TY - JOUR
T1 - Hepatocellular neoplasms arising in genetic metabolic disorders
T2 - steatosis is common in both the tumor and background liver
AU - Cheng, Lin
AU - Jain, Dhanpat
AU - Kakar, Sanjay
AU - Torbenson, Michael S.
AU - Wu, Tsung Teh
AU - Yeh, Matthew M.
N1 - Publisher Copyright:
© 2020 Elsevier Inc.
PY - 2021/2
Y1 - 2021/2
N2 - Hepatocellular neoplasms can develop in multiple genetic metabolic disorders. While there have been rare case reports, clinical and pathological characterizations have not been systematically performed. We conducted a retrospective study in 9 patients with these rare genetic metabolic disorders, including glycogen storage disease type 1, ornithine carbamyl transferase deficiency, hereditary tyrosinemia type 1, and Navajo neurohepatopathy, who developed hepatocellular neoplasms. Our results show that steatosis is a common finding in both tumor (6/9 cases, 67%) and background liver parenchyma (8/9 cases, 89%), underlying a possible role for steatosis in tumorigenesis in these genetic metabolic disorders. Our findings also raise a consideration of underlying genetic metabolic disorder when young patients with hepatocellular neoplasm show steatosis in both the tumor and background liver.
AB - Hepatocellular neoplasms can develop in multiple genetic metabolic disorders. While there have been rare case reports, clinical and pathological characterizations have not been systematically performed. We conducted a retrospective study in 9 patients with these rare genetic metabolic disorders, including glycogen storage disease type 1, ornithine carbamyl transferase deficiency, hereditary tyrosinemia type 1, and Navajo neurohepatopathy, who developed hepatocellular neoplasms. Our results show that steatosis is a common finding in both tumor (6/9 cases, 67%) and background liver parenchyma (8/9 cases, 89%), underlying a possible role for steatosis in tumorigenesis in these genetic metabolic disorders. Our findings also raise a consideration of underlying genetic metabolic disorder when young patients with hepatocellular neoplasm show steatosis in both the tumor and background liver.
KW - Genetic metabolic disorders
KW - Glycogen storage disease
KW - Hepatocellular neoplasms
KW - Hereditary tyrosinemia
KW - Navajo neurophepatopathy
KW - Ornithine carbamyl transferase deficiency
KW - Steatosis
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U2 - 10.1016/j.humpath.2020.11.012
DO - 10.1016/j.humpath.2020.11.012
M3 - Article
C2 - 33245984
AN - SCOPUS:85098659730
SN - 0046-8177
VL - 108
SP - 93
EP - 99
JO - Human Pathology
JF - Human Pathology
ER -