Resection of hepatocellular adenoma in patients with glycogen storage disease type Ia

• Published in: Journal of hepatology 2007-11, Vol.47 (5), p.658-663
• Main Authors: Reddy, Srinevas K, Kishnani, Priya S, Sullivan, Jennifer A, Koeberl, Dwight D, Desai, Dev M, Skinner, Michael A, Rice, Henry E, Clary, Bryan M
• Format: Article
• Language: English
• Subjects: Adenoma, Liver Cell - complications; Adenoma, Liver Cell - physiopathology; Adenoma, Liver Cell - surgery; Adolescent; Adult; Biliary Tract Surgical Procedures - statistics & numerical data; Biological and medical sciences; Carbohydrates (enzymatic deficiencies). Glycogenosis; Comorbidity; Disease Progression; Errors of metabolism; Female; Gastroenterology and Hepatology; Glycogen Storage Disease Type I - complications; Glycogen storage disease type Ia; Hepatic resection; Hepatocellular adenoma; Humans; Liver - metabolism; Liver - pathology; Liver - surgery; Liver Neoplasms - complications; Liver Neoplasms - physiopathology; Liver Neoplasms - surgery; Liver transplantation; Liver Transplantation - statistics & numerical data; Liver, biliary tract, pancreas, portal circulation, spleen; Male; Medical sciences; Metabolic diseases; Middle Aged; Retrospective Studies; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Surgery of the digestive system; Survival Rate; Treatment Outcome
• ISSN: 0168-8278; 1600-0641
• DOI: 10.1016/j.jhep.2007.05.012

Background/Aims Because dietary modifications have prolonged the life expectancy of patients with glycogen storage disease type Ia (GSD Ia), the incidence of hepatocellular adenoma (HCA) to carcinoma (HCC) transformation is increasing. The objective of this retrospective study is to assess the safety and effectiveness of HCA resection in GSD Ia patients. Methods Clinicopathologic, peri-operative, and long-term data were reviewed from patients who underwent HCA resection. Comparisons were made with Fisher’s exact, Mann–Whitney U , and log-rank tests; survival was estimated with Kaplan–Meier analysis. Results From 1998 to 2006, 38 patients underwent HCA resection. Seven (22%) had GSD Ia. Post-operative mortality occurred in one GSD Ia patient. GSD Ia patients had greater morbidity (86% vs. 20%) and shorter time to adenoma progression (median 23 months vs. not yet reached) after partial hepatectomy compared to the general population ( p < 0.05). Six GSD Ia patients had no evidence of HCC and recovered after resection without long-term morbidity. Three GSD Ia patients underwent liver transplantation 77, 32, and 23 months after adenoma resection. Conclusions Despite substantial morbidity, partial hepatectomy is feasible in GSD Ia patients and is an effective intermediate step in the prevention of HCC until definitive treatment with liver transplantation.