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

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Abstract

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.

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Acknowledgments

The authors thank ASCO and the SSO and its leadership during the inception and conduct of this effort: namely, Drs. Paul Bunn, Alfred Cohen, John Daly, John Niederhuber, Larry Norton, and Glenn Steele for their encouragement and support. We especially thank Dr. Charles Balch, Executive Vice President and CEO of ASCO, for his key role in initially suggesting and supporting this initiative. We also thank Dana Wollins and Fran Stigliano of ASCO for administrative support and Jenifer Levin for editorial support. The joint ASCO/SSO Task Force of Surgical Management of Cancer Predisposition Syndromes was chaired by José Guillem, MD, and Kenneth Offit, MD. The members of the Task Force were as follows: breast cancer section—William C. Wood, MD (Section Leader), Monica Morrow, MD, and Barbara Weber, MD; FAP/HNPCC section—José G. Guillem, MD, MPH (Section Leader), James Church, MD, Francis Giardiello, MD, and Miguel Rodriguez-Bigas, MD; endometrial/ovarian cancer section—Andrew Berchuck, MD (Section Leader), Beth Y. Karlan, MD, and David G. Mutch, MD; MEN 2 section—Jeffrey F. Moley, MD (Section Leader), and Robert F. Gagel, MD; and clinical cancer genetics section—Kenneth Offit, MD (Section Leader), Stephen Gruber, MD, and Jeffrey Weitzel, MD.

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Authors and Affiliations

  1. Department of Surgery, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, Room C-1077, New York, New York, 10021, USA

    José G. Guillem MD, MPH

  2. Department of Surgery, Emory University School of Medicine, Atlanta, Georgia, USA

    William C. Wood MD

  3. Department of Surgery, Washington University School of Medicine, St. Louis, Missouri, USA

    Jeffrey F. Moley MD

  4. Department of Obstetrics and Gynecology, Duke University Medical Center, Durham, North Carolina, USA

    Andrew Berchuck MD

  5. Department of Obstetrics and Gynecology, Cedars Sinai Medical Center and David Geffen School of Medicine at UCLA, Los Angeles, California, USA

    Beth Y. Karlan MD

  6. Department of Obstetrics and Gynecology, Washington University School of Medicine, St. Louis, Missouri, USA

    David G. Mutch MD

  7. Department of Surgery, The University of Texas M. D. Anderson Cancer Center, Houston, Texas, USA

    Robert F. Gagel MD & Miguel A. Rodriguez-Bigas MD

  8. Department of Clinical Cancer Genetics, City of Hope National Medical Center, Duarte, California, USA

    Jeffrey Weitzel MD

  9. Department of Surgery, Fox-Chase Cancer Center, Philadelphia, Pennsylvania, USA

    Monica Morrow MD

  10. Departments of Medicine and Genetics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA

    Barbara L. Weber MD

  11. Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA

    Francis Giardiello MD

  12. Department of Colorectal Surgery, The Cleveland Clinic Foundation, Cleveland, Ohio, USA

    James Church MD

  13. Department of Medicine, The University of Michigan Medical Center, Ann Arbor, Michigan, USA

    Stephen Gruber MD

  14. Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York, New York, USA

    Kenneth Offit MD, MPH

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  1. José G. Guillem MD, MPH
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  2. William C. Wood MD
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  15. Kenneth Offit MD, MPH
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Correspondence to José G. Guillem MD, MPH.

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Guillem, J.G., Wood, W.C., Moley, J.F. et al. ASCO/SSO Review of Current Role of Risk-Reducing Surgery in Common Hereditary Cancer Syndromes. Ann Surg Oncol 13, 1296–1321 (2006). https://doi.org/10.1245/s10434-006-9036-6

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