multiple FNH syndrome

Multiple focal nodular hyperplasia occurs in approximately 20-25% of patients with focal nodular hyperplasia (FNH). It is defined by The International Working Party as consisting of two or more FNHs in combination with :

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

Associations

• meningiomas
• astrocytomas
• liver hemangiomata (20% of cases)
• neoplasia of other organs: pheochromocytomas and GIST
• Klippel Trenauanay Weber syndrome

Differential diagnosis 

Imaging differential considerations include:

• multifocal HCC: can be a challenging differential
  • 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

Siehe auch:

• Meningeom
• Leberhämangiom
• hepatozelluläres Karzinom
• Astrozytom
• Fokale noduläre Hyperplasie
• Klippel-Trénaunay-Weber-Syndrom

und weiter:

• FNH
• noduläre regenerative Hyperplasie