"Bull’s eye” appearance of hepatocellular adenomas in patients with glycogen storage disease type I — atypical magnetic resonance imaging findings: Two case reports

Abstract

BACKGROUND Hepatocellular adenomas are rare tumors that can occur in patients with glycogen storage disease type I. CASE SUMMARY We herein report two cases of histologically proven hepatocellular adenomas in patients with glycogen storage disease type I. Magnetic resonance imaging (MRI) was performed after bolus injection of gadoxetate disodium, a liver-specific gadolinium-based MRI contrast agent. In the present cases, some of the hepatocellular adenomas showed unexpectedly a “bull’s eye” appearance on T2-weighted and post-contrast images, which was not previously described as imaging findings of hepatocellular adenomas in glycogen storage disease. A bull’s eye appearance on T2-weighted images can be encountered in both benign ( i.e. , abscess) or malignant ( i.e. , epithelioid hemangioendothelioma, cholangio-carcinoma, and metastases) hepatic lesions. CONCLUSION We present two cases of hepatocellular adenomas in patients with glycogen storage disease type 1, in which gadoxetate disodium-MRI showed atypical imaging findings for hepatocellular adenomas. At present there is no systematic study evaluating MRI findings of hepatocellular adenomas in patients with glycogen storage disease, further studies are needed to specifically investigate this issue.

Development of hepatocellular adenomas in a patient with glycogen storage disease Ia treated with growth hormone therapy

“…HCAs in patients with GSD Ia typically manifest between puberty and early adulthood, though there are patients who have presented earlier in life, 1,5–8 and they are notably different from HCAs found in the general population, including a lack of predilection for female patients, bilobar distribution, and a greater total number of HCAs as opposed to the encapsulated single HCA that result from oral contraceptive pills (OCP) 1 . Additionally, HCA associated with GSD Ia can have an atypical or “bullseye” appearance on MRI as opposed to the “atoll” sign observed in HCA in the general population 9,10 . Long‐term complications of HCA in GSD Ia include an increased risk for bleeding and potential for malignant transformation into hepatocellular carcinoma (HCC), 11 and can lead to patients undergoing resection of large HCA or orthotopic liver transplantation if tumor burden is significant enough.…”

“… 1 Additionally, HCA associated with GSD Ia can have an atypical or “bullseye” appearance on MRI as opposed to the “atoll” sign observed in HCA in the general population. 9 , 10 Long‐term complications of HCA in GSD Ia include an increased risk for bleeding and potential for malignant transformation into hepatocellular carcinoma (HCC), 11 and can lead to patients undergoing resection of large HCA or orthotopic liver transplantation if tumor burden is significant enough.…”