Sonic hedgehog-activated hepatocellular adenoma
Citation, DOI, disclosures and article data
At the time the article was created Bruno Di Muzio had no financial relationships to ineligible companies to disclose.
View Bruno Di Muzio's current disclosuresAt the time the article was last revised Rohit Sharma had no financial relationships to ineligible companies to disclose.
View Rohit Sharma's current disclosuresSonic hedgehog-activated hepatocellular adenomas (sh-HCA) are a subtype of hepatocellular adenomas.
On imaging, it is still mostly diagnosed by exclusion of the other adenomas subtypes and other liver tumors.
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Epidemiology
Most of sh-HCA were previously grouped under the unclassified adenomas, but they have been shown to represent 4-10% of all adenomas 2-4.
Clinical presentation
Although usually aysmptomatic, acute abdominal pain from intratumoral hemorrhage is the most common presentation when symptomatic 2.
Pathology
This subtype is characterized by sonic hedgehog pathway activation, which is related to tissue regeneration, via focal deletions that fuse the promoter of INHBE with GLI1 2-4.
Macroscopic appearance
Macroscopically, sh-HCAs may show intralesional fluid-filled cavities or necrotic-hemorrhagic changes 2.
Radiographic features
MRI remains the modality of choice when working up hepatocellular adenomas. Unfortunately, there are no specific imaging features for sh-HCA, with diagnosis mostly suggested on exclusion.
Fluid-filled cavities within the tumor have been pointed as a particular feature of sh-HCA compared to the other subtypes 2. These do not enhance, with the fluid representing products of hemoglobin degradation.
References
- 1. Kim H & Park Y. Hepatocellular Adenomas: Recent Updates. J Pathol Transl Med. 2021;55(3):171-80. doi:10.4132/jptm.2021.02.27 - Pubmed
- 2. Ducatel A, Trillaud H, Reizine E et al. Sonic Hedgehog Hepatocellular Adenoma: Magnetic Resonance Imaging Features and Correlation with Histology. Eur Radiol. 2023;:1-14. doi:10.1007/s00330-023-10344-5 - Pubmed
- 3. Sala M, Gonzales D, Leste‐Lasserre T et al. ASS1 Overexpression: A Hallmark of Sonic Hedgehog Hepatocellular Adenomas; Recommendations for Clinical Practice. Hepatol Commun. 2020;4(6):809-24. doi:10.1002/hep4.1514 - Pubmed
- 4. Nault J, Couchy G, Balabaud C et al. Molecular Classification of Hepatocellular Adenoma Associates With Risk Factors, Bleeding, and Malignant Transformation. Gastroenterology. 2017;152(4):880-894.e6. doi:10.1053/j.gastro.2016.11.042 - Pubmed
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