HNF 1 alpha mutated hepatic adenoma
Updates to Article Attributes
HNF 1 alpha mutated hepatic adenomas are a genetic and pathologic subtype of hepatic adenoma. Their appearance and prognosis are different from other subtypes.
Epidemiology
They are the second most common (30-35%) hepatic adenoma, after the inflammatory subtype. They occur only in female patients, most with a history of oral contraceptive (OCP) use.
Clinical presentation
May develop right upper quadrant pain from intratumoural haemorrhage.
Radiographic features
This subtype of adenoma is multiple in ~50%.
MRI
- T1: isointense or hyperintense relative to liver
-
T1 C+ (Gd):
- moderate arterial phase enhancement (less than inflammatory subtype)
- less likely to have persistent enhancement on portal venous and delayed phases, cf. inflammatory subtype
- IP/OP: hypointensity on the out-of-phase sequence
- T2: isointense to mildlyhyperintense
Treatment and prognosis
If imaging shows a hepatic adenoma, then patients usually stop OCPs and the lesions regresses.
If it does not regress, then one treatment pathway suggests:
- ≥5 cm: resection (hepatic adenomas larger than 5 cm are at increased risk of haemorrhage)
- <5 cm: biopsy
Tissue diagnosis then confirms or changes the adenoma subtype. If HNF 1 alpha mutated pathologic subtype, then:
- clinical and imaging follow up of any remaining adenomas until menopause
- resection or thermal ablation of enlarging adenomas that grow >5 cm
- genetic counselling if there is hepatic adenomatosis, familial history of hepatic adenoma, or MODY3 (mature onset diabetes of the young, type 3)
There is also a very small risk that a hepatic adenoma may develop into a hepatocellular carcinoma (HCC).
Differential diagnosis
- other types of hepatic adenoma
-
hepatocellular carcinoma(HCC)
- washout tends to leave the lesion hypointense compared to the rest of liver
- different demographics
- may be difficult to distinguish if well differentiated 7
- focal nodular hyperplasia(FNH)
- liver metastases (hypervascular)
- for other differential considerations, see the main article: hepatic adenoma
-<a href="/articles/hepatocellular-carcinoma-hcc-1">hepatocellular carcinoma (HCC)</a><ul>- +<a href="/articles/hepatocellular-carcinoma">hepatocellular carcinoma</a> (HCC)<ul>
-<li><a href="/articles/focal-nodular-hyperplasia">focal nodular hyperplasia (FNH)</a></li>- +<li>
- +<a href="/articles/focal-nodular-hyperplasia">focal nodular hyperplasia</a> (FNH)</li>