Alshak N S, Cocjin J, Podesta L, van de Velde R, Makowka L, Rosenthal P, Geller S A
Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, Calif. 90048.
Arch Pathol Lab Med. 1994 Jan;118(1):88-91.
The development of hepatocellular adenoma has been recognized in association with glycogen storage disease type I and, less often, with glycogen storage disease type III, but, to our knowledge, it has not been reported in glycogen storage disease type IV. We had the opportunity to study an 11-month-old male infant who underwent orthotopic liver transplantation for cirrhosis that developed in the setting of glycogen storage disease type IV. A clinically unsuspected hepatocellular adenoma was present in the explanted liver. Glycogen storage disease type IV should be considered as a potential precursor to the development of hepatocellular adenoma. Recognition of this association is important, both in terms of the differential diagnosis of tumors that occur in this setting and also to anticipate potential complications of this benign neoplasm.
肝细胞腺瘤的发生已被认为与I型糖原贮积病有关，较少情况下与III型糖原贮积病有关，但据我们所知，尚未见其在IV型糖原贮积病中发生的报道。我们有机会研究一名11个月大的男婴，他因在IV型糖原贮积病背景下发生的肝硬化接受了原位肝移植。在切除的肝脏中存在一个临床未被怀疑的肝细胞腺瘤。IV型糖原贮积病应被视为肝细胞腺瘤发生的潜在前驱疾病。认识到这种关联很重要，这对于在此情况下发生的肿瘤的鉴别诊断以及预测这种良性肿瘤的潜在并发症都具有重要意义。
