A Case Report of Familial Polyposis Coli Associated with Multiple Duodenal Carcinoma and Desmoid Tumor

Suguru Sawada, Katsuhiko Uesaka, Tomoyuki Katoh, Takeshi Morimoto, Yasushiro Kodera, Akihito Torii, Takashi Hirai, Kenzou Yasui, Yoshitaka Yamamura, Tsuyoshi Kitoh

Department of Gastroenterological Surgery, Aichi Cancer Center

A fifty-two-year-old female with familial adenomatous polyposis (FAP) underwent a total colectomy in 1981. She was diagnosed as having an intraabdominal desmoid tumor in 1985 and was successfully treated by oral administration of Tamoxifen and Sulindac. In 1995, she underwnet an endoscopy and was diagnosed as having three duodenal cancer-in-adenomas at the opposite side of the major papilla, accompanied by multiple duodenal adenomas. She underwent a laparotomy in September 1995, but pyloric-preserving pancreatico-duodenectomy (PPPD), initially proposed as the treatment of choice, was considered impracticable because of extensive mesenterial invasion by he desmoid tumor that would have prevented the reconstruction procedures. Consequently, removal of the tumors and coagulation of less prominent lesions were performed through duodenostomy. Although duodenal lesions are commonly found in patients with FAP, multiple cancerous lesions not arising from the periampullary region have rarely been reported. Desmoid tumor, another commonly found tumor in FAP patients, can interere with a surgical treatment for such a duodenal lesion, and calls for meticulously planned treatment strategies.

Key words
familial adenomatous polyposis, duodenal cancer, desmoid tumor

Reprint requests
Suguru Sawada Department of Surgery, Kaizu Medical Association Hospital
656-16 Hukue, Kaizu-cho, Gifu, 503-06 JAPAN

Accepted
June 12, 1996

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