemangioma of the liver.1,2 FNH was originally
mentioned in the literature by Edmundson in 1958 and
officially recognized as a distinct hepatic lesion by the
World Health Organization in 1975.3,4 Prior to this
acknowledgment, FNH may have been misdiagnosed as a
hepatic adenoma, also termed focal nodular cirrhosis or
benign hepatoma.5 Nonetheless, with more than a halfcentury of research, there still appears to be much conjecture and ongoing exploration into the etiology of the mass
frequently portrayed as the stealth lesion of the liver.
Composition of FNH
Differing studies have mentioned FNH as either a lesion
that is neoplastic in nature, consisting of polyclonal cells,
20
or nonneoplastic, consisting of monoclonal cells. Lesions
that are polyclonal consist of different types of cells,
whereas monoclonal lesions are composed of similar
cells. The determination as to whether FNH is a true neoplasm is significant, however, specifically because a differentiation usually needs to be made between FNH and
other hepatic masses, as prognosis and follow-up can vary
considerably. A recent study by Gong et al4 in 2009 established that FNH is indeed more likely a mass consisting
of proliferated polyclonal cells and that it is nonneoplastic in nature, thus confirming that FNH is a hyperplastic
rather than a neoplastic process.6
Histologically, FNH consists of hyperplastic units of
hepatocytes fixed together in an abnormal arrangement
with dense fibrous tissue.2,7 The mass also contains proliferating bile ducts, Kupffer cells, connective tissue, and a
central stellate (an arrangement resembling that of a radiating pattern, like that of a star) scar.7,8 The stellate scar
contains a large artery that courses within it, causing
hyperperfusion and arterializations of sinusoids.5 Radiating
out from the scar are often dense fibrous septa, often in a
spoke-wheel pattern toward the periphery.6,9,10 Surrounding
the FNH is a pseudo-capsule, although with imaging, the
pseudo-capsule often appears as a genuine capsule surrounding the FNH because of the compression of adjacent liver parenchyma.10
The average size of an FNH at the time of discovery is
approximately 6 cm.11 Three criteria typically have to be
recognized to differentiate FNH from other hepatic masses
microscopically: (1) the existence of a central stellate scar
within the mass, (2) the presence of multiple bile ducts in
fibrous intervals, and (3) a mass that lacks small cell
changes.12 The appreciation of this latter characteristic is
crucial, for small cell change is a fundamental step in
hepatocarcinogenesis. Moreover, it is significant to note
that the central scar can be absent in anywhere between
20% and 50% of FNH cases.4,11
Etiology of FNH
The etiology of FNH has been an area of concentrated
research. FNH has a low occurrence rate in males but is
often discovered incidentally in females of childbearing
age, which correlates with the case study discussed in
this article.12,13 These findings support a theory that
proposes that the mass is the outcome of an estrogenstimulating vascular alteration combined with hepatocyte
hyperplasia.4 The role that estrogen, and specifically the
use of oral contraceptives (OCs), plays in the development of FNH has not been clearly established. It should
be noted that FNH has been found in children, males, and
females who are not taking OCs.14 One small study
revealed no change in the character of tumors on followup imaging in patients who continued to take OCs.12 It
21
For these instances, the use of B-color to highlight the
borders of the mass can be beneficial, and a vigilant
analysis of contour changes within the liver will help
identify these masses.
Within the tumor, sonography may demonstrate the
presence of the aforementioned central scar. This scar
may appear hyperechoic or hypoechoic and contain
prominent vasculature. Color Doppler may demonstrate
evidence of vascularity surrounding the mass.23 Although
not yet approved by the US Food and Drug Administration
(FDA) for this application, contrast-enhanced ultrasound
(CEUS) is a dependable modality for the evaluation of
focal liver lesions, with a 96% success rate at differentiating FNH from the hepatic adenoma.11,24 An FNH under
CEUS interrogation will yield hypervascularity on the
arterial phase and demonstrate the presence of stellate
lesional vessels and a tortoise feeding artery.1 Germany
and France have both participated in well-known multicenter studies involving focal liver lesions and CEUS.
The German study consisted of 1349 patients with focal
liver lesions, and although the CEUS study was compared primarily with biopsy, some cases were compared
with computed tomography (CT) or MRI. The study presented an accuracy rate of 90.3%, a sensitivity rate of
95.8%, and a specificity rate of 83.1%.24 In this particular study, CEUS correctly diagnosed 87.1% of FNHs.24
The French study consisted of 1034 focal liver lesions,
and CEUS provided an accuracy rate of 86.1% compared
with standard ultrasound, which had an accuracy of only
62.4%.
CT and MRI are especially useful in differentiating
FNH from other hepatic tumors and are typically used
subsequent to the incidental identification of FNH during a sonographic study. On an unenhanced CT, FNH
will appear as a hypoattenuating or isoattenuating mass,
with about one-third of the cases presenting with a
prominent hypoattenuating central scar.9,16 Contrastenhanced CT findings typically show a mass that is
homogeneous, with some enhancement of the scar noted
during the arterial phase and possibly on delayed images
as well.9
MRI has become the gold standard in diagnosing liver
lesions, as it is considered to have a higher sensitivity and
specificity compared with CT.5,12 On T1-weighted MRI
images, FNH will appear either isointense or slightly
hypointense compared with the normal hepatic parenchyma, whereas T2-weighted images may show a relatively hyperintense mass.9 The central scar, when present,
is readily identified with MRI. Specific contrast media
used in MRI can also be used to identify the presence of
Kupffer cells.10 This is beneficial in the differential diagnosis between FNH and hepatic adenoma because adenomas do not typically contain Kupffer cells.13
22
Conclusion
FNH is a benign hepatic mass that may appear as a welldefined lesion of varying sonographic echogenicity. The
role of the sonographer in the diagnosis of FNH is apparent. Sonographers are obligated to identify the lesion,
measure it, and describe the sonographic appearance and
location relative to other hepatic anatomy. Sonographers
should also use critical thinking while obtaining clinical
history information and employ every technical tool to
examine a hepatic mass when discovered, including the
use of image optimization and color Doppler. The identification of a central scar may be difficult with sonography. Although MRI may play a larger role in obtaining a
more definitive diagnosis of FNH, it is the obligation of
the sonographer to understand any diagnostic differentiations and attempt to show these sonographic variations when a hepatic mass is identified. By obtaining an
accurate, thorough clinical history from our patients, we
can collect valuable information that can be used both
during and after the sonographic examination of a
hepatic mass, thus ultimately contributing to a definitive
diagnosis of FNH.
Funding
The authors received no financial support for the research,
authorship, and/or publication of this article.
References
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JDMXXX10.1177/8756479312460509Journ
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