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ASCO/SSO Review of Current Role of Risk-Reducing Surgery in Common Hereditary Cancer Syndromes

¹ Department of Surgery, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, Room C-1077, New York, New York 10021, USA
² Department of Surgery, Emory University School of Medicine, Atlanta, Georgia, USA
³ Department of Surgery, Washington University School of Medicine, St. Louis, Missouri, USA
⁴ Department of Obstetrics and Gynecology, Duke University Medical Center, Durham, North Carolina, USA
⁵ Department of Obstetrics and Gynecology, Cedars Sinai Medical Center and David Geffen School of Medicine at UCLA, Los Angeles, California, USA
⁶ Department of Obstetrics and Gynecology, Washington University School of Medicine, St. Louis, Missouri, USA
⁷ Department of Surgery, The University of Texas M. D. Anderson Cancer Center, Houston, Texas, USA
⁸ Department of Clinical Cancer Genetics, City of Hope National Medical Center, Duarte, California, USA
⁹ Department of Surgery, Fox-Chase Cancer Center, Philadelphia, Pennsylvania, USA
¹⁰ Departments of Medicine and Genetics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
¹¹ Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
¹² Department of Colorectal Surgery, The Cleveland Clinic Foundation, Cleveland, Ohio, USA
¹³ Department of Medicine, The University of Michigan Medical Center, Ann Arbor, Michigan, USA
¹⁴ Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York, New York, USA

Correspondence: Address correspondence and reprint requests to: José G. Guillem, MD, MPH; E-mail: guillemj{at}mskcc.org

Background: A significant portion of cancers are accounted for by a heritable component, which has increasingly been linked to mutations in specific genes. Clinical interventions have been formulated for mutation carriers within affected families. The primary interventions for mutation carriers of highly penetrant syndromes are surgical.

Methods: The American Society of Clinical Oncology and the Society of Surgical Oncology formed a task force charged with presenting an educational symposium on surgical management of hereditary cancer syndromes at annual society meetings, and this resulted in a position paper on this topic. The content of both the symposium and the position paper was developed as a consensus statement.

Results: This article addresses hereditary breast, colorectal, ovarian/endometrial, and multiple endocrine neoplasias. A brief introduction on the genetics and natural history of each disease is provided, followed by detailed descriptions of modern surgical approaches, clinical and genetic indications, timing of prophylactic surgery, and the efficacy of surgery (when known). Although several recent reviews have addressed the role of genetic testing for cancer susceptibility, this article focuses on the issues surrounding surgical technique, timing, and indications for surgical prophylaxis.

Conclusions: Risk-reducing surgical treatment of hereditary cancer is a complex undertaking. It requires a clear understanding of the natural history of the disease, realistic appreciation of the potential benefits and risks of these procedures in potentially otherwise healthy individuals, and the long-term sequelae of such interventions, as well as the individual patient’s and family’s perceptions of surgical risk and anticipated benefit.

Key Words: Hereditary cancer • Risk-reducing surgery • Surgical prophylaxis • Multiple endocrine neoplasia • Familial adenomatous polyposis • HNPCC