TY - JOUR
T1 - The clinical features of ovarian cancer in hereditary nonpolyposis colorectal cancer
AU - Watson, Patrice
AU - Bützow, Ralf
AU - Lynch, Henry T.
AU - Mecklin, Jukka Pekka
AU - Järvinen, Heikki J.
AU - Vasen, Hans F.A.
AU - Madlensky, Lisa
AU - Fidalgo, Paulo
AU - Bernstein, Inge
N1 - Funding Information:
This article was supported by revenue from Nebraska cigarette taxes awarded to Creighton University by the Nebraska Department of Health and Human Services. Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the State of Nebraska or the Nebraska Department of Health and Human Services. In addition to the listed authors, the following contributed to this study and manuscript: Miguel Rodriguez-Bigas, M.D., Roswell Park Cancer Institute (Buffalo); Rolf Sijmons, University of Groningen; Judy Ho, M.D., University of Hong Kong; Jae-Gahb Park, M.D., Seoul National University Hospital; Timothy Bishop, Ph.D., ICRF Genetic Epidemiology Laboratory, Leeds; Annika Lindblom, M.D., Karolin-ska Hospital, Stockholm; Diana Eccles M.D., MCRP, Princess Anne Hospital, Southampton.
PY - 2001
Y1 - 2001
N2 - Objective. Hereditary nonpolyposis colorectal cancer (HNPCC) is a hereditary cancer susceptibility disorder associated with a very high risk for carcinoma of the colon and an elevated risk for certain extracolonic cancers including ovarian cancer. Our aim in this study was to describe the clinicopathologic features of ovarian cancer in HNPCC family members. Methods. Members of the International Collaborative Group on HNPCC collected retrospective data on 80 ovarian cancer patients who were members of HNPCC families, including 31 known mutation carriers, 35 presumptive carriers (by colorectal/endometrial cancer status), and 14 at-risk family members. Results. Mean age at diagnosis of ovarian cancer was 42.7. Nonepithelial tumors made up only 6.4% of the cancers, and borderline tumors comprised just 4.1% of the epithelial cancers. Among frankly malignant epithelial cases, most cancers were well or moderately differentiated, and 85% were FIGO stage I or II at diagnosis. Synchronous endometrial cancer was reported in 21.5% of cases. Conclusions. Ovarian cancer in HNPCC differs from ovarian cancer in the general population in several clinically important respects. It occurs at a markedly earlier age. It is more likely to be epithelial. If it is a frankly invasive epithelial cancer, it is more likely to be well or moderately differentiated. HNPCC patients with ovarian cancer are more likely to have a synchronous endometrial cancer than other ovarian cancer patients and are more likely to be diagnosed at an early stage.
AB - Objective. Hereditary nonpolyposis colorectal cancer (HNPCC) is a hereditary cancer susceptibility disorder associated with a very high risk for carcinoma of the colon and an elevated risk for certain extracolonic cancers including ovarian cancer. Our aim in this study was to describe the clinicopathologic features of ovarian cancer in HNPCC family members. Methods. Members of the International Collaborative Group on HNPCC collected retrospective data on 80 ovarian cancer patients who were members of HNPCC families, including 31 known mutation carriers, 35 presumptive carriers (by colorectal/endometrial cancer status), and 14 at-risk family members. Results. Mean age at diagnosis of ovarian cancer was 42.7. Nonepithelial tumors made up only 6.4% of the cancers, and borderline tumors comprised just 4.1% of the epithelial cancers. Among frankly malignant epithelial cases, most cancers were well or moderately differentiated, and 85% were FIGO stage I or II at diagnosis. Synchronous endometrial cancer was reported in 21.5% of cases. Conclusions. Ovarian cancer in HNPCC differs from ovarian cancer in the general population in several clinically important respects. It occurs at a markedly earlier age. It is more likely to be epithelial. If it is a frankly invasive epithelial cancer, it is more likely to be well or moderately differentiated. HNPCC patients with ovarian cancer are more likely to have a synchronous endometrial cancer than other ovarian cancer patients and are more likely to be diagnosed at an early stage.
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U2 - 10.1006/gyno.2001.6279
DO - 10.1006/gyno.2001.6279
M3 - Article
C2 - 11531271
AN - SCOPUS:0034897120
VL - 82
SP - 223
EP - 228
JO - Gynecologic Oncology
JF - Gynecologic Oncology
SN - 0090-8258
IS - 2
ER -