Hepatocellular neoplasms arising in genetic metabolic disorders: steatosis is common in both the tumor and background liver

Lin Cheng, Dhanpat Jain, Sanjay Kakar, Michael S. Torbenson, Tsung Teh Wu, Matthew M. Yeh

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish (US)
Pages (from-to)93-99
Number of pages7
JournalHuman Pathology
Volume108
DOIs
StatePublished - Feb 2021

Keywords

  • Genetic metabolic disorders
  • Glycogen storage disease
  • Hepatocellular neoplasms
  • Hereditary tyrosinemia
  • Navajo neurophepatopathy
  • Ornithine carbamyl transferase deficiency
  • Steatosis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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