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focal nodular hyperplasia

Thursday 29 May 2003

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

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#

Portfolio

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