1) neoplastic transformation of the ovaries (can be malignant or benign) 2) classified by cell origin of abnormal growth – epithelial (70%), sex cord stromal (5%-10%), or germ cell (15%-20%) tumors

Signs and Symptoms

1) abdominal pain 2) bloating 3) urinary frequency 4) constipation 5) palpable pelvic mass on gynecologic exam 6) granulosa cell tumor (type of sex cord stromal tumor) causes vaginal bleeding and precocious puberty 7) Sertoli/Leydig cell tumors cause virilization and hirsuitism 8) generally do not become symptomatic until late stage

Characteristic Test Findings

Laboratory – 1) presence of CA-125 tumor marker (occurs in 85% of patients, but very controversial when used as a screening test because other conditions cause increased levels) 2) increased serum levels of alpha fetoprotein (AFP) in germ cell tumors 3) presence of beta-HCG in germ cell tumors 4) increased serum androgens in Sertoli/Leydig cell tumors Radiology – 5) mass on ovary in vaginal and/or abdominal ultrasound

Histology/Gross Pathology

1) epithelial subtypes – serous, mucinous, endometroid, clear cell, Brenner tumors 2) sex cord stromal subtypes – granulosa cell, theca cell, Sertoli’s cell, Leydig’s cell, and collagen-producing tumors 3) germ cell subtypes – teratomas, dysgerminoma, and endodermal sinus (yolk sac) tumors

Associated Conditions

increased incidence with – 1) nulliparity 2) infertility 3) clomiphene use 4) frequent miscarriages 5) family history of ovarian cancer 6) presence of BRCA1 and/or BRCA2 genes 7) Lynch’s syndrome (nonpolyposis colorectal cancer, endometrial cancer, ovarian cancer) 8) oncogenes c-myc, H-ras, K-ras, and neu

Inheritance/Epidemiology

1) 23,000 new cases in USA yearly 2) epithelial and some sex cord stromal tumors (Sertoli/Leydig cell tumors) are unusual before age 40 years 3) germ cell tumors and granulosa and theca cell tumors usually occur before age 30 years

Treatment

Surgery – 1) epithelial and sex cord stromal tumors – TAHBSO, omentectomy, biopsy of pelvic and periaortic nodes, and inspection of paracolic gutters with a “second-look” surgery after chemotherapy 2) germ cell tumors – removal of affected ovary only and no second look surgery Chemotherapy – 3) not for low-grade, stage 1 tumors 4) platinum-based with paclitaxel in epithelial and germ cell tumors; bleomycin-based in sex cord stromal tumors

Tips for USMLE

1) Krukenberg tumors are not primary ovarian tumors but rather metastatic “drop” tumors from the GI tract – classically described as originating in the stomach 2) if a 47 year-old woman who tried fertility drugs for 7 years develops diffuse lower abdominal pain, urinary frequency, and has a palpable mass on bimanual gynecologic exam, think ovarian cancer 3) CA-125 is usually followed post-treatment to see if tumor has recurred 4) germ cell tumors do not generally require removal of both ovaries and uterus, and fertility can be preserved.