Multiple focal nodular hyperplasia syndrome

Last revised by Daniel J Bell on 20 Jan 2019

Citation, DOI, disclosures and article data

Citation:

Yang N, Bell D, Di Muzio B, et al. Multiple focal nodular hyperplasia syndrome. Reference article, Radiopaedia.org (Accessed on 19 Apr 2024) https://doi.org/10.53347/rID-6767

DOI:

https://doi.org/10.53347/rID-6767

Permalink:

https://radiopaedia.org/articles/6767

rID:

6767

Article created:

16 Aug 2009, Natalie Yang ◈

Disclosures:

At the time the article was created Natalie Yang had no recorded disclosures.

View Natalie Yang's current disclosures

Last revised:

20 Jan 2019, Daniel J Bell ◉

Disclosures:

At the time the article was last revised Daniel J Bell had no recorded disclosures.

View Daniel J Bell's current disclosures

Revisions:

6 times, by 6 contributors - see full revision history and disclosures

Systems:

Hepatobiliary

Tags:

cases

Synonyms:

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 ¹:

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