Malignant transformation of hepatocellular adenoma with bone marrow metaplasia arising in glycogen storage disease type I: A case report

Iguchi,Tomohiro; Yamagata,Motoyuki; Sonoda,Takashi; Yanagita,Kimihiko; Fukahori,Tetsuhiro; Tsujita,Eiji; Aishima,Shinichi; Oda,Yoshinao; Maehara,Yoshihiko

doi:10.3892/mco.2016.1034

Malignant transformation of hepatocellular adenoma with bone marrow metaplasia arising in glycogen storage disease type I: A case report

Authors:
- Tomohiro Iguchi
- Motoyuki Yamagata
- Takashi Sonoda
- Kimihiko Yanagita
- Tetsuhiro Fukahori
- Eiji Tsujita
- Shinichi Aishima
- Yoshinao Oda
- Yoshihiko Maehara
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Affiliations: Department of Gastroenterological Surgery, National Kyushu Cancer Center, Fukuoka 811‑1395, Japan, Department of Surgery, Saiseikai Karatsu Hospital, Karatsu, Saga 847‑0852, Japan, Department of Internal Medicine, Saiseikai Karatsu Hospital, Karatsu, Saga 847‑0852, Japan, Department of Radiology, Saiseikai Karatsu Hospital, Karatsu, Saga 847‑0852, Japan, Department of Pathology and Microbiology, Saga Medical School Faculty of Medicine, Saga University, Saga 849‑8501, Japan, Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Fukuoka 812‑8582, Japan, Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Fukuoka 812‑8582, Japan
Published online on: September 21, 2016 https://doi.org/10.3892/mco.2016.1034
Pages: 599-603

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Abstract

Malignant transformation of hepatocellular adenoma (HA) is relatively rare and has been reported to be associated with dysregulation of the β-catenin pathway. The presence of bone marrow metaplasia in HA is an uncommon histological characteristic. The current report presents the case of a 46‑year‑old woman with glycogen storage disease type I (von Gierke's disease) who underwent resection of hepatocellular carcinoma (HCC) arising in a HA with associated bone marrow metaplasia producing three series of hematopoietic cells. The serum level of proteins induced by des‑gamma‑carboxy prothrombin (DCP) gradually increased as the tumors grew; following hepatic resection, DCP levels returned to normal. Nuclear accumulation of β‑catenin was shown in HCC by immunohistochemistry; however, no mutation was detected in exon 3 of β‑catenin. To the best of our knowledge, this is the first report of HA with absolute bone marrow metaplasia producing three series of hematopoietic cells. This occurrence suggests that elevated DCP may be an indicator of malignant transformation of HA.

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