Cancer Family Syndrome -Case Report of Three Siblings with Multiple Colorectal Cancer-

Kenjiro Kotake, Yasuo Koyama, Tadashi Ikeda, Hideaki Shimizu, Shoichi Hishinuma, Takao Inada, Iwao Ozawa, Yoshiro Ogata

Department of Surgery, Tochigi Cancer Center

The case of three young siblings with multiple colorectal carcinomas and multiple primary cancer sites are reported. Additionally, three other relatives were found with cancer in their history, and the family was thought to have cancer family syndrome, as proposed by Lynch et al. Their grandfather was known to have a rectal carcinoma. And their mother had malignant lymphoma, multiple colon cancer and uterine cancer, and died of malignancy. Also her sister had died of uterine cancer. Among three siblings in the third generation, a total 14 lesions of colorectal carcinomas, a uterine carcinoma and an ovarian carcinoma were already identified up to the present. We reviewed eighteen cancer family syndrome (CFS) families reported in Japan. The analysis showed that 74 patients of them had colorectal cancer. Among the patients with colorectal cancer, the ratio of men to women was 1:0.9, and the onset of cancer was most frequent in the fourth decade of life. Proximal colon cancer in these patients occurred more frequently than in patients in the general population. Twenty-one of the 74 patients (28%) had multiple colorectal carcinomas, and 21 of the 74 patients (19%) had multiple primary cancer. Aside from endometrial cancer, which is one of the diagnostic criteria for CFS, gastric cancer was found most frequently in non-colorectal cancer patients.

Key words
cancer family syndrome, hereditary colorectal cancer

Reprint requests
Kenjiro Kotake Department of Surgery, Tochigi Cancer Center
4-9-13 Younan, Utsunomiya, 321 JAPAN

Accepted
November 19, 1990

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