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
hepatic vascular anomalies
- congenital absence of the portal vein
- hepatic vein thrombosis
- intrahepatic shunts
- vascular anomalies in the brain berry aneurysms
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)
cystic renal dysplasia
inflammatory pseudotumor of the liver
adjacent to hepatic lesions
- hepatic hydatid cyst
adjacent to hepatic tumors
- hepatic adenoma
- hepatic hemangioma (23%)
- hepatic fibrolamellar carcinoma
- hepatic epithelioid hemangioendothelioma
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
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