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There are >22,000 new cases of ovarian cancer annually in the United States, and approximately 14,000 women will die of their disease, making this the most lethal of gynecologic cancers. This accounts for 2.3% of all cancer deaths nationally.
Malignancy that arises from the epithelium (90–95%), sex cord stromal, or germ cells of the ovary as well as tumors metastatic to the ovary. Histologic types include the following:
- High grade serous (most common, 70–80%)
- Low grade serous
- Clear cell
- Malignant Brenner tumor (transitional cell epithelium)
- Carcinosarcoma (malignant mixed müllerian tumor)
- Sex cord stromal
- Granulosa cell tumor
- Sertoli-Leydig cell tumors
- Lipid (steroid) cell tumor
- Germ cell
- Teratoma (immature)
- Embryonal carcinoma
- Endodermal sinus tumor (yolk sac tumor)
- Metastatic disease from the following:
- GI tract (Krukenberg tumor)
- System(s) affected: GI, genitourinary, endocrine
- 22,440 new cases per year in the United States; 14,070 deaths per year
- Leading cause of gynecologic cancer death in women
- Majority of ovarian cancer is diagnosed at an advanced stage.
- Average age of diagnosis
- Epithelial: 63 years
- Sex cord stromal: 50 years
- Germ cell: 10 to 30 years
Lifetime risk for general population: 1 in 78 American women develop ovarian cancer.
Etiology and Pathophysiology
- Malignant transformation of the ovarian epithelium may result from repeated trauma and inflammation during ovulation.
- Ovarian, fallopian tube, and primary peritoneal carcinomas have identical histologic and morphologic features. A higher percentage of ovarian cancers are now known to originate in the fallopian tube and other components of the secondary müllerian system, including primary peritoneal cancers.
- Hereditary breast/ovarian cancer syndrome: early-onset breast or ovarian cancer, autosomal dominant transmission with variable penetrance, usually associated with BRCA-1 or BRCA-2 (tumor suppressor gene) mutation
- Lynch syndrome: autosomal dominant inheritance; increased risk for colorectal, endometrial, stomach, small bowel, breast, pancreas, and ovarian cancers; defect in DNA mismatch repair genes
- 90% of ovarian cancer is sporadic and not inherited, but family history is the most significant risk factor. Multiple relatives with breast or ovarian cancer increase risk: Refer these patients for genetic counseling. Individuals in families with familial cancer syndromes have 20–60% risk of developing ovarian cancer.
- Risk factors: older age, white race, infertility, nulligravidity, early menarche or late menopause, endometriosis, postmenopausal estrogen replacement therapy, residence in an industrialized Western country (i.e., North America, Northern Europe)
- Association between fertility medications and risk of ovarian cancer is controversial, but women with infertility who have a successful live birth do not have an increased risk of ovarian cancer.
- Use of oral contraceptives: 5 years of use decreases risk by 20%; 15 years of use decreases risk by 50% (1)[A]
- Tubal ligation or hysterectomy
- Risk-reducing salpingo-oophorectomy
- Protective effect of aspirin and NSAIDs in ovarian cancer is controversial.
- Recommendations for high-risk (family history of a hereditary ovarian cancer syndrome) population (2)[A]
- Women should undergo pelvic examinations, CA-125 level measurement, and transvaginal US every 6 to 12 months beginning at age 30 or 10 years prior to the earliest age of diagnosis of ovarian cancer in the family.
- Women with family histories of ovarian cancer or premenopausal breast cancer should be referred for genetic counseling.
- Prophylactic oophorectomy is advised for mutation carriers after childbearing is completed or by age 35 years.
- Risk of primary peritoneal carcinoma remains 1–2% after prophylactic oophorectomy.
- Screening: No effective screening exists for ovarian cancer in the general population (2)[A].
- Routine use of CA-125 and transvaginal US for screening in women of average risk is NOT recommended. Annual pelvic examinations may be performed, particularly in postmenopausal women. An adnexal mass in a premenarchal female or a palpable adnexa in a postmenopausal female warrants further evaluation.
Commonly Associated Conditions
- Pleural effusion
- Bowel obstruction
- Breast cancer
- Endometrial cancer