Karakterisering van families met hereditair prostaatcarcinoom in Nederland

To inventory the characteristics of Dutch families with hereditary prostate carcinoma (HPC).

Design

Descriptive.

Method

From a national registry of families that meet the criteria of HPC, information was collected about patients with HPC and their first-degree relatives from 1995 through to 30 June 2001. The ages of the HPC patients at diagnosis were compared with those of all patients with prostate cancer in the Dutch population during the period 1990 to 1996. The cumulative risk of prostate cancer for HPC families was calculated on the basis of the ages of the patients with prostate cancer and their first-degree male relatives.

Results

A total of 70 families fulfilled the criteria. The families included 273 patients with prostate cancer. The diagnosis had been confirmed in 208 (76) of these patients. Two cases of prostate cancer were observed in 3 families, 3 cases were found in 31 families, and in the remaining families 4-8 cases of prostate cancer were observed. The mean age at diagnosis of prostate cancer was 65.5 years (range: 46-89). Of the 273 HPC patients, 128 (47) were younger than 65 years at the time of diagnosis, whereas in unselected cases of prostate cancer this figure was 16. The risk of developing prostate cancer before the age of 70 years for members of HPC families was 39. The mean age of death due to prostate cancer was 71 years (54-84). The mean value of prostate specific antigen (PSA), known for 47 (17) of the HPC patients, was 36.8 ng/ml (2.1-280).