Hereditary prostate cancer is a genetically heterogeneous disease, and so f
ar four different susceptibility loci have been identified. Reports of asso
ciated cancers are few, and it is generally considered a sire-specific dise
ase. However, some reports have shown an elevated risk for prostate cancer
among BRCA2 mutation carriers. In this report, we present a family in which
the father and four of his sons were diagnosed with prostate cancer at exc
eptionally early ages (51, 52, 56, 58, and 63 years, respectively). In addi
tion, three daughters were diagnosed with breast cancer between the ages of
47 and 61. In this family, a truncating mutation in exon 11, 6051delA of t
he BRCA2 gene, leading to an early termination of the protein (codon 1962),
was identified. Although BRCA2 is probably responsible only for a very sma
ll fraction of hereditary prostate cancers, this finding supports previous
reports of an increased risk of prostate cancer in BRCA2 mutation carriers,
(C) 2001 Wiley-Liss, Inc.