Question: A 48-year-old woman who suffered an accidental slip underwent abdominal computed tomography, which revealed multiple hepatic lesions in the whole liver. The patient was asymptomatic and physical examinations were unremarkable. She had normal growth and her body mass index was 23.9. No viral or autoimmune hepatitis was detected. The patient had a history of early-stage cervical carcinoma treated by radical hysterectomy 7 years ago without postoperative adjuvant therapy. History of smoking or taking oral contraceptives was denied. Laboratory tests showed the following results: alanine aminotransferase level of 14 U/L, aspartate aminotransferase level of 30 U/L, alkaline phosphatase level of 49 U/L, and bilirubin level of 13.1 μmol/L. Tumor markers of alpha-fetoprotein and CA19-9 were within normal range. Her blood count, electrolyte, glucose level, renal function, and coagulation tests were normal as well. Subsequent contrast-enhanced magnetic resonance (MR) imaging showed liver steatosis with multiple liver lesions in the whole liver. The largest lesion was found to be 28∗20 mm in segment V. The lesions were slightly hyperintense on T2-weighted sequences, with strong arterial enhancement, which persisted into the portal venous phase (Figure A). What is the diagnosis of the disease? See the Gastroenterology website (www.gastrojournal.org) for more information on submitting to Gastro Curbside Consult. The MR imaging showed multiple solid hepatic lesions characterized by predominant hypervascularization. Malignancy of multifocal hepatocellular carcinoma was first suspected, which is often hyper-vascular and encapsulated. However, cirrhosis and elevated tumor markers were not presented in our patient. Because the patient had a known primary malignancy of cervical carcinoma before, suspected diagnosis of hyper-vascular liver metastases was also included. In addition, focal nodular hyperplasia and liver adenomatosis sharing similar imaging features in noncirrhotic liver of women were considered for the differential diagnosis as well. The case was discussed with a multidisciplinary team. It was agreed that distinguishing the previously mentioned diseases might be challenging with imaging criteria alone and liver biopsy was required. The histopathologic examination showed cord-like arrangement of benign hepatocytes, which first excluded hepatocellular carcinoma and metastatic hepatic cancer. Additionally, moderate steatosis and lobar inflammation were observed (Figure B). The presence of fat and lack of portal venules distinguished adenomatosis from focal nodular hyperplasia, which led to the final diagnosis of liver adenomatosis. Additionally, adenomatosis is also characterized by thin-walled sinusoids supplied by arteries within hepatocytes, resulting in hypervascularity on arterial-phase imaging. Liver adenomatosis (LA) is defined as presence of 10 or more adenomas in the liver, which was first described by Flejou et al in 1985.1Flejou J.F. Barge J. Menu Y. et al.Liver adenomatosis. An entity distinct from liver adenoma?.Gastroenterology. 1985; 89: 1132-1138Abstract Full Text PDF PubMed Scopus (247) Google Scholar The known risk factors for the development of LA include exogenous estrogen or androgens, obesity, and glycogen storage disorder (GSD). Patients with GSD commonly present with hypoglycemia, hepatomegaly, and growth retardation at 3–6 months of age. LA of these patients usually has presentation of less than 20 years and progress in size and number. For our patient, oral contraceptives or steroids were denied. Although genetic testing was not performed, no typical clinical symptoms of GSD were observed at an early age, which lowered the risk of underlying GSD. The diagnosis of LA is mainly based on contrast-enhanced magnetic MR, which is the same for hepatic adenoma. Liver biopsy is recommended for cases in which imaging is inconclusive and histologic confirmation is able to distinguish between the 4 subtypes of adenomas.2Barbier L. Nault J.C. Dujardin F. et al.Natural history of liver adenomatosis: a long-term observational study.J Hepatol. 2019; 71: 1184-1192Abstract Full Text Full Text PDF PubMed Scopus (27) Google Scholar Based on imaging findings, LA can be further classified as massive or multifocal. The former has a higher risk of hemorrhage, whereas, for asymptomatic patients with multifocal type, the risk of malignant transformation is substantially higher.3Lammert C. Toal E. Mathur K. et al.Large hepatic adenomas and hepatic adenomatosis: a multicenter study of risk factors, interventions, and complications.Am J Gastroenterol. 2022; 117: 1089-1096Crossref PubMed Scopus (2) Google Scholar The patient in our case with multiple lesions raised the question of its management because liver resection is not possible. Considering the possibility that different subtypes of nodules may exist with high-risk malignant transformation, a 3-month imaging surveillance program with MR and tumor marker assessment were instituted in our case. After 2 years of follow-up, the patient lives a normal life without disease progress based on radiographical findings and blood tests.
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Dec 5, 2022
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