Beta catenin mutated hepatic adenoma
Updates to Synonym Attributes
Updates to Synonym Attributes
Updates to Synonym Attributes
Updates to Article Attributes
Beta catenin mutated hepatic adenomas are a genetic and pathologic subtype of hepatic adenoma. Their appearance and prognosis is different than other subtypes.
Epidemiology
They are the least common subtype of hepatic adenoma (10-15%) hepatic adenoma. They occur more frequently in men and are associated with male hormone administration, glycogen storage disease, and familial adenomatous polyposis (FAP).
Clinical presentation
May develop right upper quadrant pain from intratumoural haemorrhage.
Radiographic features
The appearance of this subtype overlaps the appearance of other subtypes. No distinctive imaging features have yet been identified.
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 beta catenin mutated pathologic subtype, then the risk of malignant transformation to hepatocellular carcinoma is higher than with other subtypes, and followup should be according to HCC guidelines.
Differential diagnosis
- other types of hepatic adenoma
- inflammatory hepatic adenoma
- HNF 1alpha mutated hepatic adenoma
- unclassified hepatic adenoma
-
hepatocellular carcinoma (HCC)
- washout tends to leave the lesion hypointense c.f. to 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
-<p><strong>Beta catenin mutated hepatic adenomas</strong> are a genetic and pathologic subtype of <a href="/articles/hepatic-adenoma">hepatic adenoma</a>. Their appearance and prognosis is different than other subtypes.</p><h4>Epidemiology</h4><p>They are the least common subtype of hepatic adenoma (10-15%) hepatic adenoma. They occur more frequently in men and are associated with male hormone administration, <a title="Glycogen storage disease" href="/articles/glycogen-storage-disease">glycogen storage disease</a>, and <a title="Familial adenomatous polyposis (FAP)" href="/articles/familial-adenomatous-polyposis-syndrome">familial adenomatous polyposis (FAP)</a>.</p><h4>Clinical presentation</h4><p>May develop right upper quadrant pain from intratumoural haemorrhage.</p><h4>Radiographic features</h4><p>The appearance of this subtype overlaps the appearance of other subtypes. No distinctive imaging features have yet been identified.</p><h4>Treatment and prognosis</h4><p>If imaging shows a hepatic adenoma, then patients usually stop OCPs and the lesions regresses.</p><p>If it does not regress, then one treatment pathway suggests:</p><ul>- +<p><strong>Beta catenin mutated hepatic adenomas</strong> are a genetic and pathologic subtype of <a href="/articles/hepatic-adenoma">hepatic adenoma</a>. Their appearance and prognosis is different than other subtypes.</p><h4>Epidemiology</h4><p>They are the least common subtype of hepatic adenoma (10-15%). They occur more frequently in men and are associated with male hormone administration, <a href="/articles/glycogen-storage-disease">glycogen storage disease</a>, and <a href="/articles/familial-adenomatous-polyposis-syndrome">familial adenomatous polyposis (FAP)</a>.</p><h4>Clinical presentation</h4><p>May develop right upper quadrant pain from intratumoural haemorrhage.</p><h4>Radiographic features</h4><p>The appearance of this subtype overlaps the appearance of other subtypes. No distinctive imaging features have yet been identified.</p><h4>Treatment and prognosis</h4><p>If imaging shows a hepatic adenoma, then patients usually stop OCPs and the lesions regresses.</p><p>If it does not regress, then one treatment pathway suggests:</p><ul>
-</ul><p>Tissue diagnosis then confirms or changes the adenoma subtype. If beta catenin mutated pathologic subtype, then the risk of malignant transformation to <a title="Hepatocellular carcinoma (HCC)" href="/articles/hepatocellular-carcinoma">hepatocellular carcinoma</a> is higher than with other subtypes, and followup should be according to HCC guidelines.</p><h4>Differential diagnosis</h4><ul>- +</ul><p>Tissue diagnosis then confirms or changes the adenoma subtype. If beta catenin mutated pathologic subtype, then the risk of malignant transformation to <a href="/articles/hepatocellular-carcinoma">hepatocellular carcinoma</a> is higher than with other subtypes, and followup should be according to HCC guidelines.</p><h4>Differential diagnosis</h4><ul>
-<li><a title="HNF 1alpha mutated hepatic adenoma" href="/articles/hnf-1alpha-mutated-hepatic-adenoma">HNF 1alpha mutated hepatic adenoma</a></li>- +<li><a href="/articles/hnf-1alpha-mutated-hepatic-adenoma">HNF 1alpha mutated hepatic adenoma</a></li>
References changed:
- 1. Katabathina VS, Menias CO, Shanbhogue AK et-al. Genetics and imaging of hepatocellular adenomas: 2011 update. Radiographics. 2011;31 (6): 1529-43. <a href="http://dx.doi.org/10.1148/rg.316115527">doi:10.1148/rg.316115527</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/21997980">Pubmed citation</a><span class="auto"></span>
- 2. Bioulac-Sage P, Laumonier H, Couchy G et-al. Hepatocellular adenoma management and phenotypic classification: the Bordeaux experience. Hepatology. 2009;50 (2): 481-9. <a href="http://dx.doi.org/10.1002/hep.22995">doi:10.1002/hep.22995</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/19585623">Pubmed citation</a><span class="auto"></span>
Tags changed:
- cases
- liver tumour
- hepatic adenoma
Systems changed:
- Hepatobiliary