A Case of Mucinous Epithelial Cysts of the Spleen Associated with Pseudomyxoma Peritonei -A Clinical Study on the Diagnosis of the Primary Organ-

Hiroshi Takahashi, Tetsuya Yamaguchi, Ryoji Takeda, Shingo Sakata and Michihiro Yamamoto

Department of Surgery, Rakuwakai Otowa Hospital

A 61-year-old man was preoperatively diagnosed with early gastric cancer and pseudomyxoma peritonei associated with an idiopathic intraperitoneal giant cyst was found at the first laparotomy to have a cyst of splenic origin. Splenectomy and debulking of pseudomyxoma implants was done. The mucin-core-protein expression of acsites was MUC1 (-), MUC2 (+), MUC5AC (+), and epithelial cells of the mucinous cyst of the spleen expressed CK-7 (-), CK-20 (+), CEA (+), Vimentin (-). These results strongly suggested the existence of lower primary intesitinal tumor. Partial gastrectomy and appenndectomy done 7 months later confirmed that both mucinous ascites and pseudomyxoma implants disappeared and the presence of mucinous cystadenocarcinoma of the appendix, diagnosed as psuedomyxoma peritonei of primary appendiceal. Considering the peculiar clinicopathological features of this case, the huge splenic cyst versus the almost normal appendix, we assumed from the literature that mucin- secreting cells of the appendix, which spilled out into the abdominal cavity, redistributed around the splenic hilum, and invaded the spleen, then excessively secreting MUC2 mucin massively accumulated in the spleen, which had no way to drain, unlike the appendix.

Key words
cysts, pseudomyxoma peritonei, spleen

Reprint requests
Hiroshi Takahashi Department of Surgery, Rakuwakai Otowa Hospital
2 Chinji-cho, Yamashina-ku, Kyoto, 607-8062 JAPAN

Accepted
June 22, 2005

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