[Liver neoplasms in patients with glycogen storage disease. Diagnostic and therapeutic problems].
In a family composed by eight brothers, five had a type I glycogen storage disease and three presented with liver tumors complicated with hemorrhage or malignant transformation, during the follow up. The periodic ultrasonographic control allowed the early diagnosis of these neoplasms. We describe the clinical picture and treatment of two patients. Metabolic alterations were corrected during the preoperative period with parenteral nutrition. Resection was successful in both cases. It is concluded that a close follow up and early treatment of these lesions is effective and avoids complications. In patients with multiple lesions and severe metabolic alterations, liver transplantation is the treatment of choice.
Rossi
R
R
Department of Surgery, Lahey Clinic, Burlington Mass, USA.
Pekolj
J
J
Glasinovic
J C
JC
Lee
A
A
D000328:Adult; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000150:complications* / Q000235:genetics; D006801:Humans; D008113:Liver Neoplasms / Q000175:diagnosis / Q000209:etiology* / Q000235:genetics / Q000628:therapy; D008297:Male; D010375:Pedigree