focal nodular hyperplasia

WKP

PO

Epidemiology

 more common in younger women than in men

Synopsis

 central stellate fibrous region containing large vessels

Associations (mostly when lesions are multiple)

 ingestion of oral contraceptives (?) (11854634, 3034543)

 local ischemia

• sickle cell anemia (1929517, 6766634)

 hepatic vascular anomalies

• congenital absence of the portal vein
• hepatic vein thrombosis
• intrahepatic shunts
• vascular anomalies in the brain berry aneurysms

 biliary atresia patient after Kasai hepatic portoenterostomy (# 18084770#, 17427766, 2004519)

 non-hepatic vascular anomalies

• cavernous hemangiomas
• non-hepatic arterial dysplasias
• hereditary hemorrhagic telangiectasia (HHT)
• Klippel-Trenaunay syndrome
• patent ductus venosus
• D-transposition of the great vessels (TGV) (6766634)

 inflammatory pseudotumor of the liver

 glycogen storage diseases

 adjacent to hepatic lesions

• hepatic hydatid cyst

 adjacent to hepatic tumors

• hepatic adenoma
• hepatic hemangioma (23%)
• hepatic fibrolamellar carcinoma
• hepatic epithelioid hemangioendothelioma

 cystic renal dysplasia

 intracranial tumors

• meningioma
• astrocytoma
• glioblastoma multiforme

 Beckwith-Wiedemann syndrome (15965690)
 hemihypertrophy

Pathological synopsis

 single nodule, anywhere within the liver parenchyma, usually clearly demarcated from the rest of the liver, but lacking a well defined capsule
 macroscopical central scar (50%)
 multiple pseudolobules with fibrovascular and ductular areas radiating from perilobular septa (80%)
 fibrous bands
 thick-walled vessels
 reactive ductules
 +/- teleangiectatic features
 +/- hyperplastic and even adenomatous parts
 +/- large-cell dysplasia has been described

Complications

 Hepatic vein obstruction
 Peripheral fatty infiltration
 Kasabach-Merrit syndrome

Variants

 telangiectatic focal nodular hyperplasia of the liver (16211451)

Differential diagnosis

 cirrhosis
 hepatocellular adenoma
 hepatoblastoma
 fibrolamellar hepatocellular carcinoma (fibrolamellar HCC)

References

 Nguyen BN, Flejou JF, Terris B, Belghiti J, Degott C. Focal nodular hyperplasia of the liver: a comprehensive pathologic study of 305 lesions and recognition of new histologic forms. Am J Surg Pathol. 1999 Dec;23(12):1441-54. PMID: 10584697

 Heaton ND, Pain J, Cowan NC, Salisbury J, Howard ER. Focal nodular hyperplasia of the liver: a link with sickle cell disease? Arch Dis Child. 1991 Sep;66(9):1073-4. PMID: 1929517

 Markowitz RI, Harcke HT, Ritchie WG, Huff DS. Focal nodular hyperplasia of the liver in a child with sickle cell anemia. AJR Am J Roentgenol. 1980 Mar;134(3):594-7. PMID: 6766634

 Knowles DM, Wolff M. Focal nodular hyperplasia of the liver: a clinicopathologic study and review of the literature. Hum Pathol. 1976 Sep;7(5):533-45. PMID: 964980