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Hereditary ovarian cancer. Heterogeneity in age at diagnosis
Author(s) -
Lynch Henry T.,
Watson Patrice,
Bewtra Chhanda,
Conway Theresa A.,
Hippee Connie Read,
Kaur Prabjhot,
Lynch Jane F.,
Ponder Bruce A. J.
Publication year - 1991
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19910301)67:5<1460::aid-cncr2820670534>3.0.co;2-s
Subject(s) - ovarian cancer , medicine , family history , cancer , breast cancer , lynch syndrome , oncology , population , natural history , age of onset , gynecology , colorectal cancer , disease , environmental health , dna mismatch repair
An unknown fraction of the ovarian cancer burden occurs in women with a family history indicative of a putative autosomal dominantly inherited cancer susceptibility syndrome. The results from a five‐generation, extended, hereditary breast‐ovarian cancer kindred are described 10 years after it was initially ascertained. Significantly more cancers were observed in high‐risk family members during this decade than were expected ( P < 0.001). The age of ovarian cancer diagnosis was studied in additional ovarian cancer‐prone families of three types: site‐specific ovarian cancer syndrome, the breast–ovarian cancer syndrome, and Lynch syndrome II. The age of onset in each of the three sets was significantly ( P < 0.001) earlier than the general population mean of 59, and there were significant differences in the age of onset ( P = 0.050) among these three cohorts. Ovarian cancer histology was similar to that of patients with negative family histories. There may be clinically significant heterogeneity in the age at diagnosis of ovarian cancer among these ovarian cancer‐prone syndromes. This has important implications for understanding its natural history and targeting surveillance–management strategies.