Japanese Registry for Adenomatosis Coli

Abstract

We analized the characteristics of adenomatosis coli in Japan. Currently, 788 cases (550 families) have been registered to the Polyposis and Intestinal Disease Study Center.
In the 39.0% of the families, no patient but the propositus was found (non familial case).
The average age at diagnosis in the propositus group was found to be 34.6 years (men), and 31.9 years (female). Carcinoma was already present in 63.3% of the propositus group and in 22.9% of the call up group, respectively at the time of diagnosis.
As to surgical treatment, standard operation (protocolectomy or total colectomy) had been employed in 55 % of the propositus group.
Ten years survival rate was 63.8 % and 72.8 %, respectively. In the call up group, standard operation had been employed in 76.9% of patients.
It was noted that the survival rate of total colectomy group had a decrease after 7-8 years postoperatively.
Aside from colorectal carcinoma, several carcinoma ocurred to the patient of adenomatosis coli. They were carcinoma of stomach (33 cases), duodenum (10), small intestine (8), uterus (8) and thyroid (8).
Conclusively, we would like to stress out the importance of a registration system for the longterm follow up and the treatment of adenomatosis coli.