The Role of Prophylactic Oophorectomy in Cancer Prevention

Prophylactic oophorectomy is presently the only effective method of ovarian cancer prevention. This study reviews current data on how prophylactic oophorectomy (PO) should be used in different risk groups. It is estimated that 7% of ovarian cancer patients have positive family history, of which 3-9% may end up having hereditary cancer syndromes. Women in direct genetic lineage of family cancer syndromes may have up to 50% lifetime risk of ovarian cancer. Because of such a high risk, PO is indicated for women with familial cancer syndromes after childbearing or the age of 35-40 at the latest. Most women with positive family history of ovarian cancer do not have one of the recognized hereditary cancer syndromes. However, women with one or two affected relatives do have an increased lifetime risk of ovarian cancer from a baseline of 1.6 to 5-7%. This risk is not high enough to warrant PO recommendation for a large number of women. After being properly informed and the patient still desires surgical prevention (i.e., cancer phobia), PO then becomes an indicated procedure. In women without family history of ovarian cancer, the role of PO remains controversial. The decision of PO as a concurrent procedure to other indicated gynecologic surgeries should depend on the individual patient and her ability to comply with lifelong estrogen replacement therapy.     

Should women with familial ovarian cancer undergo prophylactic oophorectomy?

OBJECTIVES: To estimate the lifetime probabilities of ovarian cancer in women from families with hereditary ovarian cancer syndromes and those with a family history of ovarian cancer, and to assess the needs for prevention and surveillance in such women. DATA SOURCES: We searched for studies of familial ovarian cancer published since 1966 and used ovarian cancer incidence data from the Surveillance, Epidemiology, and End Results program of the National Cancer Institute. METHODS: Pooled estimates of relative risk of ovarian cancer among women with a family history of ovarian cancer were derived using statistical methods based on fixed effects. Modified life-table methods were used to estimate the lifetime probability of ovarian cancer. DATA SYNTHESIS: The lifetime probability of ovarian cancer increases from about 1.6% in a 35-year-old woman without a family history of ovarian cancer to about 5% if she has one relative and 7% if she has two relatives with ovarian cancer. The lifetime probability may decrease to about 3-4% if she takes oral contraceptives for 5-9 years. Women from families with hereditary ovarian cancer syndromes may have as high as a 50% lifetime risk of ovarian cancer. CONCLUSIONS: The risk of ovarian cancer in women from families with hereditary ovarian cancer syndromes is sufficiently high to warrant prophylactic oophorectomy. Among women with one relative with ovarian cancer, the lifetime probability of ovarian cancer is not high enough to recommend oophorectomy. However, some women may choose oophorectomy depending on their attitudes concerning risk-taking, surgery, and hormone replacement. Oral contraceptives should be considered as preventive therapy to decrease the risk of ovarian cancer in women with a family history of ovarian cancer.     

Perioperative Management of Women Undergoing Risk-reducing Surgery for Hereditary Breast and Ovarian Cancer

Carriers of genetic mutations that predispose to cancer syndromes are often faced with complex decisions. For women with hereditary breast and ovarian cancer in particular, the decision to undergo risk-reducing mastectomy or bilateral salpingo-oophorectomy is burdensome from a physical and psychological perspective. Although risk-reducing surgery is the most effective preventative measure in reducing a genetic mutation carrier's risk of breast or ovarian cancer, the success of these procedures requires a multidisciplinary approach that centers on careful counseling regarding the risks and benefits of risk-reducing surgery. The physical and psychological distress associated with risk-reducing surgery often makes a combined surgical approach attractive to some patients. In this review, we present the evidence surrounding the comprehensive surgical care of women with hereditary breast and ovarian cancer syndromes and evaluate the perioperative factors that influence surgical management.     

Screening for familial ovarian cancer—management and outcome of women with moderate to high risk of developing ovarian cancer

Abstract.   Rieck GC, Lim K, Rogers MT, France E, Gray JR, Amso N, Evans AS, Howells RH, & Fiander AN. Screening for familial ovarian cancer—management and outcome of women with moderate to high risk of developing ovarian cancer. Int J Gynecol Cancer 2006; 16(Suppl. 1): 86–91.
Five percent to ten percent of ovarian cancers are hereditary. Individual genetic risk of developing ovarian malignancy is discussed in women. Currently, prophylactic surgery is advised to women with a moderate to high risk of developing ovarian cancer. Workload and outcome of the multidisciplinary familial ovarian screening clinic in South Wales were assessed. This was an observational study of 145 women registered with the Familial Ovarian Screening Clinic between January 1998 and December 2003. The data were retrieved from the medical notes. Yearly follow-ups were investigated with a transvaginal scan and CA125 level. Post-surgery women were followed up with yearly CA125 estimations: 46.9% fell into moderate-risk and 50.3% into high-risk category. The median age was 42 (SD 10.4), 71.7% were pre menopausal, and 10.3% had a personal history of breast cancer and 1.4% colon cancer. Whereas 36.5% opted for surgery, the remaining women (but two) opted for annual follow-up. Histology of the women who had surgery showed three cases of malignancies (fallopian tube carcinoma, atypical ovarian epithelial cells, and metastatic breast cancer). Seven women developed breast cancer during the observation period. The follow-up period is too short to come to a final conclusion as to the benefits of yearly screening in this group of women. In our series, a significant number of patients developed malignancies, despite prophylactic surgery.     

Gynecologic cancer as a "sentinel cancer" for women with hereditary nonpolyposis colorectal cancer syndrome

OBJECTIVE: Women with hereditary nonpolyposis colorectal cancer syndrome have a 40-60% lifetime risk for colon cancer, a 40-60% lifetime risk for endometrial cancer, and a 12% lifetime risk for ovarian cancer. A number of women with hereditary nonpolyposis colorectal cancer syndrome will have more than one cancer in their lifetime. The purpose of this study was to estimate whether women with hereditary nonpolyposis colorectal cancer syndrome who develop 2 primary cancers present with gynecologic or colon cancer as their "sentinel cancer." METHODS: Women whose families fulfilled Amsterdam criteria for hereditary nonpolyposis colorectal cancer syndrome and who developed 2 primary colorectal/gynecologic cancers in their lifetime were identified from 5 large hereditary nonpolyposis colorectal cancer syndrome registries. Information on age at cancer diagnoses and which cancer (colon cancer or endometrial cancer/ovarian cancer) developed first was obtained. RESULTS: A total of 117 women with dual primary cancers from 223 Amsterdam families were identified. In 16 women, colon cancer and endometrial cancer/ovarian cancer were diagnosed simultaneously. Of the remaining 101 women, 52 (51%) women had an endometrial or ovarian cancer diagnosed first. Forty-nine (49%) women had a colon cancer diagnosed first. For women who developed endometrial cancer/ovarian cancer first, mean age at diagnosis of endometrial cancer/ovarian cancer was 44. For women who developed colon cancer first, the mean age at diagnosis of colon cancer was 40. CONCLUSION: In this large series of women with hereditary nonpolyposis colorectal cancer syndrome who developed 2 primary colorectal/gynecologic cancers, endometrial cancer/ovarian cancer was the "sentinel cancer," preceding the development of colon cancer, in half of the cases. Therefore, gynecologists and gynecologic oncologists play a pivotal role in the identification of women with hereditary nonpolyposis colorectal cancer syndrome.     

林奇综合征与妇科肿瘤

林奇综合征（Lynch syndrome）又称为遗传性非息肉性结直肠癌综合征（HNPCC）,属于常染色体显性遗传性疾病,是最常见的结直肠癌遗传形式。林奇综合征患者常会患有多种肿瘤,其中子宫内膜癌及卵巢癌与其关系最为密切,可以视为林奇综合征的“前哨”肿瘤。在诊断患有林奇综合征的女性中,其患子宫内膜癌的终生风险（60%）会高于患结直肠癌的风险。结合临床表现标准及肿瘤分子学评估可以对其进行高效的诊断。在林奇综合征的女性患者中,应每1～2年（而不是每年）进行子宫内膜活检,在生育结束后行预防性手术可以起到有效的筛查及预防作用。对林奇综合征及其相关的子宫内膜癌及卵巢癌不断有新的研究进展,主要对林奇综合征的诊断、相关的子宫内膜癌及卵巢癌进行综述。     

Screening for gynaecologic cancers in genetically predisposed women

Hereditary breast and ovarian cancer syndrome and hereditary non-polyposis colon cancer syndrome are the two most important syndromes responsible for inherited cancers in gynaecology. Genetic testing is available for both these syndromes. Breast cancer gene testing is affordable and easy in women with ancestry where the mutation patterns are known, whereas other population groups need full gene screening. Hereditary non-polyposis colon cancer syndrome can now be diagnosed more frequently with the use of immunohistochemistry. Ovarian cancer risk is high in hereditary breast and ovarian cancer syndromes, and advanced screening techniques should be used when preventive surgery is not an option. Early detection techniques offer less protection than prophylactic removal, but enable women to retain their reproductive organs. Oophorectomy has the advantage of reducing breast cancer risk. In colorectal cancer syndromes, the risk for endometrial and ovarian cancer is much elevated. These risks should be recognised and addressed as these diseases are easy to prevent.     

Hereditary ovarian cancer as a separate clinical entity

Family history of ovarian cancer has been regarded as the most important risk factor in developing this type of tumor. Recent identification of cloning of specific tumor suppressor genes responsible for hereditary ovarian cancer syndromes, resulted in the surge of enthusiasm and optimism regarding the practical application of genetic information. Many investigators believe that natural history of hereditary ovarian may significantly differ from sporadic cases. Some independent findings have shown that serous adenocarcinoma was the most frequent histological type in hereditary ovarian cancer patients, whereas the mucinous and the borderline were underrepresented in this group. Also, patients with hereditary ovarian cancer tend to develop the disease at younger than expected age. At the present moment, it remains unknown if environmental/lifestyle factors play a role in penetrance and expression of specific tumor suppressor genes in mutation carriers. The era of molecular genetics has raised hopes not only for a better understanding of biology and natural history of hereditary ovarian tumors but also a rapid breakthrough in prevention and management of this disease.     

Society of Gynecologic Oncologists Education Committee statement on risk assessment for inherited gynecologic cancer predispositions

Women with germline mutations in the cancer susceptibility genes, BRCA1 or BRCA2, associated with Hereditary Breast/Ovarian Cancer syndrome, have up to an 85% lifetime risk of breast cancer and up to a 46% lifetime risk ovarian cancer. Similarly, women with mutations in the DNA mismatch repair genes, MLH1, MSH2 or MSH6, associated with the Lynch/Hereditary Non-Polyposis Colorectal Cancer (HNPCC) syndrome, have up to a 40-60% lifetime risk of both endometrial and colorectal cancer as well as a 9-12% lifetime risk of ovarian cancer. Genetic risk assessment enables physicians to provide individualized evaluation of the likelihood of having one of these gynecologic cancer predisposition syndromes, as well the opportunity to provide tailored screening and prevention strategies such as surveillance, chemoprevention, and prophylactic surgery that may reduce the morbidity and mortality associated with these syndromes. Hereditary cancer risk assessment is a process that includes assessment of risk, education and counseling conducted by a provider with expertise in cancer genetics, and may include genetic testing after appropriate consent is obtained. This commentary provides guidance on identification of patients who may benefit from hereditary cancer risk assessment for Hereditary Breast/Ovarian Cancer and the Lynch/Hereditary Non-Polyposis Colorectal Cancer syndrome.     

Systematic review of management options for women with a hereditary predisposition to ovarian cancer

OBJECTIVE: To develop through consensus and literature review management options for women with a hereditary predisposition to ovarian cancer, outcomes of interest were incidence of ovarian cancer, life expectancy, additional benefits of prophylactic oophorectomy, complications of surgery, and alternatives to prophylactic oophorectomy. METHODS: MEDLINE, CANCERLIT, and the Cochrane Library databases were searched to September 2003 using the terms ovarian, cancer, neoplasms, prophylactic oophorectomy, ovariectomy, clinical trial, metaanalysis, and systematic review. RESULTS: Four cohort studies (two retrospective, two prospective) examined whether prophylactic oophorectomy reduced the lifetime risk of developing ovarian cancer. All the cohort studies found that prophylactic oophorectomy reduced the lifetime risk of developing ovarian cancer; however, this risk reduction was statistically significant in only two cohort studies. The complication rate of laparoscopic surgery for prophylactic oophorectomy is low (0.22-4.0%). However, there are long-term adverse effects of prophylactic oophorectomy, most notably the onset of early menopause. CONCLUSIONS: Based on the evidence from the four cohort studies, prophylactic oophorectomy does protect against the development of ovarian cancer.     

Ovarian transposition for patients with cervical carcinoma treated by radiosurgical combination

OBJECTIVE: To assess the indications, effectiveness, and complications of ovarian transposition before pelvic irradiation for cervical cancer. DESIGN: Prospective study. SETTING: Gynecologic oncology department at a French cancer center. PATIENT(S): One hundred seven patients treated for cervical cancer. INTERVENTION(S): Ovarian transposition to the paracolic gutters with radical hysterectomy and lymphadenectomy. MAIN OUTCOME MEASURE(S): Clinical and laboratory follow-up tests for ovarian function. RESULT(S): Bilateral ovarian transposition was achieved in 104 patients (98%). Twelve patients were lost to follow-up or excluded because of evolution of the disease. Preservation of ovarian function was achieved in 83% of the patients having follow-up. The rates of ovarian preservation were 100% for patients treated exclusively by surgery, 90% for patients treated by postoperative vaginal brachytherapy, and 60% for patients treated by postoperative external radiation therapy and vaginal brachytherapy. The main risk for ovarian failure was found in patients treated by external radiation therapy. CONCLUSION(S): Ovarian transposition is a safe and effective procedure for preserving ovarian function in patients treated by a radiosurgical combination. This procedure should be performed in patients <40 years of age with a small invasive cervical carcinoma (<3 cm) treated by initial surgery. In such selected cases, the risk of ovarian metastasis is low.     

Advances in the understanding of risk factors for ovarian cancer

The identification of risk factors for ovarian cancer is central to the goal of preventing deaths from this disease. Reproductive and hormonal history clearly modulate the risk of ovarian cancer. Continuous ovulation associated with nulliparity increases the likelihood of ovarian malignancy. Protective factors include conditions that suspend ovulation, such as pregnancy, lactation and oral contraceptive use. Hereditary syndromes account for 10% of ovarian cancer cases. The breast ovarian cancer syndrome is caused by mutations in the BRCA1 and BRCA2 genes and is associated with an 11-40% risk of developing ovarian cancer. The hereditary nonpolyposis colorectal cancer syndrome (HNPCC, or Lynch II) is caused by mutations in DNA mismatch repair genes and carries a 12% risk of ovarian cancer. Due to a lack of adequate screening techniques, women with BRCA1, BRCA2 or HNPCC mutations should consider prophylactic removal of the ovaries and fallopian tubes when childbearing is complete. Genetic polymorphisms are hereditary genetic variations that may act in concert with other genetic, hormonal or environmental factors to potentiate the risk of ovarian cancer. Finally, ovarian cancer risk is altered by environmental and behavioral factors. Further study of the risk factors for ovarian cancer is needed to develop effective preventive strategies.     

Genetic susceptibility testing and prophylactic oophorectomy

About 10% of ovarian cancer cases are thought to have a hereditary basis and family history is the strongest risk factor for the development of this disease. In the past, prophylactic oophorectomy has been advocated for women with two or more affected first-degree relatives. More recently, with the identification of the genes responsible for most hereditary ovarian cancers (BRCA1, BRCA2), oophorectomy can now be offered specifically to women who are mutation carriers. Conversely, non-carriers in these families can be reassured that their risk of ovarian cancer is not increased. The value of oophorectomy in mutation carriers has not yet been proven, however, and there are concerns that the benefit may be less than intuitively expected. First, although the lifetime risk of ovarian cancer initially was reported to be as high as 60%, more recent studies have reported risks in the range of 15-30%. A better understanding of the genetic and/or environmental basis of variable penetrance is needed to augment our ability to counsel women regarding their risk. In addition, peritoneal papillary serous carcinoma indistinguishable from ovarian cancer occurs in some women following oophorectomy. Studies that better define how often this occurs also are needed to establish more firmly the value of prophylactic oophorectomy. In view of the uncertainty regarding the efficacy of prophylactic oophorectomy, chemopreventive and early detection approaches also deserve consideration as strategies for decreasing ovarian cancer mortality in women who carry mutations in ovarian cancer susceptibility genes.     

Laparoscopic prophylactic oophorectomy in women with inherited risk of ovarian cancer.

The aim of this study was to specify the surgical procedure most adapted for prophylactic laparoscopic oophorectomy in patients with an inherited risk of ovarian cancer. This prospective study was based on a series of 27 patients who underwent prophylactic bilateral laparoscopic oophorectomy between September 1995 and January 1998. Nine patients underwent an oophorectomy (33%) and 18 patients an adnexectomy (67%). The laparoscopic procedure was converted into a laparotomy in one patient in whom an ovarian adenocarcinoma was detected during the surgical procedure. During final histologic examination of the ovaries, 23 patients were found to have benign atypical histologic alterations, one patient had an ovarian adenocarcinoma and only three patients (11%) had normal ovaries. In women with an inherited risk of ovarian cancer, during the laparoscopic procedure for prophylactic oophorectomy, the abdomino-pelvic cavity should be thoroughly explored with peritoneal cytology and systematic peritoneal biopsies. The laparoscopic procedure could be converted into a laparotomy if an ovarian cancer is discovered.     

The role and place of the prophilactic oophorectomy

The malignant diseases of the female genital organs present 24.7% from the all neoplastic diseases at the women and they are significant medical and social problem. In our country, the structure of the oncogynecological incidence (1999) shows, that ovarian cancer with its 17.9% takes third place, as well as it is the leading cause for death (8.4%) among the women with gynecological cancer. The present study shows, that the increased risk for ovarian cancer is connected with the advanced age, the use of some hormonal medicaments, diet reach of animal fats, as well as the presence of family history or hereditary cancer syndromes (5-10% of the cases). From the other site, decrease of the risk is present when there are more deliveries, breastfeeding, low calories vegetable diet, oral contraceptives use, sterilization and prophylactic oophorectomy. It is pointed out, that prophylactic oophorectomy is the only effective method for the prevention of the ovarian cancer, especially in the cases with familial or hereditary predisposition. In Bulgaria there is not established tradition of performing of prophylactic oophorectomy when there is high risk for ovarian cancer. At the same time according to the data of the National Health Insurance Fund for the period 01.04.2003 - 01.04.2004 were performed total 5987 hysterectomies, without preliminary data for oncological disease , together with oophorectomy or salpingooophorectomy at 3108 (53.1%) cases. In this respect, the place, role, as well as, the possible risks of this surgical procedure are discussed.     

Genetik und Prävention des Ovarialkarzinoms

Ovarian cancer has the poorest prognosis of all gynaecological neoplasia. Germline mutations of the genes BRCA1 and BRCA2 are considered to be of substantial relevance in hereditary ovarian cancer. The multifactorial aetiology of this phenomenon accounts for the various phenotypes which are not only relevant in ovarian cancer. The clinical relevance of these syndromes increases with the option of predictive genetic testing for the predisposing genes. The lifetime-risk for ovarian cancer in women with mutations of the BRCA1 and/or BRCA2 genes is increased by upto 60%. The clinical implications of identifying patients at high risk necessitates the optimal use of primary or secondary prevention as proposed by the federally funded “Project for Familiar Breast and Ovarian Cancer”.     

Hereditary risk of women's cancers

The characterization of specific genes responsible for the hereditary risk of common cancers has enabled the development of clinical tests designed to identify at-risk individuals and to significantly improve the clinical outcome of such individuals. Two of the most important syndromes associated with a hereditary risk of cancer in women are hereditary breast and ovarian cancer, resulting from the BRCA1 and BRCA2 genes, and hereditary non-polyposis colorectal cancer, caused primarily by the MLH1 and MSH2 genes. As testing for the hereditary risk of breast, ovarian, endometrial and colorectal cancer enters the clinical mainstream, physicians responsible for the health care of women are increasingly required to assess and provide guidance to healthy patients with a strong family history, cancer survivors who may be at risk of a second cancer and women who discover that a family member carries a specific mutation identified through genetic testing. Obstetricians and gynaecologists must therefore become familiar with the principles of assessing the family history for specific hereditary cancer syndromes, with the appropriate use of tests to confirm such syndromes and with the management options for women who have inherited a greatly increased risk of cancer.     

Treatment of FIGO stage IV ovarian carcinoma: results of primary surgery or interval surgery after neoadjuvant chemotherapy: a retrospective study

The objective of the study is to determine whether surgery influences the outcome of stage IV ovarian cancer. The study design is as follows: From May 1995 to December 2000, 129 patients with FIGO stage IV ovarian cancer, recruited in 42 centers, were prospectively included in GINECO first-line randomized studies of platinum-based regimens with paclitaxel administered simultaneously or sequentially. In all, 109 were eligible for this study. Standard peritoneal cytoreductive surgery was defined as a procedure including at least total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and peritoneal debulking. Surgery was considered optimal if residual lesions were smaller than 1 cm. The Kaplan-Meier method was used to compare survival. Initial abdominopelvic cytoreductive surgery was considered standard in 55 (54%) patients. Abdominopelvic surgery was optimal in 29 patients and nonoptimal in 26. Twenty-two (22%) patients had a simple biopsy, and 25 (24%) patients underwent substandard surgery. Twenty-two of these 47 patients without initial standard surgery underwent a second surgical procedure, and 17 of the 22 patients completed standard surgery. The median overall survival time in the entire population was 24.3 months (95% confidence interval [CI], 19.5-29.1 months). Patients treated without a cytoreductive surgical procedure had significantly worse median survival (15.1 months; 95% CI, 5.4-24.9 months) than patients who had optimal primary surgery (22.9 months; 95% CI, 15.6-30.1 months), nonoptimal primary surgery (27.1 months; 95% CI, 21.2-32.9 months), or neoadjuvant chemotherapy followed by surgery (45.5 months; 95% CI, 23.5-67.5 months) (P= .001). In conclusion, this study shows a significant benefit of debulking surgery in stage IV ovarian cancer patients who responded to neoadjuvant chemotherapy. Neoadjuvant chemotherapy can help to select patients for surgery.     

The clinical features of ovarian cancer in hereditary nonpolyposis colorectal cancer

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.     

Preoperative CA-125 levels in patients with hereditary compared to sporadic epithelial ovarian carcinoma

OBJECTIVE: The aim of this study was to determine whether a significant difference in preoperative CA-125 levels exists between patients with BRCA-associated hereditary ovarian carcinoma and those with sporadic ovarian carcinoma and whether the CA-125 level predicts the probability of optimal cytoreductive surgery. METHODS: From a retrospective cohort of 189 consecutive ovarian cancer patients genotyped for BRCA mutation status, data on preoperative CA-125 levels were available for 49/88 (56%) hereditary cases and 43/101 (43%) sporadic cases. Data on the extent of surgical cytoreduction were obtained for all 92 patients with available CA-125 data. Comparison of preoperative CA-125 levels between hereditary and sporadic groups was assessed using the Kruskal-Wallis chi(2) test. Correlation of surgical cytoreduction with preoperative CA-125 level was assessed using Fisher's exact test. RESULTS: Mean preoperative CA-125 levels were not significantly different among BRCA1 (2289 U/ml), BRCA2 (2586 U/ml), and sporadic (3307 U/ml) cases (P = 0.5). For hereditary cases, optimal cytoreduction was achieved in 59% of patients with preoperative CA-125 levels of <500 U/ml and in 52% of patients with preoperative levels >500 U/ml. For sporadic cases, optimal cytoreduction was achieved in 62% of patients with CA-125 levels of <500 U/ml and in 20% of patients with levels >500 U/ml (P = 0.01). CONCLUSIONS: Preoperative CA-125 levels are not significantly different for patients with hereditary compared to sporadic ovarian carcinoma. The probability of optimal cytoreduction is independent of the preoperative CA-125 level for hereditary cases, but optimal cytoreduction is significantly less likely for sporadic cases with CA-125 levels of >500 U/ml.