Multiple focal nodular hyperplasia occurs in approximately 20-25% of patients with focal nodular hyperplasia (FNH).
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
- liver haemangiomata (20% of cases)
- neoplasia of other organs: pheochromocytomas and GIST
- Klippel Trenauanay Weber syndrome
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
- 1. Finley AC, Hosey JR, Noone TC et-al. Multiple focal nodular hyperplasia syndrome: diagnosis with dynamic, gadolinium-enhanced MRI. Magn Reson Imaging. 2005;23 (3): 511-3. doi:10.1016/j.mri.2004.08.008 - Pubmed citation
- 2. “Focal Nodular Hyperplasia IV — Multiple FNH Syndrome.” In Liver MRI, 122-123, 2007. [Link].