Sun Young Ahn, Soo Young Park, Young Oh Kweon, Won Young Tak, Department of Internal Medicine, Kyungpook National University Hospital, Daegu 700-721, South Korea.
World J Gastroenterol. 2013 Nov 14;19(42):7480-6. doi: 10.3748/wjg.v19.i42.7480.
Hepatocellular adenoma (HCA) is one of the important complications of glycogen storage disease type Ia (GSD-Ia) because it can be transformed into hepatocellular carcinoma. Although surgical resection is a standard treatment of choice for solitary HCA, multiple HCAs in GSD-Ia patients present as therapeutic challenges for curative treatment. Therefore, treatment strategy according to malignant potential is important in management of HCAs in GSD-Ia. The authors present a case of histologically proven multiple HCAs without β-catenin mutations occurred in a GSD-Ia patient treated successfully with percutaneous radiofrequency ablation as a minimally invasive therapy.
肝细胞腺瘤(HCA)是糖原贮积病 Ia 型(GSD-Ia)的重要并发症之一,因为它可能会转化为肝细胞癌。尽管手术切除是治疗单发 HCA 的标准选择,但 GSD-Ia 患者的多个 HCA 存在治疗挑战,难以进行根治性治疗。因此,根据恶性潜能制定治疗策略对于 GSD-Ia 患者的 HCA 管理很重要。作者报告了一例组织学证实的 GSD-Ia 患者存在多个 HCA 且无β-连环蛋白突变,成功接受了经皮射频消融作为一种微创治疗。