Download Features of Focal Nodular Hyperplasia on Multiple Imaging Modalities

Helen Xu, 2015
Gillian Lieberman, MD
December 2014
Features of Focal Nodular
Hyperplasia on Multiple Imaging
Modalities
Helen Xu, Harvard Medical School Year III
Gillian Lieberman, MD
Helen Xu, 2014
Gillian Lieberman, MD
Outline
• Introduction to focal nodular hyperplasia (FNH)
– Pathogenesis of FNH
– Pathology of FNH
– Epidemiology and typical clinical presentation
• Patient’s initial presentation
• Imaging
–
–
–
–
US
CT
MRI +/- Gd-BOPTA
DISIDA
• Patient’s clinical course
• FNH management in general
2
Helen Xu, 2014
Gillian Lieberman, MD
Outline
• Introduction to focal nodular hyperplasia (FNH)
– Pathogenesis of FNH
– Pathology of FNH
– Epidemiology and typical clinical presentation
• Patient’s initial presentation
• Imaging
–
–
–
–
US
CT
MRI +/- Gd-BOPTA
DISIDA
• Patient’s clinical course
• FNH management in general
3
Helen Xu, 2014
Gillian Lieberman, MD
FNH: What is it?
• Non-malignant hepatic tumor
Mitros, Frank A. Focal Nodular Hyperplasia Gross. uptodate.com
4
Helen Xu, 2014
Gillian Lieberman, MD
FNH: Pathogenesis
• Hyperplastic response to hyperperfusion by
anomalous arteries
5
Helen Xu, 2014
Gillian Lieberman, MD
Brief Review: Normal Structure
of Hepatic Lobule
Digestive System. Droualb.faculty.mjc.edu
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Helen Xu, 2014
Gillian Lieberman, MD
FNH: Pathology
• Central stellate scar contains an abnormally large artery in
the middle.
• Fibrotic tissue surrounds the abnormal artery, along with
portal veins and bile ducts.
• Hepatocytes are normal.
• FNH lesions contain
sinusoids and Kupffer
cells (specialized
macrophages), both of
which are absent in
hepatocellular adenoma
(HA).
Mitros, Frank A. FNH Trichrome. uptodate.com
7
Helen Xu, 2014
Gillian Lieberman, MD
FNH: Epidemiology and Clinical
Presentation
• Found predominantly in female between age 2050.
• Patient taking OCPs tend to have larger and more
vascular masses.
• Associated with hereditary hemorrhagic
telangiectasia (Osler-Weber-Rendu disease) and
hepatic hemangiomas.
• 90% solitary
• Two-thirds to three-fourths of patients are
identified incidentally. Can present with
abdominal discomfort or palpable mass.
8
Helen Xu, 2014
Gillian Lieberman, MD
Brief Review: Cross-Sectional
Anatomy of the Abdomen
med.umich.edu
9
Helen Xu, 2014
Gillian Lieberman, MD
Outline
• Introduction to focal nodular hyperplasia (FNH)
– Pathogenesis of FNH
– Pathology of FNH
– Epidemiology and typical clinical presentation
• Patient’s initial presentation
• Imaging
–
–
–
–
US
CT
MRI +/- Gd-BOPTA
DISIDA
• Patient’s clinical course
• FNH management in general
10
Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: Presentation
• 32F presenting w/ 5 days of episodic, crampy RUQ pain
associated w/ n/v.
– Episodes last a few hours
– No fevers/chills
• PMH: Obesity, Hereditary hemorrhagic
telangiectasia(HHT)  epistaxis requiring cauterization
• Family hx: HHT
• Social hx: 1 beer/wk, denies tobacco and recreational drugs
• Physical Exam:
– Vitals stable
– Abdomen soft, palpable liver edge, slightly tender. No
hepatomegaly appreciated.
• Labs: AST 45
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Helen Xu, 2014
Gillian Lieberman, MD
Outline
• Introduction to focal nodular hyperplasia (FNH)
– Pathogenesis of FNH
– Pathology of FNH
– Epidemiology and typical clinical presentation
• Patient’s initial presentation
• Imaging
–
–
–
–
US
CT
MRI +/- Gd-BOPTA
DISIDA
• Patient’s clinical course
• FNH management in general
12
Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: Work-Up (1)
#1 differential for crampy RUQ pain = biliary disease
RUQ Ultrasound
Examples of other ddx:
• Hepatitis
• Retrocecal appendicitis
• PUD
• Pancreatitis
• Liver abscess
• Liver, pancreatic, or
biliary tract cancer
• RLL pneumonia
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Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: RUQ U/S
BIDMC PACS
Sagittal RUQ U/S
•Liver: Homogeneous w/o focal lesion, or intra-hepatic duct
dilatation
•Gallbladder: thin-walled and unremarkable without
stones or pericholecystic fluid.
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Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: Work-Up (2)
Negative US
CT Abd/Pelvis
Numerous other ddx:
• Retrocecal appendicitis
• Hepatitis
• PUD
• Pancreatitis
• Liver abscess
• Liver, pancreatic, or
biliary tract cancer
• RLL pneumonia
15
Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: CT abd
BIDMC PACS
C+ axial abd CT: Portal venous phase
•At least 10 rounded hyper-enhancing lesions measuring up to 3.2cm
•
These lesions were isoechoic to liver parenchyma on U/S.
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Helen Xu, 2014
Gillian Lieberman, MD
Ddx for liver mass
•
•
•
•
•
•
FNH
Hepatic adenoma
HCC
Hemangioma
Metastatic cancer
Cyst, eg. congenital, echinococcal
(hydatid), polycystic kidney disease
• Hepatic (Amebic/Pyogenic) abscess
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Helen Xu, 2014
Gillian Lieberman, MD
Ddx based on CT and presentation
•
•
•
•
•
FNH
Hepatic adenoma
Hemangioma
HCC
Metastatic cancer—no primary CA, low
risk
• Cyst, eg. congenital, echinococcal
(hydatid), associated w/ polycystic kidney
disease— Low attenuation on CT C+
• Hepatic abscess (Amebic/pyogenic)—low
attenuation on CT C+
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Helen Xu, 2014
Gillian Lieberman, MD
Cyst and hepatic abscess are
ruled out because they have low
attenuation on CT with contrast.
Let’s explore how the other ddx
appear on CT.
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Helen Xu, 2014
Gillian Lieberman, MD
Ddx based on CT
•
•
•
•
FNH
Hepatic adenoma
HCC
Hemangioma
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Helen Xu, 2014
Gillian Lieberman, MD
Companion Pt 1: HCC on arterial
phase CT
• Main blood supply is the hepatic artery (normal liver
parenchyma has 20/80 arterial/portal blood supply)
Baron RL, Brancatelli G. 2004
C+ axial abd CT: arterial phase
•Diffuse enhancement of a large mass in the right lobe
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Helen Xu, 2014
Gillian Lieberman, MD
Companion Pt 1: HCC on portal
venous phase CT
• Portal venous phase CT: rapid washes out and become
iso/hypo-dense
• Heterogenous enhancement due to fibrosis, fat, necrosis
and calcifications.
Baron RL, Brancatelli G. 2004
C+ axial abd CT: Portal venous phase
•Heterogeneous mass, hypo-attenuating compared to adjacent liver parenchyma
•Septa hyper-attenuating because of contrast retention in the fibrotic tissue
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Helen Xu, 2014
Gillian Lieberman, MD
Ddx based on CT
•
•
•
•
FNH
Hepatic adenoma
HCC
Hemangioma
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Helen Xu, 2014
Gillian Lieberman, MD
Companion Pt 2: Hemangioma
on arterial phase CT
Morgan, Matt A., and Yuranga Weerakkody. Radiopaedia
C+ axial abd CT: arterial phase
• discontinuous, nodular, peripheral enhancement
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Helen Xu, 2014
Gillian Lieberman, MD
Companion Pt 2: Hemangioma
on portal venous phase CT
Morgan, Matt A., and Yuranga Weerakkody. Radiopaedia
C+ axial abd CT: portal venous phase
• progressive enhancement w/ centripetal fill in
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Helen Xu, 2014
Gillian Lieberman, MD
Companion Pt 2: Hemangioma
on delayed phase CT
Morgan, Matt A., and Yuranga Weerakkody. Radiopaedia
C+ axial abd CT: delayed phase
• Continued centripetal fill in
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Helen Xu, 2014
Gillian Lieberman, MD
Ddx based on CT
• FNH
• Hepatic adenoma
• Hemangioma– CT findings not characteristic, also should be
seen on US
• HCC– Low risk, CT findings not characteristic, also
should be seen on US
27
Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: Work-Up (3)
FNH vs Hepatic Adenoma
MRI w/ hepatobiliary gadolinium
contrast agents
It is very important to differentiate FNH and hepatic adenoma!
•FNH
• no evidence of malignant transformation
• Very rarely symptomatic and need resection
•Hepatic adenoma
• Associated w/ malignant transformation, spontaneous
hemorrhage and rupture
•Note: 20% of FNH can be associated with other benign tumor
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and vascular malformation.
Helen Xu, 2014
Gillian Lieberman, MD
Hepatobiliary Gd Contrast Agent
• Dual route of elimination (renal and hepatobiliary excretion)
• FNH is hyper/iso-intense on delayed phase because functional
hepatocytes and biliary ducts
• Hepatic adenoma is hypo-intense on delayed phase because
hepatocytes and biliary ducts are not functional
• Gd-DTPA
• 92% specificity for FNH
• Gd-BOPTA
• Differentiation of FNH vs HA:
• Sensitivity 96.9%, specificity 100%, PPV 100%, NPV
96.4% and overall accuracy 98.3
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Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: MRI T2
BIDMC PACS
Axial MRI T2
• Slightly hyper-intense lesions
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Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: MRI T2
BIDMC PACS
Coronal MRI T2
• Slightly hyper-intense lesions
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Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: MRI T1 early
arterial phase
BIDMC PACS
Axial MRI T1 early arterial phase w/ Gd-BOPTA (slightly delayed)
32
• Hyper-intense lesions (lack central scarring—atypical)
Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: MRI T1 late arterial
phase
BIDMC PACS
Axial MRI T1 late arterial phase w/ Gd-BOPTA (slightly delayed)
• Slightly hyper-intense lesions
33
Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: MRI T1 portal
venous phase
BIDMC PACS
Axial MRI T1 portal venous phase w/ Gd-BOPTA (slightly delayed)
• Slightly hyper-intense lesions
34
Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: MRI T1 equilibrium
phase
BIDMC PACS
Axial MRI T1 equilibrium phase w/ Gd-BOPTA (slightly delayed)
• Slightly hyper-intense lesions
35
Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: MRI T1 2hr delay
BIDMC PACS
Axial MRI T1 2hr delay w/ Gd-BOPTA
• Hyper-intense lesions (atypical)
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Helen Xu, 2014
Gillian Lieberman, MD
Our Patient:
nd
2
Presentation
• 1 month later, pt presented again w/ RUQ pain.
– Severity: 10/10
– Radiates to R flank, shoulder and R arm
– Labs were unremarkable
• More workup to rule out cholecystitis
– RUQ U/S: unremarkable, no evidence of
cholecystitis
– DISIDA
37
Helen Xu, 2014
Gillian Lieberman, MD
Now let’s look at some selected
images from our patient’s
DISIDA scan
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Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: DISIDA (1)
BIDMC PACS
• Liver
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Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: DISIDA (2)
BIDMC PACS
• CBD
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Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: DISIDA (3)
• Gallbladder
• Duodenum
BIDMC PACS
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Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: DISIDA (2)
inverted
• FNH lesion
w/ increased
uptake
BIDMC PACS
• FNH has functional hepatocytes and biliary ducts.
• may be photon deficient in 60% of cases
• Hepatic adenoma takes up the tracer but doesn’t excrete it.
• Not specific enough to differentiate FNH vs adenoma
• HCC takes up tracer in 50% of the case
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Helen Xu, 2014
Gillian Lieberman, MD
Outline
• Introduction to focal nodular hyperplasia (FNH)
– Pathogenesis of FNH
– Pathology of FNH
– Epidemiology and typical clinical presentation
• Patient’s initial presentation
• Imaging
–
–
–
–
US
CT
MRI +/- Gd-BOPTA
DISIDA
• Patient’s clinical course
• FNH management in general
43
Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: Clinical Course
• Because of patient’s demographics, history, and pain
following CCK administration (for DISIDA ), pt underwent
cholecystectomy.
• Gallstones were present on pathology which were not seen
on imaging.
• Pain significantly improved
• Initial presentation may have been due to atypical FNH or
cholecystitis
• The sensitivity of ultrasound in the detection of acute cholecystitis
is 95% and the specificity is 78-80%.
• This case of FNH is atypical: the lack of central scarring in many
lesions, the large number of nodules and pain
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Helen Xu, 2014
Gillian Lieberman, MD
Outline
• Introduction to focal nodular hyperplasia (FNH)
– Pathogenesis of FNH
– Pathology of FNH
– Epidemiology and typical clinical presentation
• Patient’s initial presentation
• Imaging
–
–
–
–
US
CT
MRI +/- Gd-BOPTA
DISIDA
• Patient’s clinical course
• FNH management in general
45
Helen Xu, 2014
Gillian Lieberman, MD
FNH: management in general
• No evidence for malignant transformation for FNH in
general.
• Follow up with Gd-DTPA at 3-6 months to confirm
stability of the lesions
• If stable, no long-term routine follow-up is required.
46
Helen Xu, 2014
Gillian Lieberman, MD
Our Patient: management
• Because this patient had atypical lesions, additional follow
up was recommended.
47
Helen Xu, 2014
Gillian Lieberman, MD
Take-home points: FNH
• Found predominantly in females between age 2050.
• Associated with hereditary hemorrhagic
telangiectasia
• 90% solitary
• 2/3 to 3/4 of lesions are identified incidentally.
Can present with abdominal discomfort or
palpable mass.
• Must be differentiated from hepatic adenoma
• 20% of cases are associated with other benign
lesions
48
Helen Xu, 2014
Gillian Lieberman, MD
Take-home points: FNH on Imaging
– US: Typically hypoechoic w/ central scar, can be
variable
– CT: Typically isodense on portal venous phase,
atypical in this case.
– MRI: use hepatobiliary Gd contrast. Early
enhancement. Hyper-/iso-intense on 2hr delay. Can
be atypical and lack central scar.
• Gd-BOPTA is more specific than Gd-DTPA
– DISIDA: normal or increased uptake and excretion
(may be photon deficient in as many as 60% of
patients). Not used to differentiate FNH from
adenoma.
49
Helen Xu, 2014
Gillian Lieberman, MD
Browse the following slides for
the appearance of typical FNH
lesions on various imaging
modalities
50
Helen Xu, 2014
Gillian Lieberman, MD
Typical FNH: U/S
Focal nodular hyperplasia and hepatic adenomas. ultrasoundcases.info
Transverse RUQ U/S
• Hypoechoic mass
• Central scar
51
Helen Xu, 2014
Gillian Lieberman, MD
Typical FNH: CT
Khan et al. 2013
CT C+: Arterial phase
• Hyperdense mass
• Central scar
Khan et al. 2013
CT C+: Portal venous phase
• Isodense mass
52
Helen Xu, 2014
Gillian Lieberman, MD
Typical FNH: MRI Gd-BOPTA
Kruskal, Jonathan B. Focal nodular hyperplasia of the liver seen
on Gd-BOPTA scan. Uptodate.com
Axial MRI T1, Gd-BOPTA, 3hr delay
• Hyper-enhancing mass
• Central scar
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Helen Xu, 2014
Gillian Lieberman, MD
References
•
•
•
•
•
•
•
•
•
Mitros, Frank A. Focal Nodular Hyperplasia Gross. N.d. Uptodate. Focal Nodular
Hyperplasia. Web. 10 Dec. 2014.
Mitros, Frank A. FNH Trichrome. N.d. Uptodate. Focal Nodular Hyperplasia. Web. 10
Dec. 2014.
Chopra, Sanjiv, and Anne C. Travis. "Focal Nodular Hyperplasia." Focal Nodular
Hyperplasia. Ed. Bruce A. Runyon. UpToDate, 19 Nov. 2014. Web. 10 Dec. 2014.
Lobular Organization. N.d. Droualb.faculty.mjc.edu. Digestive System. Web. 10 Dec.
14.
Wanless IR, Gryfe A. Nodular transformation of the liver in hereditary hemorrhagic
telangiectasia. Arch Pathol Lab Med. 1986;110(4):331-5.
Goodman ZD. Benign Tumors of the Liver. In: Neoplasms of the Liver, Okuda K, Ishak
KD (Eds), Springer, Tokyo 1987. p.105.
Morgan, Matt A., and Yuranga Weerakkody. "Hepatic Haemangioma." Radiopaedia.
N.p., n.d. Web. 10 Dec. 2014.
LaBrecque DR, Cakir-Yedidag A. Management of Liver Neoplasms in Medical
Management of Liver Disease, EL Krawitt (Ed), Marcel Dekker, Inc. 1999, p.310.
Grazioli L, Morana G, Federle MP, et al. Focal nodular hyperplasia: morphologic and
functional information from MR imaging with gadobenate dimeglumine. Radiology.
2001;221(3):731-9.
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References
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•
•
Grazioli L, Morana G, Kirchin MA, Schneider G. Accurate differentiation of focal
nodular hyperplasia from hepatic adenoma at gadobenate dimeglumine-enhanced MR
imaging: prospective study. Radiology. 2005;236(1):166-77.
"Cholecystitis." Meddean.luc.edu. N.p., n.d. Web. 13 Dec. 2014
Baron RL, Brancatelli G. Computed tomographic imaging of hepatocellular carcinoma.
Gastroenterology. 2004;127(5 Suppl 1):S133-43.
Digestive System. Droualb.faculty.mjc.edu.
http://droualb.faculty.mjc.edu/Lecture%20Notes/Unit%206/Spring%2006%20Digestive
%20system%20with%20figures.htm
http://www.med.umich.edu/lrc/coursepages/m1/anatomy2010/html/clinicalcases/cholelit
hiasis/gs6.jpg
Baron RL, Brancatelli G. Computed tomographic imaging of hepatocellular carcinoma.
Gastroenterology. 2004;127(5 Suppl 1):S133-43.
Focal nodular hyperplasia and hepatic adenomas. ultrasoundcases.info.
http://www.ultrasoundcases.info/Case-List.aspx?cat=129
Khan AN, Boylan C, Sohaib M, Tam C, Sheen AJ. Imaging in Focal Nodular
Hyperplasia. http://emedicine.medscape.com/article/368377-overview
Kruskal, Jonathan B. Focal nodular hyperplasia of the liver seen on Gd-BOPTA scan.
Uptodate. Focal Nodular Hyperplasia. Web. 10 Dec. 2014.
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Helen Xu, 2014
Gillian Lieberman, MD
Acknowledgements
• Dr. Gillian Lieberman
• Dr. Jonathan Kim
• Mr. Joseph Singer
56