A Case Report of the Granulocyte Colony-stimulating Factor Producing Intrahepatic Cholangiocarcinoma with Sarcomatous Change

• Published in: The Japanese Journal of Gastroenterological Surgery Vol. 46; no. 1; pp. 41 - 49
• Main Authors: Shimomura, Osamu, Sasaki, Ryoko, Fukunaga, Kiyoshi, Noguchi, Masayuki, Ohkohchi, Nobuhiro, Nakano, Yoritaka, Kobayashi, Akihiko, Oda, Tatsuya, Yamada, Kenji, Nowatari, Takeshi
• Format: Journal Article
• Language: Japanese
• Published: The Japanese Society of Gastroenterological Surgery 01.01.2013
• Subjects: cholangiocarcinoma; granulocyte colony-stimulating factor; paraneoplastic syndrome
• ISSN: 0386-9768; 1348-9372
• DOI: 10.5833/jjgs.2011.0349

A 64-year-old man with general fatigue visited a local hospital and received medical treatment for liver abscess. After that he was referred to our hospital because his symptoms did not improve. He had remittent fever and a marked granulocytosis was observed. Dynamic CT showed a huge liver tumor with a cystic lesion in the left lobe. We diagnosed it as cholangiocellular carcinoma and performed extended left lobectomy. Intraoperative findings were hemorrhagic ascites and ruptured tumor, but we carried out the operation in order to control bleeding from the tumor. Histopathological examination revealed intrahepatic cholangiocarcinoma with a tubular structure and sarcomatous component. Immunostaining with G-CSF revealed malignant cells. His preoperative serum G-CSF concentration was high (170 pg/ml). His condition gradually deteriorated after surgery. On postoperative day 14, CT revealed peritoneal dissemination, multiple liver metastases and metastasis to the gluteus muscle. He died of uncontrollable bleeding from peritoneal disseminated tumors on postoperative day 19. Only 7 cases of G-CSF-producing intrahepatic cholangiocarcinoma have been reported and their outcome was very poor because of their rapid tumor growth.