A Case of Pseudomyxoma Peritonei Associated with Intraductal Papillary-Mucinous Neoplasm of the Pancreas

Koichi Kato, Soichiro Inoue, Hiroyuki Sugimoto, Naohito Kanazumi, Shuji Nomoto, Shin Takeda and Akimasa Nakao

Department of Surgery II, Nagoya University Graduate School of Medicine

We report a rare case of peritoneal metastasis of an intraductal papillary-mucinous neoplasm (IPMN) of the pancreas presenting pseudomyxoma peritonei. A 58-year-old man seen for a cystic tumor of the pancreatic head, was found in CT, MRCP and US to have multilocular cystic lesions in the pancreatic head and dilation of the entire main pancreatic duct. EUS showed thickening of the septum and mural nodule in the cystic tumor. We conducted pylorus-preserving pancreaticoduodenectomy in September 1995. The mucus-containing cystic tumor was 2 cm in maximum diameter. The pathological diagnosis was noninvasive intraductal papillary carcinoma of the main pancreatic duct and branches. In January 2003, seven years and three months later after initial surgery, the 66-year-old patient was readmitted for hematochezia and abdominal distention. Serum CEA was 166.9 ng/ml and CA19-9 was 10,495 U/ml. CT showed massive ascites and dilated intestines. Cytology of the ascites showed clustered epithelial cells and mucus. Pathological reexamination of the resected specimen showed cancer nests in mucus near the surgical margin. The patient deteriorated and died in February 2003 despite systemic chemotherapy with gemcitabine. An autopsy showed numerous gelatinous nodules of different sizes throughout the abdominal cavity. Histologically, cancer nests consisting of atypical mucinous cells with papillary growth floated in the mucinous material. Cancer was not found in the remnant pancreas. The definitive diagnosis was peritoneal dissemination from the original IPMN of the pancreas.

Key words
pseudomyxoma peritonei, IPMN, pancreas

Reprint requests
Koichi Kato Department of Surgery II, Nagoya University Graduate School of Medicine
65 Tsurumai-cho, Showa-ku, Nagoya, 466-8550 JAPAN

Accepted
April 25, 2007

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