When do you refer a patient with MODY3 to GI/Hepatology for monitoring/management of hepatic adenomas?

As you know, maturity-onset diabetes of the young type 3 (MODY 3) is a consequence of heterozygous germ-line mutations in HNF1A. In addition, hepatocellular adenoma (HCA) may be caused by somatic HNF1A mutations. There are approximately a dozen cases reported of individuals with MODY 3 and HCA (1). HCA are rare and benign and may be solitary or multiple and may be asymptomatic or associated with pain, hemorrhage, and increased risk of hepatocellular carcinoma.

The exact prevalence of HCA in MODY 3 patients is still unknown. The frequency of the occurrence of both conditions in the same individual may be related to the specific mutations in HNF1A. Systematic radiologic screening of MODY 3 patients seems reasonable. I would prefer an abdominal...