Diagnostic Ultrasound - Abdomen and Pelvis
Focal Nodular Hyperplasia TERMINOLOGY Abbreviations • Focal nodular hyperplasia (FNH) Definitions • Benign tumor of liver caused by hyperplastic response to localized vascular abnormality IMAGING General Features • Best diagnostic clue ○ Homogeneously isoechoic mass often with central scar • Location ○ More common in right lobe ○ Usually subcapsular & rarely pedunculated • Size ○ Majority are smaller than 5 cm (85%) ○ Mean diameter at time of diagnosis: 3 cm • Key concepts ○ 2nd most common benign tumor of liver after hemangioma ○ Most frequent hepatic tumor in young women ○ Benign congenital hamartomatous malformation ○ Accounts for 8% of primary hepatic tumors in autopsy series ○ Usually solitary lesion (80%); multiple in 20% ○ Multiple FNHs associated with multiorgan vascular malformations and certain brain neoplasms Ultrasonographic Findings • Grayscale ultrasound ○ Mass: Mostly homogeneous and isoechoic to liver,occasionally hypoechoic or hyperechoic ○ Mass effect: Displacement of normal hepatic vessels and ducts ○ Central scar: Mostly hypoechoic, may be hyperechoic ○ Prominent draining veins seen as hypoechoic nodules around lesion • Color Doppler ○ Spoke-wheel pattern – Large central feeding artery with multiple small vessels radiating peripherally ○ Large draining veins at tumor margins ○ Highly vascular tumor, but hemorrhage is rare ○ High-velocity Doppler signals – Due to increased blood flow or arteriovenous shunts • Contrast-enhanced ultrasound ○ Arterial phase: Brisk enhancement with spoke-wheel pattern – Centrifugal enhancement: More common in small (≤ 3cm) FNH ○ Portal and delayed phase: No significant enhancement or washout CT Findings • NECT ○ Isodense or hypodense to normal liver • CECT ○ Hepatic arterial phase – Transient, intense, and homogeneous enhancement ○ Portal venous phase – Hypodense or isodense to normal liver – Large draining veins → hepatic veins ○ Delayed phase – Mass: Isodense to liver – Central scar: Hyperdense due to fibrous tissue – Scar visible in 2/3 of large (> 3 cm) & 1/3 of small FNH MR Findings • T1WI ○ Mass: Isointense to slightly hypointense ○ Central scar: Hypointense • T2WI ○ Mass: Slightly hyperintense to isointense ○ Central scar: Hyperintense • T1WI C+ ○ Arterial phase: Homogeneously hyperintense ○ Portal venous: Isointense ○ Delayed phase: Isointense mass with retention of contrast in central scar • Specific hepatobiliary MR contrast agents ○ Gadoxetate (Eovist or Primovist) – Bright homogeneous enhancement on arterial phase – Delayed scan: Significant enhancement of scar – Prolonged enhancement on hepatobiliary phase (20 minutes) scan □ Iso-/hyperintense mass with hypointense central scar □ Due to functioning hepatocytes and malformed bile ductules □ Most specific test to distinguish from all other hepatic masses Angiographic Findings • Conventional angiography ○ Arterial phase – Hypervascular mass with hypovascular central scar – Enlargement of main feeding artery with centripetal blood supply – Spoke-wheel pattern as on color Doppler ○ Venous phase: Large draining veins → hepatic veins ○ Capillary phase – Intense & nonhomogeneous stain – No avascular zones Nuclear Medicine Findings • Technetium sulfur colloid ○ Normal or increased uptake ○ Only FNH has both Kupffer cells & bile ductules ○ Almost pathognomonic in 60% of cases • Tc-HIDA scan (hepatic iminodiacetic acid) ○ Normal or increased uptake ○ Prolonged enhancement (80%) • Tc-99m tagged red blood cell scan (not useful) ○ Early isotope uptake and late defect Imaging Recommendations • Best imaging tool ○ CECT or contrast-enhanced MR for diagnosis – MR with gadoxetate hepatobiliary phase scans is most specific http://radiologyebook.com/ Diagnoses: Liver 239
Focal Nodular Hyperplasia 240 Diagnoses: Liver ○ Color Doppler ultrasound/contrast enhanced ultrasound – Spoke-wheel pattern vessels, brisk centrifugal enhancement on arterial phase, especially in smaller lesions CLINICAL ISSUES Presentation • Most common signs/symptoms ○ Often asymptomatic (in 50-90% incidental finding) ○ Vague abdominal pain (10-15%) due to mass effect ○ Hepatomegaly and abdominal mass: Very rare ○ Lab data: Usually normal liver function tests ○ Diagnosis – Characteristic imaging findings – Core needle biopsy (include central scar) DIFFERENTIAL DIAGNOSIS Hepatic Adenoma • Usually heterogeneous echogenicity due to hemorrhage, necrosis, or fat • Rarely has central scar • Washout on portal/delayed phase of CEUS/CT/MR Fibrolamellar Carcinoma Demographics • Usually Large (> 12 cm) heterogeneous mass • Large fibrous central scar with calcification ○ Hypointense on T2WI • Biliary, vascular, & nodal invasion may be present • Metastases (70% of cases) Cavernous Hemangioma • Isoechoic or heterogeneous lesions may simulate FNH • Nodular peripheral centripetal enhancement, no central scar Natural History & Prognosis • No risk of malignant transformation Hepatic Metastasis • No hemorrhagic complication • Multiple lesions, known primary tumor Treatment PATHOLOGY • Discontinuation of oral contraceptives • FNH seldom requires surgery General Features • Etiology DIAGNOSTIC CHECKLIST ○ Ischemia caused by occult occlusion of intrahepatic vessels Consider – Followed by hyperplastic response to abnormal vasculature ○ Localized arteriovenous shunting caused by anomalous Image Interpretation Pearls arterial supply ○ Oral contraceptives do not cause FNH, but have trophic effect on growth • Associated abnormalities ○ Hepatic hemangioma (23%) ○ Hepatic adenoma ○ Multiple lesions of FNH are associated with SELECTED REFERENCES – Brain neoplasms: Meningioma, astrocytoma – Vascular malformations of various organs Gross Pathologic & Surgical Features • Localized, well-delineated, usually solitary (80%), subcapsular mass • No true capsule, frequently central fibrous scar • No intratumoral calcification, hemorrhage, or necrosis Microscopic Features • Normal hepatocytes with large amounts of fat, triglycerides, & glycogen • Thick-walled arteries in fibrous septa radiating from center to periphery • Proliferation & malformation of bile ducts lead to slowing of bile excretion • Absent portal triads & central veins • Difficult differentiation from regenerative cirrhotic nodule and liver adenoma by histology http://radiologyebook.com/ • Age ○ Common in young to middle-aged women – 3rd-4th decades of life • Gender ○ M:F = 1:8 • Epidemiology ○ 4% of all primary hepatic tumors in pediatric population ○ 3-8% in adult population • Imaging is more reliable than histology in making diagnosis of FNH • Immediate, intense, homogeneously enhancing lesion on arterial phase followed rapidly by isodensity on venous phase with delayed enhancement of scar • Classic FNH looks like cross section of orange (central "scar," radiating septa) 1. Kong WT et al: Contrast-enhanced ultrasound in combination with color Doppler ultrasound can improve the diagnostic performance of focal nodular hyperplasia and hepatocellular adenoma. Ultrasound Med Biol. 41(4):944-51, 2015 2. Li W et al: Differentiation of atypical hepatocellular carcinoma from focal nodular hyperplasia: diagnostic performance of contrast-enhanced US and microflow imaging. Radiology. 140911, 2015 3. Suh CH et al: The diagnostic value of Gd-EOB-DTPA-MRI for the diagnosis of focal nodular hyperplasia: a systematic review and meta-analysis. Eur Radiol. 25(4):950-60, 2015 4. Bertin C et al: Contrast-enhanced ultrasound of focal nodular hyperplasia: a matter of size. Eur Radiol. 24(10):2561-71, 2014 5. Pei XQ et al: Quantitative analysis of contrast-enhanced ultrasonography: differentiating focal nodular hyperplasia from hepatocellular carcinoma. Br J Radiol. 86(1023):20120536, 2013 6. Wang W et al: Contrast-enhanced ultrasound features of histologically proven focal nodular hyperplasia: diagnostic performance compared with contrast-enhanced CT. Eur Radiol. 23(9):2546-54, 2013 7. Kamaya A et al: Hypervascular liver lesions. Semin Ultrasound CT MR. 30(5):387-407, 2009
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Focal Nodular Hyperplasia<br />
240<br />
Diagnoses: Liver<br />
○ Color Doppler ultrasound/contrast enhanced ultrasound<br />
– Spoke-wheel pattern vessels, brisk centrifugal<br />
enhancement on arterial phase, especially in smaller<br />
lesions<br />
CLINICAL ISSUES<br />
Presentation<br />
• Most common signs/symptoms<br />
○ Often asymptomatic (in 50-90% incidental finding)<br />
○ Vague abdominal pain (10-15%) due to mass effect<br />
○ Hepatomegaly <strong>and</strong> abdominal mass: Very rare<br />
○ Lab data: Usually normal liver function tests<br />
○ Diagnosis<br />
– Characteristic imaging findings<br />
– Core needle biopsy (include central scar)<br />
DIFFERENTIAL DIAGNOSIS<br />
Hepatic Adenoma<br />
• Usually heterogeneous echogenicity due to hemorrhage,<br />
necrosis, or fat<br />
• Rarely has central scar<br />
• Washout on portal/delayed phase of CEUS/CT/MR<br />
Fibrolamellar Carcinoma<br />
Demographics<br />
• Usually Large (> 12 cm) heterogeneous mass<br />
• Large fibrous central scar with calcification<br />
○ Hypointense on T2WI<br />
• Biliary, vascular, & nodal invasion may be present<br />
• Metastases (70% of cases)<br />
Cavernous Hemangioma<br />
• Isoechoic or heterogeneous lesions may simulate FNH<br />
• Nodular peripheral centripetal enhancement, no central<br />
scar<br />
Natural History & Prognosis<br />
• No risk of malignant transformation<br />
Hepatic Metastasis<br />
• No hemorrhagic complication<br />
• Multiple lesions, known primary tumor<br />
Treatment<br />
PATHOLOGY<br />
• Discontinuation of oral contraceptives<br />
• FNH seldom requires surgery<br />
General Features<br />
• Etiology<br />
DIAGNOSTIC CHECKLIST<br />
○ Ischemia caused by occult occlusion of intrahepatic<br />
vessels<br />
Consider<br />
– Followed by hyperplastic response to abnormal<br />
vasculature<br />
○ Localized arteriovenous shunting caused by anomalous<br />
Image Interpretation Pearls<br />
arterial supply<br />
○ Oral contraceptives do not cause FNH, but have trophic<br />
effect on growth<br />
• Associated abnormalities<br />
○ Hepatic hemangioma (23%)<br />
○ Hepatic adenoma<br />
○ Multiple lesions of FNH are associated with<br />
SELECTED REFERENCES<br />
– Brain neoplasms: Meningioma, astrocytoma<br />
– Vascular malformations of various organs<br />
Gross Pathologic & Surgical Features<br />
• Localized, well-delineated, usually solitary (80%),<br />
subcapsular mass<br />
• No true capsule, frequently central fibrous scar<br />
• No intratumoral calcification, hemorrhage, or necrosis<br />
Microscopic Features<br />
• Normal hepatocytes with large amounts of fat,<br />
triglycerides, & glycogen<br />
• Thick-walled arteries in fibrous septa radiating from center<br />
to periphery<br />
• Proliferation & malformation of bile ducts lead to slowing<br />
of bile excretion<br />
• Absent portal triads & central veins<br />
• Difficult differentiation from regenerative cirrhotic nodule<br />
<strong>and</strong> liver adenoma by histology<br />
http://radiologyebook.com/<br />
• Age<br />
○ Common in young to middle-aged women<br />
– 3rd-4th decades of life<br />
• Gender<br />
○ M:F = 1:8<br />
• Epidemiology<br />
○ 4% of all primary hepatic tumors in pediatric population<br />
○ 3-8% in adult population<br />
• Imaging is more reliable than histology in making diagnosis<br />
of FNH<br />
• Immediate, intense, homogeneously enhancing lesion on<br />
arterial phase followed rapidly by isodensity on venous<br />
phase with delayed enhancement of scar<br />
• Classic FNH looks like cross section of orange (central "scar,"<br />
radiating septa)<br />
1. Kong WT et al: Contrast-enhanced ultrasound in combination with color<br />
Doppler ultrasound can improve the diagnostic performance of focal<br />
nodular hyperplasia <strong>and</strong> hepatocellular adenoma. <strong>Ultrasound</strong> Med Biol.<br />
41(4):944-51, 2015<br />
2. Li W et al: Differentiation of atypical hepatocellular carcinoma from focal<br />
nodular hyperplasia: diagnostic performance of contrast-enhanced US <strong>and</strong><br />
microflow imaging. Radiology. 140911, 2015<br />
3. Suh CH et al: The diagnostic value of Gd-EOB-DTPA-MRI for the diagnosis of<br />
focal nodular hyperplasia: a systematic review <strong>and</strong> meta-analysis. Eur Radiol.<br />
25(4):950-60, 2015<br />
4. Bertin C et al: Contrast-enhanced ultrasound of focal nodular hyperplasia: a<br />
matter of size. Eur Radiol. 24(10):2561-71, 2014<br />
5. Pei XQ et al: Quantitative analysis of contrast-enhanced ultrasonography:<br />
differentiating focal nodular hyperplasia from hepatocellular carcinoma. Br J<br />
Radiol. 86(1023):20120536, 2013<br />
6. Wang W et al: Contrast-enhanced ultrasound features of histologically<br />
proven focal nodular hyperplasia: diagnostic performance compared with<br />
contrast-enhanced CT. Eur Radiol. 23(9):2546-54, 2013<br />
7. Kamaya A et al: Hypervascular liver lesions. Semin <strong>Ultrasound</strong> CT MR.<br />
30(5):387-407, 2009