Multiple FNH syndrome

Multiple focal nodular hyperplasia occurs in approximately 20-25% of patients with focal nodular hyperplasia (FNH).

Definition

Multiple FNH syndrome is defined by The International Working Party as consisting of two or more FNHs in combination with 1:

  • liver haemangioma or
  • vascular malformations (most frequent types: hepatic haemangioma, arterial dysplasia, portal vein atresia, berry aneurysms or pulmonary arterial hypertension) or
  • intracranial tumours 
Associations

Differential diagnosis 

Imaging differential considerations include

  • multifocal HCC: can be a challenging differential. 
  • hepatocellular carcinoma (HCC): has a true fibrotic capsule present in 60-80% of cases, but FNH may have a pseudocapsule composed of compressed parenchyma, perilesional vessels and inflammation.
  • multiple hepatic adenomas: do not tend to have a central scar and majority of them contain fat
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Article information

rID: 6767
Tag: cases
Synonyms or Alternate Spellings:
  • Multiple focal nodular hyperplasia syndrome

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