Cause: Neoplasia; 5-10% familial association w breast cancer chromosome 17 BRCA-1 and BRCA-2 gene deletions (Nejm 2002;346:1609, 1616, 1660); perhaps somatic nonsense repeat mutations on chromosome 3 (Nejm 2009;360:2719)
Pathophys: Epithelial type cancer, 35-50% serous, 6-10% mucinous
5th most fatal female cancer in US, ahead of cervical and uterine; 1/70 lifetime risk is 1/20 if a 1st-degree relative has had ovarian cancer; 16000 deaths/yr in US; 25 000/yr incidence in US
Increased w low parity, mumps, perineal talc use, ERT users × 2, esp after 10 yr (Jama 2001;285:1460), BRCA-1 or -2 gene carriers
Decreased incidence (40%) with bcp use, even 3 mo of rx protects for 15+ yr (Nejm 1987;316:650); w high FSH levels postmenopausally and w low androgen levels (Jama 1996;274:1926)
Usually postmenopausal women age 50-60 yr; 5% of cases are familial
Sx:Often asx until widespread; or (Jama 2004;291:2705) back pain (45%), fatigue (34%), abdominal pain (22%), pelvic or lower abdominal mass, pelvic discomfort, urinary pain (16%), bowel bloating (27%), and/or constipation (24%); upper abdominal sense of fullness and increasing abdominal girth
Si:Abdominal or pelvic mass, often (50%) bilateral; may be huge (largest = 148 kg); ascites. Occasionally unique polyarthritis, palmar fasciitis syndrome (Ann IM 1982;96:424), hypercalcemia, multiple seb keratoses.
Table 11.1 Overian Cancer Staging and Survival
| Stage | 5-Yr Survival (%) |
|---|---|
| I | |
| Ia: Confined to 1 ovary | 90+ |
| Ib: Both ovaries | 90+ |
| Ic: 1-2 ovaries, w ascites + positive peritoneal cytology | 80 |
| II: 1-2 ovaries w local pelvic extension | |
| IIa: Confined to uterus and tubes | 51 |
| IIb: Other pelvic tissues | 42 |
| IIc: Ascites or pos abdominal cytology | 42 |
| III: 1 or 2 ovaries and peritoneal or retroperitoneal mets | |
| IIIa: Microscopically out of pelvis | |
| IIIb: Peritoneal mets <2 cm2 | All ~20 |
| IIIc: Retroperitoneal nodes or mets >2 cm2 | |
| IV: Distant mets | 10 |
R/o common, benign ovarian corpus luteum cyst by rechecking pelvic during a different part of menstrual cycle, getting ultrasound if increasing in size, doing surgery if >5 cm; germ cell or sex cord struma types, 13-20% of ovarian cancers, very treatable; mets from elsewhere (Krukenberg's tumors); Meig's syndrome
Lab:
Chem:Ca-125 monoclonal antibodies elevated in 80% but not useful as screening test (Nejm 1992;327:197) unless 2 1st-degree relatives w same type of ovarian cancer (Jama 1995;273:491); serum inhibin levels elevated in many, esp mucinous cystadenocarcinomas, like Ca-125, not useful screen but helpful postop tumor marker (Nejm 1993;329:1539)
Path:"Borderline malignant" cell types of both serous and mucinous have much better prognosis than numbers shown above, eg, 85-95% 5-yr survival
Xray:Pelvic ultrasound; screen w vaginal probe if pos fam hx as above
Rx:
Prevent in BRCA-1 and -2 w prophylactic oophorectomy (BSOO) after childbearing reduces ovarian and breast Ca risk ×75% (Nejm 2002;346:1609, 1616, 1660); maybe bcp's decr incidence by 50+%? (Nejm 1998;339:424 vs 2001;345:235)
Preventive screening w tumor markers ineffective primarily due to low prevalence (Ann IM 1994;121:124; 1993;119:901), and w pelvic exams because fast growing and usually already spread when palpable
Staging laparoscopy/otomy to fully judge extent of metastases, eg, often on diaphragm
Debulking surgery after initial chemotherapy (Nejm 1995;332:629) but a second debulking surgery after chemoRx no help (Nejm 2001;351:2489)
Chemotherapy for most except good histology stage Ia, esp for stages III and IV with (Med Let 1996;38:96): cisplatin iv (Nejm 1996;335:1950), or intraperitoneal (Nejm 2006;354:34), plus paclitaxel (Taxol) (Nejm 1996;334:1)