Case report: hepatic adenoma with bone marrow metaplasia in a patient with glycogen storage disease type 1a.

A 34 year old man with glycogen storage disease type 1a had two hepatic tumours since 18 years of age. They had continued to grow until he was 24 years old, but showed no further growth since then. He underwent a right hepatic trisegmentectomy with caudate lobectomy under veno-venous bypass. The tumour in the posterior segment showed nodule-within-nodule appearance. Histologically, the inner adenoma with dysplasia, bone marrow metaplasia and lymphocytic infiltration was separated by a fibrous band from the outer adenoma of usual histology.