Given the false-positive results for both CA125 and transvaginal ultrasonography, particularly in premenopausal women, and the absence of evidence from randomized trials that these tests reduce the mortality of ovarian cancer, these tests should not be used routinely to screen women at risk for ovarian cancer.
Most epithelial ovarian cancers are sporadic, but at least 5% to 10% result from inherited susceptibility and are hereditary. Hereditary ovarian cancers, particularly those caused by BRCA1 mutations, occur in women approximately 10 years younger than those with nonhereditary tumors.
Most hereditary ovarian cancers result from germline mutations in the BRCA1 and BRCA2 genes. The mutations are inherited in an autosomal dominant fashion, and therefore a full pedigree analysis (i.e., both maternal and paternal sides of the family history for both breast and ovarian cancer) must be carefully evaluated in all patients with epithelial ovarian cancer, and those with fallopian tube cancer and peritoneal cancer. The value of prophylactic salpingo-oophorectomy in these patients is well documented and is the most effective way to reduce risk of these cancers.
The importance of thorough surgical staging cannot be overemphasized, because subsequent treatment and prognosis will be determined by the stage of disease. Patients with advanced-stage disease should undergo debulking or cytoreductive surgery to remove as much of the tumor and its metastases as possible, if the patient is medically fit for major surgery. The performance of a debulking operation as early as possible in the course of the patient’s treatment is considered the standard of care for most patients. Most patients have a primary debulking surgery, while a smaller proportion of patients not suitable for initial surgery receive two to three cycles of primary chemotherapy followed by interval debulking surgery.
Combination chemotherapy with carboplatin and paclitaxel is recommended for patients with high-risk, low-stage disease. For advanced-stage epithelial ovarian cancer, the choice of intravenous versus intraperitoneal platinum and taxane chemotherapy should be individualized.
In the first two decades of life, almost 70% of ovarian tumors are of germ cell origin, and one-third of these are malignant. In contrast to the relatively slow-growing epithelial ovarian tumors, germ cell malignancies grow rapidly.
The most common types of malignant germ cell tumors are dysgerminomas, immature teratomas, and endodermal sinus tumors. Preservation of fertility should be standard in most patients. The most effective chemotherapy is bleomycin, etoposide, and cisplatin (BEP).
Stromal tumors include granulosa cell tumors, which are low-grade malignancies. In premenopausal women, they can be treated conservatively. Adjuvant chemotherapy is of unproven value.
Metastatic tumors to the ovaries are most frequently from the breast and gastrointestinal tract.
Fallopian tube carcinomas and peritoneal cancers are treated the same as ovarian cancer, with staging and cytoreductive surgery followed by platinum and taxane chemotherapy.