Table 1. Clinical Features of Hereditary Cancer Syndromes Evaluated.
Syndrome | Most Common Lifetime Cancer Risks | Onset | Management Options | |
---|---|---|---|---|
Risk-reducing Surgery* | Early Detection | |||
HBOC | Breast 40-87% | Adult | ✓ | ✓ |
Ovarian 10-44% | ✓ | |||
LS | Colorectal 20-80% | Adult | ✓ | |
Endometrial 15-60% | ✓ | |||
FAP | Colorectal 95+% | Child | ✓ | |
MEN1 | Islet cell 40-75% | Child | ✓ | |
MEN2 | Thyroid 95+% | Child | ✓ | ✓ |
HBOC= hereditary breast and ovarian cancer syndrome; LS = Lynch syndrome; FAP = familial adenomatous polyposis; MEN = multiple endocrine neoplasia
For the purpose of this study, FAP is classified a syndrome without a risk-reducing surgical option since the surgery is typically performed when the polyp burden becomes too high to manage endoscopically, thus it is performed at a time when the patient is already affected with disease.