ASCO/SSO review of current role of risk-reducing surgery in common hereditary cancer syndromes.
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.
Guillem
José G
JG
Department of Surgery, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, Room C-1077, New York, New York 10021, USA. guillemj@mskcc.org
Wood
William C
WC
Moley
Jeffrey F
JF
Berchuck
Andrew
A
Karlan
Beth Y
BY
Mutch
David G
DG
Gagel
Robert F
RF
Weitzel
Jeffrey
J
Morrow
Monica
M
Weber
Barbara L
BL
Giardiello
Francis
F
Rodriguez-Bigas
Miguel A
MA
Church
James
J
Gruber
Stephen
S
Offit
Kenneth
K
D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D008297:Male; D008495:Medical Oncology / Q000379:methods* / Q000639:trends; D009154:Mutation; D009369:Neoplasms / Q000235:genetics* / Q000601:surgery*; D009386:Neoplastic Syndromes, Hereditary / Q000235:genetics* / Q000601:surgery*; D012306:Risk; D018570:Risk Assessment; D012955:Societies, Medical; D016896:Treatment Outcome; D014481:United States