Resection of hepatocellular adenoma in patients with glycogen storage disease type Ia.
OBJECTIVE
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.
Reddy
Srinevas K
SK
Department of Surgery, Duke University Medical Center, Box 3247, Durham, NC 27710, USA. reddy005@mc.duke.edu
Kishnani
Priya S
PS
Sullivan
Jennifer A
JA
Koeberl
Dwight D
DD
Desai
Dev M
DM
Skinner
Michael A
MA
Rice
Henry E
HE
Clary
Bryan M
BM
D018248:Adenoma, Liver Cell / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D000293:Adolescent; D000328:Adult; D001662:Biliary Tract Surgical Procedures / Q000706:statistics & numerical data*; D015897:Comorbidity; D018450:Disease Progression; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000150:complications*; D006801:Humans; D008099:Liver / Q000378:metabolism / Q000473:pathology / Q000601:surgery*; D008113:Liver Neoplasms / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D016031:Liver Transplantation / Q000706:statistics & numerical data; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome