ASCO/SSO review of current role of risk-reducing surgery in common hereditary cancer syndromes Journal Article

Authors: Guillem, J. G.; Wood, W. C.; Moley, J. F.; Berchuck, A.; Karlan, B. Y.; Mutch, D. G.; Gagel, R. F.; Weitzel, J.; Morrow, M.; Weber, B. L.; Giardiello, F.; Rodriguez-Bigas, M. A.; Church, J.; Gruber, S.; Offit, K.
Article Title: ASCO/SSO review of current role of risk-reducing surgery in common hereditary cancer syndromes
Abstract: Although the etiology of solid cancers is multifactorial, with environmental and genetic factors playing a variable role, a significant portion of the burden of cancer is accounted for by a heritable component. Increasingly, the heritable component of cancer predispositions has been linked to mutations in specific genes, and clinical interventions have been formulated for mutation carriers within affected families. The primary interventions for mutations carriers for highly penetrant syndromes such as multiple endocrine neoplasias, familial adenomatous polyposis, hereditary nonpolyposis colon cancer, and hereditary breast and ovarian cancer syndromes are primarily surgical. For that reason, the American Society of Clinical Oncology (ASCO) and the Society of Surgical Oncology (SSO) have undertaken an educational effort within the oncology community. A joint ASCO/SSO Task Force was charged with presenting an educational symposium on the surgical management of hereditary cancer syndromes at the annual ASCO and SSO meetings, resulting in an educational position article on this topic. Both the content of the symposium and the article were developed as a consensus statement by the Task Force, with the intent of summarizing the current standard of care. This article is divided into four sections addressing breast, colorectal, ovarian and endometrial cancers, and multiple endocrine neoplasia. For each, a brief introduction on the genetics and natural history of the disease is provided, followed by a detailed description of modern surgical approaches, including a description of the clinical and genetic indications and timing of prophylactic surgery, and the efficacy of prophylactic surgery when known. Although a number of recent reviews have addressed the role of genetic testing for cancer susceptibility, including the richly illustrated Cancer Genetics and Cancer Predisposition Testing curriculum by the ASCO Cancer Genetics Working Group (available through, this article focuses on the issues surrounding the why, how, and when of surgical prophylaxis for inherited forms of cancer. This is a complex process, which requires a clear understanding of the natural history of the disease and variance of penetrance, a realistic appreciation of the potential benefit and risk of a risk-reducing procedure in a potentially otherwise healthy individual, the long-term sequelae of such surgical intervention, as well as the individual patient and family's perception of surgical risk and anticipated benefit. © 2006 Society of Surgical Oncology. All rights reserved.
Keywords: cancer surgery; gene mutation; review; cancer risk; cancer staging; endometrium cancer; genetic analysis; clinical practice; colorectal cancer; treatment indication; cancer susceptibility; consensus; ovary cancer; breast cancer; tumor biopsy; brca1 protein; brca2 protein; heterozygote; health program; clinical education; conservative treatment; colon cancer; medical society; familial cancer; curriculum development; desmoid tumor; endocrine tumor; multiple endocrine neoplasia; colon polyposis
Journal Title: Journal of Clinical Oncology
Volume: 24
Issue: 28
ISSN: 0732-183X
Publisher: American Society of Clinical Oncology  
Date Published: 2006-10-01
Start Page: 4642
End Page: 4660
Language: English
DOI: 10.1200/jco.2005.04.5260
PROVIDER: scopus
Notes: --- - "Cited By (since 1996): 89" - "Export Date: 4 June 2012" - "CODEN: JCOND" - "Source: Scopus"
Altmetric Score
MSK Authors
  1. Monica Morrow
    450 Morrow
  2. Kenneth Offit
    519 Offit
  3. Jose Guillem
    373 Guillem