Identifying HBOC syndrome has clinical implication for both the clinician and the individuals diagnosed, as they have an increased risk of several malignancies. Management strategies to address the increased cancer risk include genetic counseling and testing, targeted surveillance, chemoprevention, and prophylactic surgery. Genetic testing in high-risk family members is strongly recommended. The available current data indicate superiority of surgical prophylaxis compared with surveillance in reducing cancer risk in carriers of BRCA mutations. Surgery limitations and related adverse effects on morbidity and quality of life should always be discussed in extensive preoperative counseling. For evidence-based medical recommendation, more prospective data with appropriate surveillance protocol, longer follow-up, and quality-adjusted overall survival as endpoints are needed.
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