Case of cholangiocellular carcinoma in a patient with glycogen storage disease type Ia |
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| Authors: | Hiroyuki Kanamori Yukiomi Nakade Takaya Yamamoto Yuji Kobayashi Ken Sato Kiyoaki Ito Tomohiko Ohashi Noiku Nakao Norimitsu Ishii Emiko Takahashi Toyoharu Yokoi Haruhisa Nakao Tsuyoshi Kurokawa Chikara Yamaguchi Masashi Yoneda |
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| Affiliation: | 1. Department of Internal Medicine, Aichi Medical University, Nagakute, Japan;2. Department of Surgery, Division of Gastroenterology, Aichi Medical University, Nagakute, Japan;3. Department of Pathology, Aichi Medical University, Nagakute, Japan;4. Masuko Hospital, Nagoya, Japan;5. Setoguchi Psychosomatic Clinic, Seto, Japan |
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| Abstract: | Glycogen storage disease (GSD) type Ia is caused by a deficiency in glucose‐6‐phosphatase. Long‐term complications, including renal disease, gout, osteoporosis and pulmonary hypertension, develop in patients with GSD type Ia. In the second or third decade, 22–75% of GSD type Ia patients develop hepatocellular adenoma (HCA). In some of these patients, the HCA evolves into hepatocellular carcinoma. However, little is known about GSD type Ia patients with HCA who develop cholangiocellular carcinoma (CCC). Here, we report for the first time, a patient with GSD type Ia with HCA, in whom intrahepatic CCC was developed. |
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| Keywords: | cholangiocellular carcinoma glycogen storage disease hepatocellular adenoma |
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