[A case of glycogen storage disease type I with hepatocellular carcinoma].
The patient was a 55-year-old female. In 1997, she was diagnosed as type-I glycogen storage disease (von Gierke disease). In March 2002, abdominal ultrasound tomography revealed an early enhanced lesion at liver S2, which suspected to a well differentiated hepatocellular carcinoma (HCC) with super-paramagnetic iron oxide (SPIO) enhanced magnetic resonance imaging (MRI). From 2002 to 2006, she received three times trans-arterial chemo-embolization (TACE) at enhanced lesions. But abdominal computer tomography (CT) revealed a 3.6 cm-in diameter early enhanced lesion near this tumor at S2, which was suspected to a diagnosis of recurrent HCC in December 2008. Therefore, she received a partial hepatectomy at S2. This resected specimen was diagnosed as poorly differentiated HCC. This patient is still alive with no recurrence after 6 months from operation. In conclusion, it might be effective for an early detection of recurrent HCC to see the doctor for a long regular checkup, because the growth of HCC with glycogen storage disease would be very slow.
Okuda
Yukiko
Y
Department of Surgery, Suita Municipal Hospital.
Ota
Hideo
H
Mikami
Koji
K
Nagase
Hirotsugu
H
Mukai
Ryota
R
Okada
Kazuyuki
K
Ide
Yoshihito
Y
Yanagisawa
Tetsu
T
Maruyama
Kentaro
K
Murata
Kohei
K
Yokouchi
Hideoki
H
Nagase
Atsuhiko
A
Tamai
Masamitsu
M
Kinuta
Masakatsu
M
D006528:Carcinoma, Hepatocellular / Q000209:etiology* / Q000601:surgery; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000150:complications*; D006498:Hepatectomy; D006801:Humans; D008113:Liver Neoplasms / Q000209:etiology* / Q000601:surgery; D008875:Middle Aged